CHiP Network Congenital Heart Journal Watch – June 2019- Archive

Pediatric Cardiology Featured Articles

Pediatric Cardiology Featured Manuscripts of April 2019

 

Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles.

Biko DM, Gaynor JW, Partington SL, Harris MA, Whitehead KK, Trusty P, Yoganathan AP, Fogel M.

Ann Thorac Surg. 2019 Apr 5. pii: S0003-4975(19)30496-5. doi: 10.1016/j.athoracsur.2019.03.019. [Epub ahead of print]

PMID: 30959013

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Take Home Points:

 

  • Single ventricle patients with previous aortic reconstruction have higher aortic pulse wave velocity (PWV) compared to single ventricle (SV) patients without aortic reconstruction.
  • There is an inverse relationship between aortic distensibility and exercise stress test parameters in SV patients who underwent aortic reconstruction.


Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:
 In this very interesting prospective study, the authors examined the relationship between parameters of aortic stiffness and exercise performance in SV patients after Fontan completion. 48 SV patients underwent real-time exercise cardiovascular magnetic resonance (CMR) imaging. 18 out of 48 patients had previous aortic reconstruction. Aortic pulse wave velocity PWV, aortic distensibility, ventricular volumes as well as metabolic exercise stress test parameters were measured. Patients with previous aortic reconstruction had a higher aortic PWV compared to those without aortic reconstruction (figure 1). In addition, the authors found an inverse relationship between PWV and indexed end-diastolic volume, indexed end-systolic volume and stroke volume (at rest and during exercise) in the group of patients with aortic reconstruction. There was no significant difference in aortic distensibility between patients with a reconstructed aorta and those without aortic reconstruction. In patients with previous aortic reconstruction the authors found inverse correlations between aortic distensibility and peak O2 pulse, peak oxygen consumption (figure 2), oxygen consumption at the anaerobic threshold as well as peak work, respectively.

 

These results might improve our understanding of decreased exercise capacity in single ventricle patients who had previous aortic reconstruction.

Figure 1

 

 

Figure 2

 

  1. Right and Left Ventricular Strain Patterns After the Atrial Switch Operation for D-Transposition of the Great Arteries-A Magnetic Resonance Feature Tracking Study.

Burkhardt BEU, Kellenberger CJ, Franzoso FD, Geiger J, Oxenius A, Valsangiacomo Buechel ER.

Front Cardiovasc Med. 2019 Apr 9;6:39. doi: 10.3389/fcvm.2019.00039. eCollection 2019.

PMID: 31024933 Free PMC Article

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Take Home Points:

 

  • In patients with transposition of the great arteries (TGA) after atrial switch operation the longitudinal strain of the systemic right ventricle is impaired compared to both a normal subpulmonary right ventricle and a systemic left ventricle.
  • Free wall ventricular strain values for both ventricles were higher compared to global strain values in TGA patients.

 

Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  This is a Magnetic Resonance feature tracking study in adult patients with TGA after atrial switch operation. The authors included 29 adult patients with TGA after atrial switch operation and 19 healthy controls. Cardiac Magnetic Resonance (CMR) cine images were used for the feature tracking analysis and to measure ventricular volumes, ejection fraction (EF) as well as mitral and tricuspid annular plane systolic excursion. Cine phase contrast imaging was applied for internal validation of stroke volumes. The feature tracking analysis included the measurement of longitudinal and circumferential strain of both ventricles. Regional strain values for the left ventricle (LV) were measured using the American Heart Association 17-segment model; for the right ventricle (RV) a “mirror imaged” model for defining the RV segments was used. Cardiopulmonary exercise (CPEX) data were also collected.

The main findings include that the RV in the systemic position had larger volumes and lower EF than the systemic LV in healthy controls. Furthermore, the subpulmonary LV in patients was smaller and had a higher EF than the RV in healthy controls. In TGA patients, annular plane systolic excursion of the systemic and subpulmonary ventricles were lower compared to the corresponding systemic and subpulmonary ventricle in the control group. Global longitudinal strain of the systemic RV in patients was lower compared to the RV in controls (Figure 2). Compared to the systemic LV in healthy subjects the systemic RV in patients had both, lower global circumferential and longitudinal strains (Figure 3). The subpulmonary LV had a lower global circumferential strain compared to the systemic LV in controls (Figure 2). However, when compared to the subpulmonary RV in controls global circumferential strain of the subpulmonary LV was increased (Figure 3). Regional strain values in TGA patients after atrial switch were higher in the free wall compared to global strain values (Table 3). CPEX data did not correlate with ventricular strain values.

 

Overall this study increases our knowledge about the global and regional function of the systemic RV in TGA patients after atrial switch operation and opens the way for further studies in this field.

 

  1. Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein’s Anomaly.

Meierhofer C, Kühn A, Müller J, Shehu N, Hager A, Martinoff S, Stern H, Ewert P, Vogt M.

Pediatr Cardiol. 2019 Apr;40(4):792-798. doi: 10.1007/s00246-019-02066-7. Epub 2019 Feb 6.

PMID: 30726509

 

Take Home Points

 

  • Exercise capacity is used to prognosticate in patients with Ebstein’s anomaly (EA) and cardiac magnetic resonance (CMR) is used to assess volumetric and functional parameters
  • Functional parameters measured by CMR have been shown to correlate with exercise capacity and can be used in follow-up for patients with EA, possibly to predict the need for surgical intervention prior to development of symptoms

 

Comment from Dr. Shelby White (Tucson AZ), section editor of Pediatric Cardiology Journal Watch:  Ebstein’s anomaly (EA) results in volume overload and dysfunction of the right ventricle due to tricuspid regurgitation and atrialization of the ventricle.  Exercise capacity is used as a prognostic variable, specifically % predicted peak oxygen uptake (peak VO2%), for event free survival in EA.  CMR is used for right ventricular assessment in congenital heart disease, this study sought to evaluate variables assessed by CMR and their correlation to peak VO2%.

As seen in table 4, the majority of the parameters that correlated significantly with functional status as determined by peak VO2% were physiologic parameters rather than volumetric measurements.  These parameters which influence cardiac output may be followed over time to predict a decline in function in patients with EA.

 

Pediatric cardiology April 2019

 

  1. Selected acculturation factors and birth defects in the National Birth Defects Prevention Study, 1997-2011.

Hoyt AT, Shumate CJ, Canfield MA, Le M, Ramadhani T, Scheuerle AE; National Birth Defects Prevention Study.

Birth Defects Res. 2019 Apr 25. doi: 10.1002/bdr2.1494. [Epub ahead of print]

PMID: 31021057

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  1. EPAS 1, Congenital Heart Disease, and High Altitude: Disclosures by Genetics, Bioinformatics, and Experimental Embryology.

Sergi C.

Biosci Rep. 2019 Apr 23. pii: BSR20182197. doi: 10.1042/BSR20182197. [Epub ahead of print]

PMID: 31015364

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  1. Accuracy of cardiac auscultation in detection of neonatal congenital heart disease by general paediatricians.

Zhao QM, Niu C, Liu F, Wu L, Ma XJ, Huang GY.

Cardiol Young. 2019 Apr 23:1-5. doi: 10.1017/S1047951119000799. [Epub ahead of print]

PMID: 31012400

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  1. A Primer on Multimodal Imaging and Cardiology-Radiology Congenital Heart Interface.

Gupta-Malhotra M, Schaaf W, Kutty S.

Children (Basel). 2019 Apr 23;6(4). pii: E61. doi: 10.3390/children6040061.

PMID: 31018523 Free Article

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  1. Usefulness of Liver Stiffness on Ultrasound Shear-Wave Elastography for the Evaluation of Central Venous Pressure in Children With Heart Diseases.

Terashi E, Kodama Y, Kuraoka A, Ishikawa Y, Nakamura M, Sagawa K, Ishikawa S.

Circ J. 2019 Apr 23. doi: 10.1253/circj.CJ-18-1313. [Epub ahead of print]

PMID: 31019158 Free Article

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  1. Association between maternal exposure to pollutant particulate matter 2.5 and congenital heart defects: a systematic review.

Hall KC, Robinson JC.

JBI Database System Rev Implement Rep. 2019 Apr 23. doi: 10.11124/JBISRIR-2017-003881. [Epub ahead of print]

PMID: 31021973

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  1. A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery.

Zhang Y, Wang B, Li Y, Xie M.

Echocardiography. 2019 Apr 22. doi: 10.1111/echo.14355. [Epub ahead of print]

PMID: 31012154

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  1. Tetralogy of Fallot with absent pulmonary valve-When the ductus is present: A case of isolated branch pulmonary artery and review of literature.

Rao S, Najm HK, Stewart RD, Ahmad M, Erenberg F, Yaman M.

Echocardiography. 2019 Apr 22. doi: 10.1111/echo.14334. [Epub ahead of print]

PMID: 31006901

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  1. Cefpodoxime proxetil as a therapeutic option in switching therapy for infective endocarditis in children: case reports and literature review.

Krajcar N, Marić LS, Šarić D, Milić N, Tešović G.

J Chemother. 2019 Apr 22:1-5. doi: 10.1080/1120009X.2019.1603797. [Epub ahead of print]

PMID: 31007148

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  1. Oxygen Saturation and Perfusion Index-Based Enhanced Critical Congenital Heart Disease Screening.

Siefkes H, Kair L, Tancredi DJ, Vasquez B, Garcia L, Bedford-Mu C, Lakshminrusimha S.

Am J Perinatol. 2019 Apr 19. doi: 10.1055/s-0039-1685445. [Epub ahead of print]

PMID: 31003242

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  1. Mean platelet volume and major adverse cardiovascular events in congenital heart disease patients.

Martínez-Quintana E, Rodríguez-Hernández JL, Riaño-Ruiz M, Rodríguez-González F.

Clin Hemorheol Microcirc. 2019 Apr 19. doi: 10.3233/CH-180471. [Epub ahead of print]

PMID: 31006669

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  1. Comparison of creatinine and cystatin C for estimation of glomerular filtration rate in pediatric patients after Fontan operation.

Kirelik D, Fisher M, DiMaria M, Soranno DE, Gist KM.

Congenit Heart Dis. 2019 Apr 16. doi: 10.1111/chd.12776. [Epub ahead of print]

PMID: 30993817

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  1. Bicuspid Aortic Valve Alters Aortic Protein Expression Profile in Neonatal Coarctation Patients.

Skeffington KL, Bond AR, Abdul-Ghani S, Iacobazzi D, Kang SL, Heesom KJ, Wilson MC, Ghorbel M, Stoica S, Martin R, Suleiman MS, Caputo M.

J Clin Med. 2019 Apr 16;8(4). pii: E517. doi: 10.3390/jcm8040517.

PMID: 30995723 Free Article

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  1. Reverse Remodeling of Pulmonary Arterioles After Pulmonary Artery Banding in Patients ≥ 2 Years Old with Severe Pulmonary Arterial Hypertension and Congenital Heart Disease.

Liu C, Cheng P, Liu A, Li B, Yang Y, Wang Z, Su J.

Pediatr Cardiol. 2019 Apr 13. doi: 10.1007/s00246-019-02097-0. [Epub ahead of print]

PMID: 30982075

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  1. A novel method optimizing the normalization of cardiac parameters in small animal models: the importance of dimensional indexing.

Hagdorn QAJ, Bossers GPL, Koop AMC, Piek A, Eijgenraam TR, van der Feen DE, Silljé HHW, de Boer RA, Berger RMF.

Am J Physiol Heart Circ Physiol. 2019 Apr 12. doi: 10.1152/ajpheart.00182.2019. [Epub ahead of print]

PMID: 30978120

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  1. Hand-held echocardiography in children with hypoplastic left heart syndrome.

Riley AF, Ocampo EC, Hagan J, Lantin-Hermoso MR.

Congenit Heart Dis. 2019 Apr 11. doi: 10.1111/chd.12774. [Epub ahead of print]

PMID: 30973683

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  1. Cardiovascular profile and biophysical profile scores predict short-term prognosis in infants with congenital heart defect.

Miyoshi T, Katsuragi S, Neki R, Kurosaki KI, Shiraishi I, Nakai M, Nishimura K, Yoshimatsu J, Ikeda T.

J Obstet Gynaecol Res. 2019 Apr 11. doi: 10.1111/jog.13970. [Epub ahead of print]

PMID: 30977251

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  1. Surveillance of Congenital Heart Defects among Adolescents at Three U.S. Sites.

Lui GK, McGarry C, Bhatt A, Book W, Riehle-Colarusso TJ, Dunn JE, Glidewell J, Gurvitz M, Hoffman T, Hogue CJ, Hsu D, Obenhaus S, Raskind-Hood C, Rodriguez FH 3rd, Zaidi A, Van Zutphen AR.

Am J Cardiol. 2019 Apr 10. pii: S0002-9149(19)30409-6. doi: 10.1016/j.amjcard.2019.03.044. [Epub ahead of print]

PMID: 31030970 Free Article

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  1. A rare association of left pulmonary artery sling with right pulmonary hypoplasia and total anomalous pulmonary venous connection.

Kitadai Y, Kan N, Tsukimori K.

Cardiol Young. 2019 Apr 10:1-3. doi: 10.1017/S1047951119000131. [Epub ahead of print]

PMID: 30968804

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  1. [A case of 10p15.3 microdeletion syndrome detected by whole exome sequencing].

Chen W, Fu N, Liang J, Qin J.

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2019 Apr 10;36(4):331-335. doi: 10.3760/cma.j.issn.1003-9406.2019.04.010. Chinese.

PMID: 30950019

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  1. Should we start a nationwide screening program for critical congenital heart disease in Turkey? A pilot study on four centres with different altitudes.

Dilli D, Doğan V, Özyurt BM, Özyurt A, Hakan N, Bozabalı S, Caner İ, Olgun H, Koç M, Taşoğlu İ, Karademir S, Zenciroğlu A.

Cardiol Young. 2019 Apr 8:1-6. doi: 10.1017/S1047951119000052. [Epub ahead of print]

PMID: 30957737

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  1. A YWHAZ Variant Associated With Cardiofaciocutaneous Syndrome Activates the RAF-ERK Pathway.

Popov IK, Hiatt SM, Whalen S, Keren B, Ruivenkamp C, van Haeringen A, Chen MJ, Cooper GM, Korf BR, Chang C.

Front Physiol. 2019 Apr 8;10:388. doi: 10.3389/fphys.2019.00388. eCollection 2019.

PMID: 31024343 Free PMC Article

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  1. Relationship of Aortic Stiffness to Exercise and Ventricular Volumes in Single Ventricles.

Biko DM, Gaynor JW, Partington SL, Harris MA, Whitehead KK, Trusty P, Yoganathan AP, Fogel M.

Ann Thorac Surg. 2019 Apr 5. pii: S0003-4975(19)30496-5. doi: 10.1016/j.athoracsur.2019.03.019. [Epub ahead of print]

PMID: 30959013

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Select item 30948702

 

  1. Management of Congenital Heart Disease: State of the Art-Part II-Cyanotic Heart Defects.

Rao PS.

Children (Basel). 2019 Apr 4;6(4). pii: E54. doi: 10.3390/children6040054.

PMID: 30987364 Free Article

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  1. Increased blood pressure is associated with increased carotid artery intima-media thickness in children with repaired coarctation of the aorta.

Dempsey AA, Parraga G, Altamirano-Diaz L, Welisch E, Park TS, Grattan M, Al-Khazraji BK, Norozi K.

J Hypertens. 2019 Apr 3. doi: 10.1097/HJH.0000000000002077. [Epub ahead of print]

PMID: 30950974

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  1. Assessing the criteria for definition of perimembranous ventricular septal defects in light of the search for consensus.

Tretter JT, Tran VH, Gray S, Ta H, Loomba RS, O’Connor W, Spicer DE, Cook AC, Anderson RH.

Orphanet J Rare Dis. 2019 Apr 3;14(1):76. doi: 10.1186/s13023-019-1044-2.

PMID: 30944003 Free PMC Article

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  1. Accuracy of pulse oximeters at low oxygen saturations in children with congenital cyanotic heart disease: An observational study.

Kim EH, Lee JH, Song IK, Kim HS, Jang YE, Yoo S, Kim JT.

Paediatr Anaesth. 2019 Apr 2. doi: 10.1111/pan.13642. [Epub ahead of print]

PMID: 30938906

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Select item 30938017

 

  1. Echocardiographic screening of 4107 Nigerian school children for rheumatic heart disease.

Ekure EN, Amadi C, Sokunbi O, Kalu N, Olusegun-Joseph A, Kushimo O, Hassan O, Ikebudu D, Onyia S, Onwudiwe C, Nwankwo V, Akinwunmi R, Awusa F, Akere Z, Dele-Salawu O, Ajayi E, Ale O, Muoneke D, Muenke M, Kruszka P, Beaton A, Sable C, Adeyemo A.

Trop Med Int Health. 2019 Apr 2. doi: 10.1111/tmi.13235. [Epub ahead of print]

PMID: 30938017

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Select item 30709599

 

  1. Preduodenal Portal Vein Associated with Complex Congenital Heart Disease and Heterotaxy Syndrome: An Unexpected Cause of Common Complaint.

Okada S, Muneuchi J, Furuno W, Kamimura T.

Am Surg. 2019 Apr 1;85(4):e233-e234. No abstract available.

PMID: 31043224

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Select item 30911773

 

  1. Validation of a Second-Generation Near-Infrared Spectroscopy Monitor in Children With Congenital Heart Disease.

Nasr VG, Bergersen LT, Lin HM, Benni PB, Bernier RS, Anderson ME, Kussman BD.

Anesth Analg. 2019 Apr;128(4):661-668. doi: 10.1213/ANE.0000000000002796.

PMID: 29324491

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  1. Physiologic diagnosis of congenital heart disease in cyanotic neonates.

Desai K, Rabinowitz EJ, Epstein S.

Curr Opin Pediatr. 2019 Apr;31(2):274-283. doi: 10.1097/MOP.0000000000000742.

PMID: 30730315

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Select item 31026813

 

  1. Three-dimensional echocardiography in congenital heart disease.

Simpson J, van den Bosch AE.

Echo Res Pract. 2019 Apr 1. pii: ERP-18-0074.R1. doi: 10.1530/ERP-18-0074. [Epub ahead of print] Review.

PMID: 31026813 Free Article

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  1. Tricuspid atresia with restrictive foramen ovale: A rare combination with implications on fetal growth.

Tseng SY, Alsaied T, Barnard K, Hahn E, Divanovic AA, Cnota JF.

Echocardiography. 2019 Apr;36(4):800-802. doi: 10.1111/echo.14267. Epub 2019 Jan 28.

PMID: 30693549

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  1. Intake of sucrose-sweetened soft beverages during pregnancy and risk of congenital heart defects (CHD) in offspring: a Norwegian pregnancy cohort study.

Dale MTG, Magnus P, Leirgul E, Holmstrøm H, Gjessing HK, Brodwall K, Haugen M, Stoltenberg C, Øyen N.

Eur J Epidemiol. 2019 Apr;34(4):383-396. doi: 10.1007/s10654-019-00480-y. Epub 2019 Jan 19.

PMID: 30661159

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Select item 30936990

 

  1. Haploinsufficiency of vascular endothelial growth factor related signaling genes is associated with tetralogy of Fallot.

Reuter MS, Jobling R, Chaturvedi RR, Manshaei R, Costain G, Heung T, Curtis M, Hosseini SM, Liston E, Lowther C, Oechslin E, Sticht H, Thiruvahindrapuram B, Mil SV, Wald RM, Walker S, Marshall CR, Silversides CK, Scherer SW, Kim RH, Bassett AS.

Genet Med. 2019 Apr;21(4):1001-1007. doi: 10.1038/s41436-018-0260-9. Epub 2018 Sep 20.

PMID: 30232381

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  1. Trisomy of the Short Arm of Chromosome 12 Associated with High Cardiovascular Risk: A Case Report.

Fulcheri C, Balietti P, Rabbia F, Schiavone D, Magnino C, Abate Daga F, Gollin M, Veglio F.

High Blood Press Cardiovasc Prev. 2019 Apr;26(2):143-144. doi: 10.1007/s40292-019-00307-7. Epub 2019 Feb 26.

PMID: 30806948

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Select item 30967519

 

  1. Incidence and risk factors of unplanned intubation during pediatric sedation for MRI.

Kim D, Lee EK, Jeong JS, Gil NS, Hahm TS, Shin YH.

J Magn Reson Imaging. 2019 Apr;49(4):1053-1061. doi: 10.1002/jmri.26314. Epub 2018 Oct 22.

PMID: 30350443

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  1. Is echocardiography necessary for all single umbilical artery fetuses? A retrospective study in a selected Chinese population.

Wang J, Ye Y, Xin T, Zhang X, Chen S, Wu Y, Sun K.

J Obstet Gynaecol Res. 2019 Apr;45(4):803-809. doi: 10.1111/jog.13912. Epub 2019 Jan 15.

PMID: 30644151

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Select item 30528575

 

  1. Characterization of Post-Thrombotic Syndrome in Children with Cardiac Disease.

Manlhiot C, McCrindle BW, Williams S, Menjak IB, O’Shea S, Chan AK, Brandão LR.

J Pediatr. 2019 Apr;207:42-48. doi: 10.1016/j.jpeds.2018.10.064. Epub 2018 Dec 7.

PMID: 30528575

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  1. Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium.

Lal DR, Gadepalli SK, Downard CD, Minneci PC, Knezevich M, Chelius TH, Rapp CT, Billmire D, Bruch S, Carland Burns R, Deans KJ, Fallat ME, Fraser JD, Grabowski J, Hebel F, Helmrath MA, Hirschl RB, Kabre R, Kohler J, Landman MP, Leys CM, Mak GZ, Ostlie DJ, Raque J, Rymeski B, Saito JM, St Peter SD, von Allmen D, Warner BW, Sato TT; Midwest Pediatric Surgery Consortium.

J Pediatr Surg. 2019 Apr;54(4):688-692. doi: 10.1016/j.jpedsurg.2018.08.002. Epub 2018 Aug 21.

PMID: 30224238

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Select item 30670916

 

  1. Transthoracic Echocardiographic Evaluation of Pulmonary Valve Anomalies in Pediatric Patients.

Wang SS, Xu MG, Zhuang J, Li WB, Zhang ZW, Xu G.

J Ultrasound Med. 2019 Apr;38(4):1091-1096. doi: 10.1002/jum.14780. Epub 2018 Oct 7.

PMID: 30294866

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Select item 30171623

 

  1. Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease.

Foulks MG, Meyer RML, Gold JI, Herrington CS, Kallin K, Menteer J.

Pediatr Cardiol. 2019 Apr 1. doi: 10.1007/s00246-019-02093-4. [Epub ahead of print]

PMID: 30937501

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Select item 30918992

 

  1. Recurrent Congenital Heart Diseases Among Neonates Born to Mothers with Congenital Heart Diseases.

Yokouchi-Konishi T, Yoshimatsu J, Sawada M, Shionoiri T, Nakanishi A, Horiuchi C, Tsuritani M, Iwanaga N, Kamiya CA, Neki R, Miyake A, Kurosaki K, Shiraishi I.

Pediatr Cardiol. 2019 Apr;40(4):865-870. doi: 10.1007/s00246-019-02083-6. Epub 2019 Mar 4.

PMID: 30830281

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Select item 30830280

 

  1. Relationship Between Pulmonary Arterial Resistance and Compliance in Patients with Down Syndrome.

Iwaya Y, Muneuchi J, Inoue Y, Watanabe M, Okada S, Ochiai Y.

Pediatr Cardiol. 2019 Apr;40(4):841-847. doi: 10.1007/s00246-019-02080-9. Epub 2019 Mar 4.

PMID: 30830280

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Select item 30729260

 

  1. Non-invasive Hemodynamic CMR Parameters Predicting Maximal Exercise Capacity in 54 Patients with Ebstein’s Anomaly.

Meierhofer C, Kühn A, Müller J, Shehu N, Hager A, Martinoff S, Stern H, Ewert P, Vogt M.

Pediatr Cardiol. 2019 Apr;40(4):792-798. doi: 10.1007/s00246-019-02066-7. Epub 2019 Feb 6.

PMID: 30726509

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Select item 30652193

 

  1. Effects of oxygen therapy in a pediatric normoxemic patient with pulmonary arterial hypertension and congenital heart disease.

Green S, Stuart D.

Pulm Circ. 2019 Apr-Jun;9(2):11772045894019845610. doi: 10.1177/2045894019845610.

PMID: 30942130

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Select item 30767601

 

  1. Equine Congenital Heart Disease.

Scansen BA.

Vet Clin North Am Equine Pract. 2019 Apr;35(1):103-117. doi: 10.1016/j.cveq.2018.11.001. Epub 2019 Feb 27. Review.

PMID: 30826104

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  1. Efficacy and Safety of Oral Paracetamol vs. Oral Ibuprofen in the Treatment of Symptomatic Patent Ductus Arteriosus in Premature Infants.

Karabulut B, Paytoncu S.

Paediatr Drugs. 2019 Apr 26. doi: 10.1007/s40272-019-00331-z. [Epub ahead of print]

PMID: 31025304

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Select item 31045530

 

  1. A Primer on Multimodal Imaging and Cardiology-Radiology Congenital Heart Interface.

Gupta-Malhotra M, Schaaf W, Kutty S.

Children (Basel). 2019 Apr 23;6(4). pii: E61. doi: 10.3390/children6040061.

PMID: 31018523 Free Article

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Select item 31019158

 

  1. The Bayley-III scale may underestimate neurodevelopmental disability after cardiac surgery in infants†.

Goldstone AB, Baiocchi M, Wypij D, Stopp C, Andropoulos DB, Atallah J, Atz AM, Beca J, Donofrio MT, Duncan K, Ghanayem NS, Goldberg CS, Hövels-Gürich H, Ichida F, Jacobs JP, Justo R, Latal B, Li JS, Mahle WT, McQuillen PS, Menon SC, Pike NA, Pizarro C, Shekerdemian LS, Synnes A, Williams IA, Bellinger DC, Newburger J, Gaynor JW.

Eur J Cardiothorac Surg. 2019 Apr 21. pii: ezz123. doi: 10.1093/ejcts/ezz123. [Epub ahead of print]

PMID: 31006006

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Select item 31006348

 

  1. Truncus arteriosus communis associated with interrupted aortic arch – Type B and right aberrant subclavian artery: A rare entity.

Olejnik P, Majerova L, Tomko J, Bjelosevic M.

J Cardiovasc Comput Tomogr. 2019 Apr 19. pii: S1934-5925(19)30128-5. doi: 10.1016/j.jcct.2019.04.011. [Epub ahead of print] No abstract available.

PMID: 31027931

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Select item 31002586

 

  1. Comparison of Clinical Symptoms and Cardiac Lesions in Children with Typical and Atypical Kawasaki Disease.

Behmadi M, Alizadeh B, Malek A.

Med Sci (Basel). 2019 Apr 18;7(4). pii: E63. doi: 10.3390/medsci7040063.

PMID: 31003567 Free Article

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Select item 31005576

 

 

  1. Whole-Exome Sequencing Reveals Novel Genetic Variation for Dilated Cardiomyopathy in Pediatric Chinese Patients.

Dai G, Pu Z, Cheng X, Yin J, Chen J, Xu T, Zhang H, Li Z, Chen X, Chen J, Qin Y, Yang S.

Pediatr Cardiol. 2019 Apr 16. doi: 10.1007/s00246-019-02096-1. [Epub ahead of print]

PMID: 30993396

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  1. Paediatric cardio-oncology: epidemiology, screening, prevention, and treatment.

Chow EJ, Leger KJ, Bhatt NS, Mulrooney DA, Ross CJ, Aggarwal S, Bansal N, Ehrhardt MJ, Armenian SH, Scott JM, Hong B.

Cardiovasc Res. 2019 Apr 15;115(5):922-934. doi: 10.1093/cvr/cvz031.

PMID: 30768157

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Select item 30423020

 

  1. Targeted expression of cyclin D2 ameliorates late stage anthracycline cardiotoxicity.

Zhu W, Reuter S, Field LJ.

Cardiovasc Res. 2019 Apr 15;115(5):960-965. doi: 10.1093/cvr/cvy273.

PMID: 30423020

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Select item 30989806

 

  1. Usefulness of 4D-Flow MRI in Mapping Flow Distribution Through Failing Fontan Circulation Prior to Cardiac Intervention.

McLennan D, Schäfer M, Mitchell MB, Morgan GJ, Ivy D, Barker AJ, Jacobsen R.

Pediatr Cardiol. 2019 Apr 13. doi: 10.1007/s00246-019-02101-7. [Epub ahead of print]

PMID: 30982076

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  1. Diuretic Responsiveness and its Prognostic Significance in Children with Heart Failure.

Price JF, Younan S, Cabrera AG, Denfield SW, Tunuguntla H, Choudhry S, Dreyer WJ, Akcan-Arikan A.

J Card Fail. 2019 Apr 12. pii: S1071-9164(18)31235-1. doi: 10.1016/j.cardfail.2019.03.019. [Epub ahead of print]

PMID: 30986498

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  1. Right and Left Ventricular Strain Patterns After the Atrial Switch Operation for D-Transposition of the Great Arteries-A Magnetic Resonance Feature Tracking Study.

Burkhardt BEU, Kellenberger CJ, Franzoso FD, Geiger J, Oxenius A, Valsangiacomo Buechel ER.

Front Cardiovasc Med. 2019 Apr 9;6:39. doi: 10.3389/fcvm.2019.00039. eCollection 2019.

PMID: 31024933 Free PMC Article

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  1. Anomalous aortic origin of the right coronary artery from the non-coronary sinus of Valsalva.

Doshi AR, Opfer EK, Forsha D.

Cardiol Young. 2019 Apr 8:1-3. doi: 10.1017/S1047951119000039. [Epub ahead of print]

PMID: 30957729

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  1. Assessing the criteria for definition of perimembranous ventricular septal defects in light of the search for consensus.

Tretter JT, Tran VH, Gray S, Ta H, Loomba RS, O’Connor W, Spicer DE, Cook AC, Anderson RH.

Orphanet J Rare Dis. 2019 Apr 3;14(1):76. doi: 10.1186/s13023-019-1044-2.

PMID: 30944003 Free PMC Article

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  1. Revisitation of Double-Inlet Left Ventricle or Tricuspid Atresia With Transposed Great Arteries.

Park WK, Baek JS, Kwon BS, Im YM, Lee JH, Choi ES, Park CS, Yun TJ.

Ann Thorac Surg. 2019 Apr;107(4):1212-1217. doi: 10.1016/j.athoracsur.2018.11.052. Epub 2018 Dec 23.

PMID: 30586578

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  1. Differences in Cost of Care by Palliation Strategy for Infants With Ductal-Dependent Pulmonary Blood Flow.

Goldstein BH, O’Byrne ML, Petit CJ, Qureshi AM, Dai D, Griffis HM, France A, Kelleman MS, McCracken CE, Mascio CE, Shashidharan S, Ligon RA, Whiteside W, Wallen WJ, Agrawal H, Aggarwal V, Glatz AC.

Circ Cardiovasc Interv. 2019 Apr;12(4):e007232. doi: 10.1161/CIRCINTERVENTIONS.118.007232.

PMID: 30998390

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  1. Physiologic diagnosis of congenital heart disease in cyanotic neonates.

Desai K, Rabinowitz EJ, Epstein S.

Curr Opin Pediatr. 2019 Apr;31(2):274-283. doi: 10.1097/MOP.0000000000000742.

PMID: 30730315

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  1. Exercise stress echocardiography: Impact on clinical decision-making in pediatric patients.

Dasgupta S, Friedman H, Allen N, Stark M, Ferguson E, Sachdeva R, Border WL.

Echocardiography. 2019 Apr 1. doi: 10.1111/echo.14326. [Epub ahead of print]

PMID: 30934142

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  1. Heart rate reduction strategy using ivabradine in end-stage Duchenne cardiomyopathy.

Adorisio R, Calvieri C, Cantarutti N, D’Amico A, Catteruccia M, Bertini E, Baban A, Filippelli S, Perri G, Amodeo A, Drago F.

Int J Cardiol. 2019 Apr 1;280:99-103. doi: 10.1016/j.ijcard.2019.01.052. Epub 2019 Jan 17.

PMID: 30686494

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  1. Role of echocardiography in the assessment of right ventricular function in the pediatric population.

Kamra K, Punn R.

Paediatr Anaesth. 2019 Apr 1. doi: 10.1111/pan.13641. [Epub ahead of print]

PMID: 30934152

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  1. Screening Echocardiography and Brain Natriuretic Peptide Levels Predict Late Pulmonary Hypertension in Infants with Bronchopulmonary Dysplasia.

Behere S, Alapati D, McCulloch MA.

Pediatr Cardiol. 2019 Apr 1. doi: 10.1007/s00246-019-02100-8. [Epub ahead of print]

PMID: 30937503

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  1. Standardization of a Continuous Ramp Ergometer Protocol for Clinical Exercise Testing in Children.

Octavio JM, Folk AL, Falini L, Xie S, Goudie BW, Gidding SS, Robinson BW.

Pediatr Cardiol. 2019 Apr;40(4):834-840. doi: 10.1007/s00246-019-02079-2. Epub 2019 Mar 5.

PMID: 30834959

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  1. Relationship Between Pulmonary Arterial Resistance and Compliance in Patients with Down Syndrome.

Iwaya Y, Muneuchi J, Inoue Y, Watanabe M, Okada S, Ochiai Y.

Pediatr Cardiol. 2019 Apr;40(4):841-847. doi: 10.1007/s00246-019-02080-9. Epub 2019 Mar 4.

PMID: 30830280

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  1. Usefulness of Red Cell Width Distribution (RDW) in the Assessment of Children with Pulmonary Arterial Hypertension (PAH).

Zuk M, Migdal A, Dominczak J, Brzezinska-Rajszys G.

Pediatr Cardiol. 2019 Apr;40(4):820-826. doi: 10.1007/s00246-019-02077-4. Epub 2019 Mar 4.

PMID: 30830279 Free PMC Article

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  1. Arterial Stiffness and Its Relationship to Cardiorespiratory Fitness in Children and Young Adults with a Fontan Circulation.

Noortman LCM, Haapala EA, Takken T.

Pediatr Cardiol. 2019 Apr;40(4):784-791. doi: 10.1007/s00246-019-02065-8. Epub 2019 Feb 15.

PMID: 30770935 Free PMC Article

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  1. Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience.

Wittekind SG, Ryan TD, Gao Z, Zafar F, Czosek RJ, Chin CW, Jefferies JL.

Pediatr Cardiol. 2019 Apr;40(4):694-704. doi: 10.1007/s00246-018-2043-0. Epub 2018 Dec 12.

PMID: 30542921

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  1. [Idiopathic dilated cardiomyopathy in children: Prognostic indicators].

Arı ME, Yoldaş T, Örün UA, Karademir S.

Turk Kardiyol Dern Ars. 2019 Apr;47(3):207-215. doi: 10.5543/tkda.2018.72809. Turkish.

PMID: 30982820 Free Article

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ACHD Featured Articles in association with ISACHD

ACHD Featured Articles in association with ISACHD

Adult Congenital Heart Disease Featured Reviews of April 2019 Manuscripts (sponsored by ISACHD)

 

Exercise Capacity After Repair of Ebstein Anomaly in Adults.

Morrical BD, Dearani JA, Bonnichsen CR, Taggart NW.

Pediatr Cardiol. 2019 Apr;40(4):726-732. doi: 10.1007/s00246-019-02056-9. Epub 2019 Jan 30.

PMID: 30701277

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Take Home Points:

 

  • Most patients reported symptomatic improvement after surgical repair of Ebstein anomaly – as assessed by NYHA class and self-reported symptoms.
  • Formal exercise testing did not reveal objective evidence of functional improvement.
  • Echo parameters showed improvement in terms of the degree of TR and RV size.
  • Those on betablockers at ANY time had a reduced exercise capacity as measured by METs and VO2

 

Commentary by Dr. Blanche Cupido (Cape Town), section editor of ACHD Journal Watch:  The surgical repair techniques for Ebstein anomaly have evolved over the last few decades but timing of surgery remains variable. Though patients report symptomatic improvement, there are no objective functional capacity data for those patients post-repair in adulthood. This retrospective review, from the Mayo Clinic, aimed to describe the effect of repairing or replacing the tricuspid valve (TV) in Ebstein anomaly by comparing exercise test data in adults before and after surgery.

They enrolled all patients with Ebstein anomaly who underwent tricuspid surgery at the Mayo Clinic between June 2007 and January 2015. All patients had both pre- and postoperative exercise test data and echocardiograms.

Three hundred and twenty two patients had tricuspid surgery during the study period. Of these, 32 had pre-and post-exercise tests adequate for analysis. The mean age at time of surgery was 40 years old, 69% of the cohort was female.

  • 19 patients had a ‘Cone’ repair
  • 13 had a tricuspid valve replacement
  • 26% were re-operations
  • 75% were symptomatic prior to surgery

All patients had good surgical results with the tricuspid regurgitation being significantly reduced post-operatively (77% had severe TR pre-op; 91% had no/mild TR post-op) – p<0.001. Though the RV size did not normalize, it was significantly decreased.

The NYHA class improved dramatically after surgery with 68% of patients improving to class I.

There was no significant difference in the functional aerobic capacity, metabolic equivalents, exercise time or VO2max between the pre- and post-operative exercise tests.

 

 

Beta blockers had a significant effect on net exercise performance. Those patients who were never on a betablocker before, achieved higher METS and higher VO2max than those on betablockers. Functional aerobic capacity was not affected.

The addition of betablockers did not alter pre-and post-exercise parameters. Furthermore, there was no association with pre-or post-operative arrhythmia and betablocker usage.

There was no difference in exercise parameters between those who had a repair and those who had a tricuspid valve replacement. There was no difference in exercise capacity between those with a first repair vs a re-do repair.

 

Elevated non-invasive liver fibrosis markers and risk of liver carcinoma in adult patients after repair of tetralogy of Fallot.

Yamamura K, Sakamoto I, Morihana E, Hirata Y, Nagata H, Yamasaki Y, Okumura Y, Kohashi K, Koto K, Tsutsui H, Ohga S.

Int J Cardiol. 2019 Jul 15;287:121-126. doi: 10.1016/j.ijcard.2019.04.032. Epub 2019 Apr 11.

PMID:  31006598

 

Take Home Points:

 

  • Liver fibrosis increases the risk of cardiac operation with cardiopulmonary bypass especially if it is associated with thrombocytopenia and/or coagulopathy.
  • Elevated liver fibrosis markers due to hepatic congestion is present not only in adult patients after Fontan procedure but also after TOF repair.
  • However, RV dysfunction and liver congestion could be resolved by pulmonary valve replacement in rTOF
  • Careful monitoring of the liver fibrosis markers and reoperation at an appropriate time may be beneficial in improving the long-term outcomes in rTOF patients with elevated right atrial pressure.

 

Commentary by Dr. Soha Romeih (Aswan, Egypt), section editor of ACHD Journal Watch:  In Fontan patients, the elevated systemic venous pressure leads to hepatic congestion and hepatic fibrosis. Chronic RV dysfunction and hepatic congestion develops over time and may lead to liver fibrosis, and subsequent development of liver cirrhosis late after repair of TOF (rTOF). However, the incidence and severity remain unclear.

This study aimed to elucidate the incidence and severity of liver fibrosis in patients with rTOF. 50 TOF patients with PR/PS, 50 age-matched patients after Fontan procedure, and 11 control subjects were enrolled. Patients who had other forms of liver diseases, such as viral hepatitis or alcoholic liver disease were excluded. In all patients, liver fibrosis markers (hyaluronic acid and type IV collagen), hemodynamic parameters obtained from CMR and cardiac catheterization, and abdominal ultrasonography and liver biopsy data were documented.

Hyaluronic acid levels in patients with rTOF were significantly higher than controls, and were lower than those after Fontan palliations. Type IV-collagen levels in patients with rTOF were higher than controls, and tended to be lower than those after Fontan. Patients with rTOF showed abnormal hyaluronic acid levels more frequently than controls, and less frequently than those after Fontan.

In patients with rTOF, hyaluronic acid levels correlated positively with time after repair (r= 0.39, p =0.005) and type IV collagen levels correlated positively with RA pressure (r=0.42, p= 0.007). On the other hand, in patients after Fontan, liver fibrosis markers showed no significant correlation with time after surgery or RA pressure.

Type IV-collagen levels in patients with rTOF with moderate or severe TR (n = 13) was higher than those with less than moderate TR (n = 37). Other than that, there was no difference in type IV-collagen or hyaluronic acid levels among patients with PR, PR + PS, and PS, between patients with and without moderate or severe TR or between patients with and without restrictive RV physiology.

Patients with hepatic congestion on abdominal ultrasound had significantly higher type IV collagen, higher total bilirubin, lower total protein, lower platelet count and higher RV end-diastolic pressure than those without hepatic congestion (p b 0.05, respectively)

23 patients with rTOF underwent PVR and 13 of them received the assessment of fibrosis markers after PVR. Liver fibrosis markers did not show statistically significant decrease after PVR in this small number of subjects (hyaluronic acid: median, 32 to 28 ng/mL, p = 0.588; type IV collagen: median 145 to 124 ng/mL, p =0.105). However, 12 patients (92%) and 10 patients (77%) had normalization of the hyaluronic acid and type IV collagen levels after PVR.

Elevated RA pressure and pulmonary regurgitant fraction had an association with elevated type IV collagen.

Although the predictive value of serum markers for liver fibrosis is not completely validated with comprehensive imaging tests or liver biopsy in this population, this association indicates the fibrosis markers should reflect the degree of liver fibrosis due to elevated RA pressure.

There are several limitations about this study: the study population is relatively small, especially in patients who underwent imaging tests and biopsy of the liver, cardiac catheter data in the controls and CMR data in the Fontan patients and controls were not available in the majority of subjects because of the retrospective data collection, and there was no data to investigate the relationship between elevated liver fibrosis markers and prognosis. A prospective study with a larger population is needed to determine the clinical impact of elevation of these fibrosis markers.

 

 

Heart or heart-lung transplantation for patients with congenital heart disease in England.

Dimopoulos K, Muthiah K, Alonso-Gonzalez R, Banner NR, Wort SJ, Swan L, Constantine AH, Gatzoulis MA, Diller GP, Kempny A.

Heart. 2019 Apr;105(8):596-602. doi: 10.1136/heartjnl-2018-313984. Epub 2019 Jan 12.

PMID:  30636220

 

 

Take Home Points:

 

  • Retrospective analysis of patients with congenital heart disease who underwent heart or heart-lung transplantation between 1997-2015 in England, UK.
  • Over an 18 year period, in 444 patients, there were 469 transplants – 83% heart and the remainder heart-lung.
  • The majority of first heart or heart lung transplants were performed in patients <18 yrs. old (53%). The median age at transplantation was 19.5 years (range 0-63.6 years)
  • Just over half of all transplants (54%, n=239) were in patents with complex congenital heart disease.
  • Of patients undergoing heart-lung transplantation, 92% had complex congenital heart disease. Comparatively, of those patients undergoing heart transplant alone, 46% were complex and the remainder mild or moderate.
  • Older age and heart-lung transplant were strong predictors of death.
  • Although there is an increasing need for transplantation in the congenital cardiac population there is a supply-demand mismatch, which is ever widening.

 

Commentary by Dr. Damien Cullington (Liverpool, UK), section editor of ACHD Journal Watch:  Heart failure is the leading cause of demise in the adult congenital cardiac population. Surgical, interventional, device and medical treatments have become ever more ambitious and proactively pursued both by clinical teams. Naturally, there is an ever increasing number of ACHD patients who are palliated for their condition and who eventually need assessment for their suitability for advanced heart failure therapies. The biggest difficulty we are facing is an adequate supply of donor organs for the ever increasing demand in the congenital population. In addition, for our patients with a Fontan, there is the further complex issue of the potential for heart-liver transplantation due to insidiously progressive Fontan associated liver disease.

Dimopoulos et al. published this UK retrospective analysis of the Hospital Episode Statistics database of all patients with a code for congenital heart disease who have underwent heart or heart-lung transplant between 1997-2015 in England. The baseline characteristics of the population are shown in Table 1. The annual number of heart and heart-lung transplants performed and the age distribution at the time of transplant is shown in Figure 1.

 

Transplant numbers and projections for the future

 

Figure 1 shows that following an unexplained drop in transplant numbers in 2003, there has been and increasing number of heart transplants with a declining number of heart-lung transplants. The proportion of adult-to-paediatric transplants is essentially unaltered over time. Over the next decade, it is projected that there will be an increase by about a third of the number of heart and heart-lung transplants (Figure 2A). Figure 2B demonstrates the clearly evolving supply-demand mismatch between 2004-14 of an increasing number of AHCD patients hospitalised with heart failure with no corresponding increase in the number of transplants.

Survival post transplantation (Figures 3A and 3B)

 In the combined heart and heart-lung first transplant population (n=444), over a median follow up of nearly 5 years, 29% of patients (n=130) died.  In patients who had only a heart transplant, 30 day, 1, 5 and 10 year mortality was  11%, 14%, 21% and 27% respectively. At the same time interval, for patients undergoing heart-lung transplantation, mortality was  19%, 30%, 38% and 55%. Interestingly, for patients undergoing heart transplant, the complexity of congenital heart disease had no significant influence on survival.  From the immediate time of transplant, children (<18 years old) had better survival than adults (15 year survival 67% vs 53%, p=0.01). In survivors after one year, there was no significant difference in survival between children or adults after transplant  74% vs 66%, p=0.66). Figures 3A and 3B show Kaplan-Meier curves for patients undergoing heart and heart-lung transplant from the time of transplant as baseline and 1 year following transplant as baseline.

 

 

Familial clustering of bicuspid aortic valve and its relationship with aortic dilation in first-degree relatives.

Galian-Gay L, Carro Hevia A, Teixido-Turà G, Rodríguez Palomares J, Gutiérrez-Moreno L, Maldonado G, Gonzàlez-Alujas MT, Sao-Aviles A, Gallego P, Calvo-Iglesias F, Bermejo J, Robledo-Carmona J, Sánchez V, Saura D, Sevilla T, Burillo-Sanz S, Guala A, Garcia-Dorado D, Evangelista A; BICUSPID investigators.

Heart. 2019 Apr;105(8):603-608. doi: 10.1136/heartjnl-2018-313802. Epub 2018 Oct 15.

PMID: 30322846

 

 

Take Home Points:

 

  • In this cross-sectional, multi-center, Spanish study, bicuspid aortic valve is present in about 6.4% of first degree relatives of bicuspid aortic valve patients and an affected member was identified in 14.8% of families.
  • The subtype of valve morphology did not follow a heritable pattern.
  • Of first degree relatives with trileaflet aortic valve, almost 10% had aortic dilation that was usually mild and more frequently associated with hypertension.
  • In a subset of patients with aortic dilation and trileaflet aortic valve who had CT angiography, 41% were actually found to have subtle bicuspid valve features with mini-raphe that were not obvious by echo.

 

Commentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:  In patients with bicuspid aortic valve (BAV), screening of first degree relative (FDR) for BAV is recommended by society guidelines. This is due to the observation of BAV familial clustering, which likely has polygenic inheritance with incomplete penetrance and variable expression. Less known are the inheritance patterns of BAV morphological types or aortic dilation without bicuspid valve in FDR of BAV patients.

Dr. Galian–Gay and colleagues performed a cross-sectional multi-center study and screened FDR of BAV probands who agreed to undergo echo screening to establish BAV prevalence, aortic dilation prevalence, and describe BAV morphologic type inheritance pattern in FDR of BAV patients.

Between 2012 and 2015, 852 BAV patients were identified prospectively from 8 Spanish tertiary referral centers and 256 agreed to undergo family screening.  Transthoracic echocardiography was performed on each index case and FDR participant as well as 3 generation pedigree.

Out of the 1253 FDR candidates, only 724 participated. The remaining FDR were either not available (292; 23.3%) or had died (237; 18.9%). The mean FDR age was 41.7 years and nearly half were males and 6.4% were found to have BAV. A new affected family member was identified in nearly 15% of families. FDR with BAV were more frequently men at 71.7% and more often hypertensive.

BAV was classified by morphologic subtype (fusion of the right-left cusps 73.9%, right-non cusps 23.9%, and left-non cusps 2.2%).  No significant relationship was found among probands and relatives according to BAV morphotype.

The degree of valve dysfunction was described and aortas were considered to be dilated if they were 1.96 standard deviations above the predicted diameter for particular patient. About 48% of FDR with BAV had aortic dilation compared with about 10% of FDR who had trileaflet aortic valve (TAV). Of interest, tubular ascending aorta dilation was more frequent in FDR with BAV 43.5% compared with 7.5% of FDR with TAV.  Conversely, aortic root dilation was comparable in both FDR groups (4.3% with BAV and 2.1% in TAV).

Aortic dilation ≥45 mm was present in 6.5% of FDR with BAV compared with 0.4% of FDR with TAV. When a cutoff of greater than 50 mm was used, 6% of FDR with BAV and 0% of FDR with TAV.

Notably, nearly 10% of FDR with TAV had aortic dilation, but these patients were older, more likely to be hypertensive, or have aortic regurgitation when compared to those without aortic dilation.  One subset of FDR with TAV and aortic dilation had CT angiography and 9 out 22 (41%) were actually found to have BAV with subtle cusp fusion/mini-raphe that were not seen with echo.

In this cross-sectional, multi-centered, Spanish study, the prevalence of BAV in FDR was 6.4% and a new affected member identified in 14.8% of families.  The subtype of valve morphology did not follow a heritable pattern.  Of FDR with TAV, almost 10% at aortic dilation that was usually mild and more frequently associated with hypertension.  In a subset of patients with aortic dilation and TAV who had CT angiography, 41% were actually found to have subtle bicuspid valve that was not as obvious by echo.

The findings of the study suggest that the familial clustering of BAV is not completely determined by genetic factors.  These findings also suggest that BAV morphology may be less determined by genetic factors and more likely related to epi genetic and/or environmental factors.  Ascending aorta dilation is common in BAV and often occurs in the absence of significant valve dysfunction, which bolsters the genetic/aortopathy theory, though hemodynamic causes for aortic dilation are probably also contributory.

This was a well-designed study but notable limitations include the potential selection bias of patients seen and tertiary referral centers as well as only 30% possible candidates agreeing to participate in the study.

The findings of this study probably support the recommendation to screen first-degree relatives of bicuspid aortic valve patients for bicuspid aortic valve and/or aortic dilation.

 

ACHD April 2019

 

  1. Prediction of heart failure and death in an adult population of Fontan patients.

Sieweke JT, Haghikia A, Riehle C, Klages C, Akin M, König T, Zwadlo C, Treptau J, Schäfer A, Bauersachs J, Westhoff-Bleck M.

Cardiol Young. 2019 Apr 30:1-8. doi: 10.1017/S1047951119000258. [Epub ahead of print]

PMID: 31036097

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  1. Long-term health-related burden of adult congenital heart diseases in Hong Kong.

Lee VW, Fong TM, Fung AK, Cheng FW.

J Med Econ. 2019 Apr 30:1. doi: 10.1080/13696998.2019.1613239. [Epub ahead of print]

PMID: 31038380

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  1. Prevalence and risk factors of sleep apnea in adult patients with congenital heart disease.

Harada G, Takeuchi D, Inai K, Shinohara T, Nakanishi T.

Cardiol Young. 2019 Apr 29:1-7. doi: 10.1017/S1047951119000179. [Epub ahead of print]

PMID: 31030704

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  1. Inhibition of miR-495 Improves Both Vascular Remodeling and Angiogenesis in Pulmonary Hypertension.

Fu J, Bai P, Chen Y, Yu T, Li F.

J Vasc Res. 2019 Apr 26:1-10. doi: 10.1159/000500024. [Epub ahead of print]

PMID: 31030195

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  1. Silibinin efficacy in a rat model of pulmonary arterial hypertension using monocrotaline and chronic hypoxia.

Zhang T, Kawaguchi N, Yoshihara K, Hayama E, Furutani Y, Kawaguchi K, Tanaka T, Nakanishi T.

Respir Res. 2019 Apr 25;20(1):79. doi: 10.1186/s12931-019-1041-y.

PMID: 31023308 Free Article

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  1. The bicuspid aortic valve: Is it an immunological disease process?

Howard C, Picca L, Smith T, Sharif M, Bashir M, Harky A.

J Card Surg. 2019 Apr 23. doi: 10.1111/jocs.14050. [Epub ahead of print]

PMID: 31012137

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Select item 31021973

 

  1. Right single coronary artery as an incidental finding in Takotsubo syndrome and acute heart failure: Case report and review of the literature.

Neiva J, Passos Silva M, Pires-Morais G, Dias A, Ponte M, Caeiro D, Braga JP, Ferreira N, Ribeiro V.

Rev Port Cardiol. 2019 Apr 20. pii: S0870-2551(17)30995-2. doi: 10.1016/j.repc.2018.06.013. [Epub ahead of print] Review. English, Portuguese.

PMID: 31014998 Free Article

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  1. The multiple systemic artery to pulmonary artery fistulas resulting in severe irreversible pulmonary arterial hypertension in patient with previous history of pneumothorax.

Jacheć W, Tomasik A, Kurzyna M, Pietura R, Torbicki A, Głowacki J, Nowalany-Kozielska E, Wojciechowska C.

BMC Pulm Med. 2019 Apr 16;19(1):80. doi: 10.1186/s12890-019-0832-8.

PMID: 30991994 Free PMC Article

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Select item 30813762

 

  1. Substantial Cardiovascular Morbidity in Adults With Lower-Complexity Congenital Heart Disease.

Saha P, Potiny P, Rigdon J, Morello M, Tcheandjieu C, Romfh A, Fernandes SM, McElhinney DB, Bernstein D, Lui GK, Shaw GM, Ingelsson E, Priest JR.

Circulation. 2019 Apr 16;139(16):1889-1899. doi: 10.1161/CIRCULATIONAHA.118.037064.

PMID: 30813762

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  1. Public Health Approach to Improve Outcomes for Congenital Heart Disease Across the Life Span.

Jenkins KJ, Botto LD, Correa A, Foster E, Kupiec JK, Marino BS, Oster ME, Stout KK, Honein MA.

J Am Heart Assoc. 2019 Apr 16;8(8):e009450. doi: 10.1161/JAHA.118.009450. No abstract available.

PMID: 30982389 Free Article

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Select item 30966855

 

  1. Thoracic Aortic Aneurysm Growth in Bicuspid Aortic Valve Patients: Role of Aortic Stiffness and Pulsatile Hemodynamics.

Rooprai J, Boodhwani M, Beauchesne L, Chan KL, Dennie C, Nagpal S, Messika-Zeitoun D, Coutinho T.

J Am Heart Assoc. 2019 Apr 16;8(8):e010885. doi: 10.1161/JAHA.118.010885.

PMID: 30966855 Free Article

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  1. Contraceptive Choices in the Immediate Postpartum Period in Women With Cardiac Disease.

Sobhani NC, Schultz H, Kheiwa A, Killion M, Parikh NI, Harris IS, Gonzalez JM, Agarwal A.

Am J Cardiol. 2019 Apr 15;123(8):1364-1369. doi: 10.1016/j.amjcard.2019.01.006. Epub 2019 Jan 25.

PMID: 30712772

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Select item 31045920

 

  1. Subclinical thrombus formation in bioprosthetic pulmonary valve conduits.

Jewgenow P, Schneider H, Bökenkamp R, Hörer J, Cleuziou J, Foth R, Horke AP, Eicken A, Paul T, Sigler M.

Int J Cardiol. 2019 Apr 15;281:113-118. doi: 10.1016/j.ijcard.2019.01.095. Epub 2019 Jan 30.

PMID: 30732866

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Select item 30987651

 

  1. Clinical and genetic insights into non-compaction: a meta-analysis and systematic review on 7598 individuals.

Kayvanpour E, Sedaghat-Hamedani F, Gi WT, Tugrul OF, Amr A, Haas J, Zhu F, Ehlermann P, Uhlmann L, Katus HA, Meder B.

Clin Res Cardiol. 2019 Apr 12. doi: 10.1007/s00392-019-01465-3. [Epub ahead of print]

PMID: 30980206

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Select item 30982719

 

  1. Peripartum outcomes in a large population of women with pulmonary arterial hypertension associated with congenital heart disease.

Li Q, Dimopoulos K, Liu T, Xu Z, Liu Q, Li Y, Zhang J, Gu H.

Eur J Prev Cardiol. 2019 Apr 11:2047487318821246. doi: 10.1177/2047487318821246. [Epub ahead of print]

PMID: 30971116

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Select item 31006598

 

  1. Elevated non-invasive liver fibrosis markers and risk of liver carcinoma in adult patients after repair of tetralogy of Fallot.

Yamamura K, Sakamoto I, Morihana E, Hirata Y, Nagata H, Yamasaki Y, Okumura Y, Kohashi K, Koto K, Tsutsui H, Ohga S.

Int J Cardiol. 2019 Apr 11. pii: S0167-5273(18)36764-0. doi: 10.1016/j.ijcard.2019.04.032. [Epub ahead of print]

PMID: 31006598

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Select item 30982681

 

  1. Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation.

Holbein CE, Veldtman GR, Moons P, Kovacs AH, Luyckx K, Apers S, Chidambarathanu S, Soufi A, Eriksen K, Jackson JL, Enomoto J, Fernandes SM, Johansson B, Alday L, Dellborg M, Berghammer M, Menahem S, Caruana M, Kutty S, Mackie AS, Thomet C, Budts W, White K, Sluman MA, Callus E, Cook SC, Khairy P, Cedars A; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).

Am J Cardiol. 2019 Apr 10. pii: S0002-9149(19)30398-4. doi: 10.1016/j.amjcard.2019.03.039. [Epub ahead of print]

PMID: 31030969

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Select item 30968804

 

  1. Hemodynamic Changes During Physiological and Pharmacological Stress Testing in Healthy Subjects, Aortic Stenosis and Aortic Coarctation Patients-A Systematic Review and Meta-Analysis.

Runte K, Brosien K, Salcher-Konrad M, Schubert C, Goubergrits L, Kelle S, Schubert S, Berger F, Kuehne T, Kelm M.

Front Cardiovasc Med. 2019 Apr 10;6:43. doi: 10.3389/fcvm.2019.00043. eCollection 2019.

PMID: 31024935 Free PMC Article

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Select item 30972721

 

  1. Right-sided endocarditis on Contegra tube in a complex cianotic congenital heart disease.

Olivella A, Pizzi MN, Dos-Subirà L, Aguadé-Bruix S, Fernández-Hidalgo N, Cuéllar-Calabria H, Roque A.

J Nucl Cardiol. 2019 Apr 10. doi: 10.1007/s12350-019-01708-w. [Epub ahead of print] No abstract available.

PMID: 30972721

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Select item 30950019

 

  1. New pathogenic variant of BMPR2 in pulmonary arterial hypertension.

Yang X, Kong Q, Zhao C, Cai Z, Wang M.

Cardiol Young. 2019 Apr 8:1-5. doi: 10.1017/S1047951119000015. [Epub ahead of print]

PMID: 30957726

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Select item 30957982

 

  1. Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation.

Egbe AC, Miranda WR, Pellikka PA, Pislaru SV, Borlaug BA, Kothapalli S, Ananthaneni S, Sandhyavenu H, Najam M, Farouk Abdelsamid M, Connolly HM.

Congenit Heart Dis. 2019 Apr 8. doi: 10.1111/chd.12768. [Epub ahead of print]

PMID: 30957982

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Select item 30957968

 

  1. Right ventricular and pulmonary vascular function indices for risk stratification of patients with pulmonary regurgitation.

Egbe AC, Miranda WR, Pellikka PA, Pislaru SV, Borlaug BA, Kothapalli S, Ananthaneni S, Sandhyavenu H, Najam M, Farouk Abdelsamid M, Connolly HM.

Congenit Heart Dis. 2019 Apr 8. doi: 10.1111/chd.12768. [Epub ahead of print]

PMID: 30957982

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Select item 30957968

 

  1. Right ventricular pressure overload alters cardiac lipid composition.

Koop AMC, Hagdorn QAJ, Bossers GPL, van Leusden T, Gerding A, van Weeghel M, Vaz FM, Koonen DPY, Silljé HHW, Berger RMF, Bartelds B.

Int J Cardiol. 2019 Apr 8. pii: S0167-5273(18)36438-6. doi: 10.1016/j.ijcard.2019.04.004. [Epub ahead of print]

PMID: 31003793

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Select item 30947942

 

  1. All-cause mortality and survival in adults with 22q11.2 deletion syndrome.

Van L, Heung T, Graffi J, Ng E, Malecki S, Van Mil S, Boot E, Corral M, Chow EWC, Hodgkinson KA, Silversides C, Bassett AS.

Genet Med. 2019 Apr 5. doi: 10.1038/s41436-019-0509-y. [Epub ahead of print]

PMID: 30948858

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Select item 30981569

 

  1. Do we have the ACHD physician resources we need to care for the burgeoning ACHD population?

Ezzeddine FM, Moe T, Ephrem G, Kay WA.

Congenit Heart Dis. 2019 Apr 4. doi: 10.1111/chd.12771. [Epub ahead of print]

PMID: 30945809

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Select item 31019488

 

  1. Adults With Complex Congenital Heart Disease: Cerebrovascular Considerations for the Neurologist.

Smith JMC, Andrade JG, Human D, Field TS.

Front Neurol. 2019 Apr 4;10:329. doi: 10.3389/fneur.2019.00329. eCollection 2019. Review.

PMID: 31019488 Free PMC Article

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Select item 30949869

 

  1. Subtraction of time-resolved magnetic resonance angiography images improves visualization of the pulmonary veins and left atrium in adults with congenital heart disease: a novel post-processing technique.

Sugrue G, Cradock A, McGee A, McEntee C, Eustace SK, Fitzpatrick P, Lawler LP, Murray JG.

Int J Cardiovasc Imaging. 2019 Apr 4. doi: 10.1007/s10554-019-01585-x. [Epub ahead of print]

PMID: 30949869

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Select item 31006521

 

  1. Risk factors for death or heart transplantation in single-ventricle physiology (tricuspid atresia, pulmonary atresia, and heterotaxy): A systematic review and meta-analysis.

Kulkarni A, Patel N, Singh TP, Mossialos E, Mehra MR.

J Heart Lung Transplant. 2019 Apr 4. pii: S1053-2498(19)31458-5. doi: 10.1016/j.healun.2019.04.001. [Epub ahead of print]

PMID: 31006521

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Select item 31043318

 

  1. Correction to: 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

[No authors listed]

Circulation. 2019 Apr 2;139(14):e833-e834. doi: 10.1161/CIR.0000000000000683. No abstract available.

PMID: 30933622

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Select item 30933620

 

  1. Correction to: 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

[No authors listed]

Circulation. 2019 Apr 2;139(14):e831-e832. doi: 10.1161/CIR.0000000000000684. No abstract available.

PMID: 30933620

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Select item 30586770

 

  1. Medical Therapy for Systemic Right Ventricles: A Systematic Review (Part 1) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Zaragoza-Macias E, Zaidi AN, Dendukuri N, Marelli A.

Circulation. 2019 Apr 2;139(14):e801-e813. doi: 10.1161/CIR.0000000000000604.

PMID: 30586770

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Select item 30586769

 

  1. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF.

Circulation. 2019 Apr 2;139(14):e637-e697. doi: 10.1161/CIR.0000000000000602. No abstract available. Erratum in: Circulation. 2019 Apr 2;139(14):e831-e832.

PMID: 30586768

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Select item 30586767

 

  1. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF.

Circulation. 2019 Apr 2;139(14):e698-e800. doi: 10.1161/CIR.0000000000000603. No abstract available. Erratum in: Circulation. 2019 Apr 2;139(14):e833-e834.

PMID: 30586767

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Select item 30991346

 

  1. Is there a role for autophagy in ascending aortopathy associated with tricuspid or bicuspid aortic valve?

Forte A, Cipollaro M, De Feo M, Della Corte A.

Clin Sci (Lond). 2019 Apr 2;133(7):805-819. doi: 10.1042/CS20181092. Print 2019 Apr 15. Review.

PMID: 30991346

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Select item 30121241

 

  1. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF.

J Am Coll Cardiol. 2019 Apr 2;73(12):1494-1563. doi: 10.1016/j.jacc.2018.08.1028. Epub 2018 Aug 16. No abstract available.

PMID: 30121240

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Select item 30121239

 

  1. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF.

J Am Coll Cardiol. 2019 Apr 2;73(12):e81-e192. doi: 10.1016/j.jacc.2018.08.1029. Epub 2018 Aug 16. No abstract available.

PMID: 30121239

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Select item 30121238

 

  1. Medical Therapy for Systemic Right Ventricles: A Systematic Review (Part 1) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Zaragoza-Macias E, Zaidi AN, Dendukuri N, Marelli A.

J Am Coll Cardiol. 2019 Apr 2;73(12):1564-1578. doi: 10.1016/j.jacc.2018.08.1030. Epub 2018 Aug 16.

PMID: 30121238

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Select item 30938874

 

  1. The impact of congenital heart disease on the psychological well-being and quality of life of Hong Kong Chinese adolescents: A cross-sectional study.

So SCY, Li WHC, Ho KY.

J Clin Nurs. 2019 Apr 2. doi: 10.1111/jocn.14864. [Epub ahead of print]

PMID: 30938874

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Select item 30938906

 

  1. Effects of Congenital Heart Disease Treatmenton Quality of Life.

Boukovala M, Müller J, Ewert P, Hager A.

Am J Cardiol. 2019 Apr 1;123(7):1163-1168. doi: 10.1016/j.amjcard.2018.12.048. Epub 2019 Jan 8.

PMID: 30709599

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Select item 31043224

 

  1. Large Right Ventricle Thrombus in Uhl’s Anomaly: A Rare Presentation of Extremely Rare Disease.

Singh G, Khemani H, Shaikh S, Bade A, Singla R, Bansal NO.

Cardiol Res. 2019 Apr;10(2):124-127. doi: 10.14740/cr758w. Epub 2019 Apr 11.

PMID: 31019643 Free PMC Article

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Select item 30953410

 

  1. Imaging the Lymphatic System in Fontan Patients.

Udink Ten Cate FEA, Tjwa ETTL.

Circ Cardiovasc Imaging. 2019 Apr;12(4):e008972. doi: 10.1161/CIRCIMAGING.119.008972. No abstract available.

PMID: 30943768

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Select item 30635235

 

  1. Misclassification of bicuspid aortic valves is common and varies by imaging modality and patient characteristics.

Cramer PM, Prakash SK.

Echocardiography. 2019 Apr;36(4):761-765. doi: 10.1111/echo.14295. Epub 2019 Mar 4.

PMID: 30834578

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Select item 30693549

 

  1. Big data analytics in adult congenital heart disease: why coding matters.

Asselbergs FW, Meijboom FJ.

Eur Heart J. 2019 Apr 1;40(13):1078-1080. doi: 10.1093/eurheartj/ehz089. No abstract available.

PMID: 30863860

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Select item 30689812

 

  1. Machine learning algorithms estimating prognosis and guiding therapy in adult congenital heart disease: data from a single tertiary centre including 10 019 patients.

Diller GP, Kempny A, Babu-Narayan SV, Henrichs M, Brida M, Uebing A, Lammers AE, Baumgartner H, Li W, Wort SJ, Dimopoulos K, Gatzoulis MA.

Eur Heart J. 2019 Apr 1;40(13):1069-1077. doi: 10.1093/eurheartj/ehy915.

PMID: 30689812

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Select item 30376060

 

  1. ESC Working Group Position Paper.

Chessa M, Baumgartner H, Michel-Behnke I, Berger F, Budts W, Eicken A, Søndergaard L, Stein J, Wiztsemburg M, Thomson J.

Eur Heart J. 2019 Apr 1;40(13):1043-1048. doi: 10.1093/eurheartj/ehy676. No abstract available.

PMID: 30376060

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Select item 30137263

 

  1. Pharmacological therapy in adult congenital heart disease: growing need, yet limited evidence.

Brida M, Diller GP, Nashat H, Strozzi M, Milicic D, Baumgartner H, Gatzoulis MA.

Eur Heart J. 2019 Apr 1;40(13):1049-1056. doi: 10.1093/eurheartj/ehy480.

PMID: 30137263

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Select item 30239653

 

  1. Predictors of quality of life in young adults with congenital heart disease.

Rometsch S, Greutmann M, Latal B, Bernaschina I, Knirsch W, Schaefer C, Oxenius A, Landolt MA.

Eur Heart J Qual Care Clin Outcomes. 2019 Apr 1;5(2):161-168. doi: 10.1093/ehjqcco/qcy046.

PMID: 30239653

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Select item 31043891

 

  1. Aortic morphological variability in patients with bicuspid aortic valve and aortic coarctation.

Sophocleous F, Biffi B, Milano EG, Bruse J, Caputo M, Rajakaruna C, Schievano S, Emanueli C, Bucciarelli-Ducci C, Biglino G.

Eur J Cardiothorac Surg. 2019 Apr 1;55(4):704-713. doi: 10.1093/ejcts/ezy339.

PMID: 30380029 Free PMC Article

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Select item 30991358

 

  1. X-chromosome gene dosage as a determinant of congenital malformations and of age-related comorbidity risk in patients with Turner syndrome, from childhood to early adulthood.

Fiot E, Zénaty D, Boizeau P, Haignere J, Dos Santos S, Leger J.

Eur J Endocrinol. 2019 Apr 1. pii: EJE-18-0878.R3. doi: 10.1530/EJE-18-0878. [Epub ahead of print]

PMID: 30991358

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Select item 30661159

 

  1. Transplantation: the final hurdle to longevity in patients with congenital heart disease.

Ruygrok PN.

Heart. 2019 Apr;105(8):582-583. doi: 10.1136/heartjnl-2018-314262. Epub 2019 Jan 12. No abstract available.

PMID: 30636221

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Select item 30636220

 

  1. Heart or heart-lung transplantation for patients with congenital heart disease in England.

Dimopoulos K, Muthiah K, Alonso-Gonzalez R, Banner NR, Wort SJ, Swan L, Constantine AH, Gatzoulis MA, Diller GP, Kempny A.

Heart. 2019 Apr;105(8):596-602. doi: 10.1136/heartjnl-2018-313984. Epub 2019 Jan 12.

PMID: 30636220 Free Article

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Select item 30593461

 

  1. Familial clustering of bicuspid aortic valve and its relationship with aortic dilation in first-degree relatives.

Galian-Gay L, Carro Hevia A, Teixido-Turà G, Rodríguez Palomares J, Gutiérrez-Moreno L, Maldonado G, Gonzàlez-Alujas MT, Sao-Aviles A, Gallego P, Calvo-Iglesias F, Bermejo J, Robledo-Carmona J, Sánchez V, Saura D, Sevilla T, Burillo-Sanz S, Guala A, Garcia-Dorado D, Evangelista A; BICUSPID investigators.

Heart. 2019 Apr;105(8):603-608. doi: 10.1136/heartjnl-2018-313802. Epub 2018 Oct 15.

PMID: 30322846

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Select item 30871758

 

  1. Adult Congenital Heart Disease in Australia and New Zealand: A Call for Optimal Care.

Nicolae M, Gentles T, Strange G, Tanous D, Disney P, Bullock A, Grigg L, Chen SSM, Pressley L, Cordina R, Hornung T, O’Donnell C, Celermajer DS.

Heart Lung Circ. 2019 Apr;28(4):521-529. doi: 10.1016/j.hlc.2018.10.015. Epub 2018 Oct 28. Review.

PMID: 30392984

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Select item 30318390

 

  1. Readmissions to the ICU Among Children With Tracheostomies Placed After Cardiac Arrest.

Heneghan JA, Shein SL.

Hosp Pediatr. 2019 Apr;9(4):256-264. doi: 10.1542/hpeds.2018-0269. Epub 2019 Mar 13.

PMID: 30867193

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Select item 30665803

 

  1. The outcome of adults born with pulmonary atresia: High morbidity and mortality irrespective of repair.

Montanaro C, Merola A, Kempny A, Alvarez-Alvarez B, Alonso-Gonzalez R, Swan L, Uebing A, Li W, Babu-Narayan SV, Gatzoulis MA, Dimopoulos K.

Int J Cardiol. 2019 Apr 1;280:61-66. doi: 10.1016/j.ijcard.2018.11.011. Epub 2018 Nov 7.

PMID: 30477927

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Select item 30937683

 

  1. Mid-term Clinical Outcomes in a Cohort of Asymptomatic or Mildly Symptomatic Korean Patients with Bicuspid Aortic Valve in a Tertiary Referral Hospital.

Sun BJ, Oh JK, Lee SH, Jang JY, Lee JH, Lee S, Kim DH, Song JM, Kang DH, Song JK.

J Cardiovasc Imaging. 2019 Apr;27(2):105-118. doi: 10.4250/jcvi.2019.27.e19.

PMID: 30993945 Free PMC Article

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Select item 30676496

 

  1. Improving health perception through a transition care model for adolescents with congenital heart disease.

Flocco SF, Dellafiore F, Caruso R, Giamberti A, Micheletti A, Negura DG, Piazza L, Carminati M, Chessa M.

J Cardiovasc Med (Hagerstown). 2019 Apr;20(4):253-260. doi: 10.2459/JCM.0000000000000770.

PMID: 30676496

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Select item 29786408

 

  1. Management of Coarctation of The Aorta in Adult Patients: State of The Art.

Alkashkari W, Albugami S, Hijazi ZM.

Korean Circ J. 2019 Apr;49(4):298-313. doi: 10.4070/kcj.2018.0433. Review.

PMID: 30895757 Free PMC Article

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Select item 30808079

 

  1. Body mass index stratification in hospitalized Italian adults with congenital heart disease in relation to complexity, diagnosis, sex and age.

Malavazos AE, Capitanio G, Chessa M, Matelloni IA, Milani V, Stella E, Al Kassem LF, Sironi F, Boveri S, Giamberti A, Masocco M, Ranucci M, Menicanti L, Morricone L.

Nutr Metab Cardiovasc Dis. 2019 Apr;29(4):367-377. doi: 10.1016/j.numecd.2019.01.009. Epub 2019 Jan 29.

PMID: 30795994

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Select item 30983902

 

  1. High-risk pregnancy in a patient with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) with temporary shunt inversion and deoxygenation.

Hohmann C, Dumitrescu D, Gerhardt F, Kramer T, Rosenkranz S, Huntgeburth M.

Pulm Circ. 2019 Apr-Jun;9(2):2045894019835649. doi: 10.1177/2045894019835649.

PMID: 30767601 Free PMC Article

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Select item 30857977

 

  1. Advanced Heart Failure in Congenital Heart Disease: Role of Heart Transplant and Ventricular Assist Devices.

Alonso-Gonzalez R.

Rev Esp Cardiol (Engl Ed). 2019 Apr;72(4):285-287. doi: 10.1016/j.rec.2018.09.018. Epub 2019 Mar 8. English, Spanish. No abstract available.

PMID: 30857977

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Select item 30957130

 

  1. Management of high risk cardiac conditions in pregnancy: Anticoagulation, severe stenotic valvular disease and cardiomyopathy.

Lau E, DeFaria Yeh D.

Trends Cardiovasc Med. 2019 Apr;29(3):155-161. doi: 10.1016/j.tcm.2018.07.009. Epub 2018 Jul 22. Review.

PMID: 30104111

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Select item 30739593

 

  1. High incidence and clinical characteristics of fibromuscular dysplasia in patients with spontaneous cervical artery dissection: The ARCADIA-POL study.

Talarowska P, Dobrowolski P, Klisiewicz A, Kostera-Pruszczyk A, Członkowska A, Kurkowska-Jastrzębska I, Gąsecki D, Warchoł-Celińska E, Światłowski Ł, Florczak E, Januszewicz M, Michałowska I, Józwik-Plebanek K, Szczudlik P, Błażejewska-Hyżorek B, Protasiewicz M, Odrowąż-Pieniążek P, Tekieli Ł, Michel-Rowicka K, Hanus K, Widecka K, Sołtysiak M, Tykarski A, Stryczyński Ł, Szczerbo-Trojanowska M, Hoffman P, Prejbisz A, Januszewicz A.

Vasc Med. 2019 Apr;24(2):112-119. doi: 10.1177/1358863X18811596. Epub 2019 Feb 10.

PMID: 30739593

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Select item 30826104

 

  1. Altered brain diffusion tensor imaging indices in adolescents with the Fontan palliation.

Singh S, Roy B, Pike N, Daniel E, Ehlert L, Lewis AB, Halnon N, Woo MA, Kumar R.

Neuroradiology. 2019 Apr 30. doi: 10.1007/s00234-019-02208-x. [Epub ahead of print]

PMID: 31041457

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Select item 31041461

 

  1. Prevalence and risk factors of sleep apnea in adult patients with congenital heart disease.

Harada G, Takeuchi D, Inai K, Shinohara T, Nakanishi T.

Cardiol Young. 2019 Apr 29:1-7. doi: 10.1017/S1047951119000179. [Epub ahead of print]

PMID: 31030704

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Select item 31033192

 

  1. LRP1 Deficiency in Vascular SMC Leads To Pulmonary Arterial Hypertension That Is Reversed By PPARγ Activation.

Calvier L, Boucher P, Herz J, Hansmann G.

Circ Res. 2019 Apr 26. doi: 10.1161/CIRCRESAHA.119.315088. [Epub ahead of print]

PMID: 31023188

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Select item 31026225

 

  1. Platypnea-Orthodeoxia Syndrome Due to Atrial Septal Defect and Combined Thoracic Deformities in a Young Woman.

Asano R, Fukui S, Nishii T, Konagai N, Hasegawa T, Kitano M, Ogo T, Yasuda S.

Circ J. 2019 Apr 25;83(5):1080. doi: 10.1253/circj.CJ-18-0750. Epub 2018 Sep 20. No abstract available.

PMID: 30232293 Free Article

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Select item 31030099

 

  1. Aspernolide F, as a new cardioprotective butyrolactone against doxorubicin-induced cardiotoxicity.

El-Agamy DS, Ibrahim SRM, Ahmed N, Khoshhal S, Abo-Haded HM, Elkablawy MA, Aljuhani N, Mohamed GA.

Int Immunopharmacol. 2019 Apr 25;72:429-436. doi: 10.1016/j.intimp.2019.04.045. [Epub ahead of print]

PMID: 31030099

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Select item 31023305

 

  1. Fully automated quantification of biventricular volumes and function in cardiovascular magnetic resonance: applicability to clinical routine settings.

Backhaus SJ, Staab W, Steinmetz M, Ritter CO, Lotz J, Hasenfuß G, Schuster A, Kowallick JT.

J Cardiovasc Magn Reson. 2019 Apr 25;21(1):24. doi: 10.1186/s12968-019-0532-9.

PMID: 31023305 Free Article

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Select item 31023308

 

  1. Silibinin efficacy in a rat model of pulmonary arterial hypertension using monocrotaline and chronic hypoxia.

Zhang T, Kawaguchi N, Yoshihara K, Hayama E, Furutani Y, Kawaguchi K, Tanaka T, Nakanishi T.

Respir Res. 2019 Apr 25;20(1):79. doi: 10.1186/s12931-019-1041-y.

PMID: 31023308 Free Article

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Select item 31021025

 

  1. Myocardial Ischemia and Anomalous Origin of the Right Coronary Artery From the Pulmonary Artery in the Adult: Management Implications and Follow-Up.

VanLoozen D, Bykhovsky MR, Kapoor D, Bates WB, Murdison KA, Polimenakos AC.

World J Pediatr Congenit Heart Surg. 2019 Apr 24:2150135118816291. doi: 10.1177/2150135118816291. [Epub ahead of print]

PMID: 31018762

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Select item 31018755

 

  1. Tetralogy of Fallot with absent pulmonary valve-When the ductus is present: A case of isolated branch pulmonary artery and review of literature.

Rao S, Najm HK, Stewart RD, Ahmad M, Erenberg F, Yaman M.

Echocardiography. 2019 Apr 22. doi: 10.1111/echo.14334. [Epub ahead of print]

PMID: 31006901

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  1. Human genotyping and an experimental model reveal NPR-C as a possible contributor to morbidity in coarctation of the aorta.

LaDisa JF Jr, Tomita-Mitchell A, Stamm KD, Bazan K, Mahnke DK, Goetsch MA, Wegter BJ, Gerringer JW, Repp K, Palygin O, Zietara AP, Krolikowski MM, Eddinger TJ, Alli AA, Mitchell ME.

Physiol Genomics. 2019 Apr 19. doi: 10.1152/physiolgenomics.00049.2018. [Epub ahead of print]

PMID: 31002586

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  1. Focal scar and diffuse myocardial fibrosis are independent imaging markers in repaired tetralogy of Fallot.

Cochet H, Iriart X, Allain-Nicolaï A, Camaioni C, Sridi S, Nivet H, Fournier E, Dinet ML, Jalal Z, Laurent F, Montaudon M, Thambo JB.

Eur Heart J Cardiovasc Imaging. 2019 Apr 16. pii: jez068. doi: 10.1093/ehjci/jez068. [Epub ahead of print]

PMID: 30993335

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  1. Erratum to: Physical Exercise Is Safe for Fontan Patients in Hypoxia.

Härtel JA, Müller N, Manunzio U, Manunzio C, De Marées M, Wolber M, Leibold D, Jung T, Breuer J, Herberg U.

Thorac Cardiovasc Surg. 2019 Apr 15. doi: 10.1055/s-0039-1685202. [Epub ahead of print] No abstract available.

PMID: 30986865

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  1. Peripartum outcomes in a large population of women with pulmonary arterial hypertension associated with congenital heart disease.

Li Q, Dimopoulos K, Liu T, Xu Z, Liu Q, Li Y, Zhang J, Gu H.

Eur J Prev Cardiol. 2019 Apr 11:2047487318821246. doi: 10.1177/2047487318821246. [Epub ahead of print]

PMID: 30971116

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  1. Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation.

Holbein CE, Veldtman GR, Moons P, Kovacs AH, Luyckx K, Apers S, Chidambarathanu S, Soufi A, Eriksen K, Jackson JL, Enomoto J, Fernandes SM, Johansson B, Alday L, Dellborg M, Berghammer M, Menahem S, Caruana M, Kutty S, Mackie AS, Thomet C, Budts W, White K, Sluman MA, Callus E, Cook SC, Khairy P, Cedars A; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).

Am J Cardiol. 2019 Apr 10. pii: S0002-9149(19)30398-4. doi: 10.1016/j.amjcard.2019.03.039. [Epub ahead of print]

PMID: 31030969

 

  1. Left Ventricular Noncompaction: Phenotype in an Integrated Model of Cardiomyopathy?

Oechslin E, Klaassen S.

J Am Coll Cardiol. 2019 Apr 9;73(13):1612-1615. doi: 10.1016/j.jacc.2018.11.064. No abstract available.

PMID: 30947912

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  1. Loss of Endothelium-Derived Wnt5a Is Associated With Reduced Pericyte Recruitment and Small Vessel Loss in Pulmonary Arterial Hypertension.

Yuan K, Shamskhou EA, Orcholski ME, Nathan A, Reddy S, Honda H, Mani V, Zeng Y, Ozen MO, Wang L, Demirci U, Tian W, Nicolls MR, de Jesus Perez VA.

Circulation. 2019 Apr 2;139(14):1710-1724. doi: 10.1161/CIRCULATIONAHA.118.037642.

PMID: 30586764

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  1. Impact of Treatment Modality on Vascular Function in Coarctation of the Aorta: The LOVE – COARCT Study.

Martins JD, Zachariah J, Selamet Tierney ES, Truong U, Morris SA, Kutty S, de Ferranti SD, Guarino M, Thomas B, Oliveira D, Marinho A, António M, Gauvreau K, Jalles N, Geva T, Carmo MM, Prakash A; LOVE‐COARCT Study.

J Am Heart Assoc. 2019 Apr 2;8(7):e011536. doi: 10.1161/JAHA.118.011536.

PMID: 30929556 Free Article

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  1. Importance of cardiovascular examination in patients with multiple lentigines: two cases of LEOPARD syndrome with hypertrophic cardiomyopathy.

Jurko T, Jurko A Jr, Krsiakova J, Jurko A, Minarik M, Mestanik M.

Acta Clin Belg. 2019 Apr;74(2):82-85. doi: 10.1080/17843286.2018.1467531. Epub 2018 May 2.

PMID: 29717636

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  1. The Natural and Unnatural History of Congenital Aortic Arch Abnormalities Evaluated in an Adult Survival Cohort.

Lodeweges JE, Dikkers FG, Mulder BJM, Roos-Hesselink JW, Vliegen HW, van Dijk APJ, Sieswerda GT, Konings TC, Berger RMF, Slebos DJ, Ebels T, van Melle JP.

Can J Cardiol. 2019 Apr;35(4):438-445. doi: 10.1016/j.cjca.2018.12.004. Epub 2018 Dec 7.

PMID: 30935634

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  1. Noncompaction-like membranous structure in left ventricle.

Ceylan O, Arman D, Kara N.

Echocardiography. 2019 Apr;36(4):813-814. doi: 10.1111/echo.14294. Epub 2019 Feb 25.

PMID: 30803005

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  1. Predictors of quality of life in young adults with congenital heart disease.

Rometsch S, Greutmann M, Latal B, Bernaschina I, Knirsch W, Schaefer C, Oxenius A, Landolt MA.

Eur Heart J Qual Care Clin Outcomes. 2019 Apr 1;5(2):161-168. doi: 10.1093/ehjqcco/qcy046.

PMID: 30239653

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  1. The Role of Matrix Metalloproteinases and Tissue Inhibitors of Metalloproteinases in Duchenne Muscular Dystrophy Cardiomyopathy.

Soslow JH, Xu M, Slaughter JC, Crum K, Chew JD, Burnette WB, Su YR, Tomasek K, Parra DA, Markham LW.

J Card Fail. 2019 Apr;25(4):259-267. doi: 10.1016/j.cardfail.2019.02.006. Epub 2019 Feb 11.

PMID: 30763738

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  1. Exercise Capacity After Repair of Ebstein Anomaly in Adults.

Morrical BD, Dearani JA, Bonnichsen CR, Taggart NW.

Pediatr Cardiol. 2019 Apr;40(4):726-732. doi: 10.1007/s00246-019-02056-9. Epub 2019 Jan 30.

PMID: 30701277

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ACHD Electrophysiology Featured Articles

ACHD Electrophysiology

 

Congenital Heart and Pediatric EP Featured Manuscripts of April 2019

 

Catheter Ablation for Atrial Tachycardia in Adults With Congenital Heart Disease: Electrophysiological Predictors of Acute Procedural Success and Post-Procedure Atrial Tachycardia Recurrence.

Grubb CS, Lewis M, Whang W, Biviano A, Hickey K, Rosenbaum M, Garan H.

JACC Clin Electrophysiol. 2019 Apr;5(4):438-447. doi: 10.1016/j.jacep.2018.10.011. Epub 2019 Jan 30.

PMID: 31000097 Free Article

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Take Home Points:

 

  • Atrial tachycardia is a common complication in adults with congenital heart disease leading to increased morbidity and mortality.
  • The majority of atrial tachycardia is macro reentrant (93%) followed by focal (7%).
  • Nearly half (45%) had typical cavo-tricuspid isthmus (CTI) flutter.
  • Acute procedural success for CTI dependent flutter was high at 98.4% but success for > 1 arrhythmia mechanism or a single complex mechanism was only 80%.
  • Only a single arrhythmia mechanism was associated with acute procedural success
  • Recurrence after the first ablation procedure was common (44%) with a median time to recurrence of ~4 years. Recurrence after the second procedure was common (55%) with a median time to recurrence of ~4.7 years.
  • Recurrence was often due to different atrial tachycardia (52%).
  • Presence of prior maze and failed initial procedure were associated with recurrence of atrial tachycardia .
  • Improvements in surgical and catheter-based ablation will likely improve acute and long-term outcomes in ACHD patients but there may be a continued need for repeat procedures to disease evolution over time.

 

Comment from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch  Atrial tachycardias are a common complication in patients with congenital heart disease. These include atrial fibrillation, focal atrial tachycardias, and macro reentrant atrial tachycardia (flutter/IART).  Presence of atrial fibrillation/atrial flutter/IART have been shown to increase morbidity (heart failure, stroke) and mortality. This has resulted in the increased use of catheter ablation as primary therapy due to the long-term side effects and efficacy of medical management. The overall acute success rates have improved over time and reported to be between 70-90% but recurrence rates continue to be high (40-60%). The predictors for acute and long-term success are limited and the focus has been on IART. This single center study (Columbia University, NY) aimed to describe the spectrum of atrial tachycardia referred for ablation, determine predictors for acute success, and determine risk factors for atrial tachycardia free survival in adult patients with congenital heart disease.

This was a retrospective single tertiary center study of all consecutive patients with congenital heart disease who underwent ablation procedure for atrial tachycardia from December 2005 to July 2017.  Subjects were excluded if the primary indication for the procedure was for SVT due to AV node reentry, SVT due to accessory pathway, or VT. Baseline clinical, electrocardiographic, ablation and mapping data were included.

Electrophysiology studies were conducted using a standard approach and 3-D electroanatomic mapping in all patients. Irrigated (open/closed) or non-irrigated radiofrequency ablation catheters were used for all cases. For irrigated catheters, temperature and power were limited to 43-45°C and 25 to 40 W. For non-irrigated catheters, the temperature and power were limited to 60 °C and 50 W. Pulmonary vein isolation for atrial fibrillation ablation was not performed in these patients and cardioversion was used to convert sustained episodes.

Ablation success was defined as termination of all atrial tachycardias during radiofrequency ablation and non-inducibility with programmed stimulation after ablation. For patients with macro-reentrant atrial tachycardia with a critical isthmus, bidirectional conduction block was confirmed.

The study group included 140 patients (51% male) with a mean age of 45.0 ± 1.2  years. The most common diagnoses were atrial septal/ventricular septal defects (31%), tetralogy of Fallot  (27%), Fontan (14%), Mustard/Senning (9%), Aortic Stenosis/BAV (5%), AV Canal (5%), Ebstein (2%), and other (6%).  Medications included beta-blockers (57%), amiodarone (16%), sotalol (10%), calcium channel blockers (6%), sodium channel blockers (1%).  Prior surgical maze was done in 6%.  Prior cardioversion was required in 39%.

The baseline cardiac rhythm was atrial tachycardia in 66%.   There was no mention of prior atrial fibrillation.  The atrial tachycardia cycle length was 275 ± 5 msec . There were 180 atrial tachycardias (spontaneous or induced) noted in 140 patients. 23% had 2 mechanisms and 3% had > 2 mechanisms. 93% (n=170) were macro-reentry and 7% (n=12) were focal.  Of the macro-reentry arrhythmia types, 84 (49%) were cavotricuspid isthmus (CTI)-dependent and 19 (11%)  were mitral isthmusdependent.  45% of patients had  CTI dependent flutter as the only mechanism.

Acute success was seen in 124 (89%) of the overall cohort. Acute success for CTI dependent flutter was high at 98.4% but the success for > 1 mechanism or complex single mechanism was lower at 80%. On multivariate analysis, presence of a single arrhythmia mechanism was associated with acute procedural success (HR 0.27, p=0.03) but not CTI dependent flutter.   On multivariate analysis, presence of a Senning/Mustard (HR 4.46, p=0.05)  and non CTI-Dependent flutter (HR 3.74, p=0.04) were associated with procedural failure. See Figure Below.

 

Atrial tachycardia recurrence after the first procedure was common and seen in 44% with a median time to recurrence of 49.9 months (IQR: 6.7 -73.7). See Figure Below.

Recurrence occurred in 40% of biventricular patients and 65% of Fontan patients. Recurrence occurred much sooner in Fontan than non-Fontan patients (12.5 vs. 50.1 months, p=0.01). On multivariate analysis,  prior surgical maze (HR3.34, p=0.03) and lack of acute procedural success at first ablation (HR 2.16, p=0.04) were associated with recurrence. See Figure Below.

 

A second procedure was attempted in 68% of patients who had recurrence.  The baseline cardiac rhythm was atrial tachycardia in 69%.   There was no mention of prior atrial fibrillation. There were 57 atrial tachycardias (spontaneous or induced) noted in 42 patients. Of these patients, 52% had a new atrial tachycardia. 93% (n=53) were macro-reentry and 7% (n=4) were focal.

Recurrence after the second procedure was seen in 55% with a median time to recurrence of 56.0 months (IQR: 22.0 – 90.9). See Figure Below.

Catheter ablation for atrial tachycardias in ACHD patients was safe in this cohort. The incidence of major complications was low at 1.4% (n=2). There was one patient with post-procedure AV block requiring a pacemaker and one patient who died of an acute stroke 1 month after procedure.

Of note, the overall incidence of death or transplant in this cohort was significant at 7% over  the 8 year study period. 8 patients died a median of 9.2 months after the procedure and 2 had heart transplant. There are no details of who these patients are but this is consistent with known data on atrial arrhythmias and morbidity and mortality in ACHD patients.  It would be important with the authors detailed if these patients were procedural success or failure, the arrhythmia mechanisms noted, and if they had recurrence despite ablation attempts.

This study demonstrates that the acute success rates for atrial tachycardias are comparable to published data at 89%  and the majority are macro-reentrant.   Risk factors for acute procedural failure were noted to be Mustard/Senning patients and non-CTI dependent IART. As other studies have demonstrated, Mustard/Senning patients pose technical challenges for ablation but success can be improved with ablation techniques and approach.  In addition, this patient cohort will continue to shrink with the advent of the arterial switch operation as demonstrated by this cohort (9%).

The risk of recurrence as expected is dependent on acute procedural success. However, like other recent studies have shown,  the majority of recurrence was due to new arrhythmia mechanisms.  The use of ablation and need for repeat procedures may be common but long term success without the need for chronic antiarrhythmic medications may be achievable in this relatively young adult old cohort.  Of note, this study did not assess the impact of atrial fibrillation which is important.  Other recent studies have shown this to be a risk factor for acute procedural failure and recurrence.  It is unclear what portion of the cohort had atrial fibrillation but 39% required cardioversion. The impact of this arrhythmia on acute procedural success and recurrence would be important.  In particular, there was an acute stroke noted in one patient 1 month after an ablation that resulted in death. Overall, this studies adds to the growing data that ablation can be successful,  but recurrence is common and repeat procedures are often needed.  With continued advances in mapping and ablation strategies for catheter and surgical approaches,  hopefully there can be continued improvements in the acute and long-term success of ablation for atrial arrhythmias in ACHD patients.

 

CHD EP April 2019

 

  1. Radiofrequency ablation of the cavotricuspid isthmus for management of atrial flutter in patients with congenital heart disease after tricuspid valve surgery: A single Center experience.

Kella DK, Yasin OZ, Isath AM, McLeod CJ, Canon B, Asirvatham SJ, Wackel PL.

Heart Rhythm. 2019 Apr 29. pii: S1547-5271(19)30428-X. doi: 10.1016/j.hrthm.2019.04.045. [Epub ahead of print]

PMID: 31048063

 

Select item 31029153

 

  1. Late complete heart block post-tetralogy of Fallot repair: a possible new predicting, precipitating factor and review of related cases.

Hejazi R, Balubaid M, Alata J, Waggass R.

BMJ Case Rep. 2019 Apr 24;12(4). pii: e228642. doi: 10.1136/bcr-2018-228642.

PMID: 31023735

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  1. Causes of fetal third-degree atrioventricular block and use of hydroxychloroquine in pregnant women with Ro/La antibodies.

Mollerach FB, Scolnik M, Catoggio LJ, Rosa J, Soriano ER.

Clin Rheumatol. 2019 Apr 17. doi: 10.1007/s10067-019-04556-8. [Epub ahead of print]

PMID: 30997589

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  1. Prediction and prevention of sudden cardiac death in transposition of the great arteries: A step closer.

Kharbanda RK, de Groot NMS.

Int J Cardiol. 2019 Apr 17. pii: S0167-5273(19)31829-7. doi: 10.1016/j.ijcard.2019.04.040. [Epub ahead of print] No abstract available.

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  1. Sudden Death in Athletes.

Farzam K, Ahmad T.

StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.

2019 Apr 2.

PMID: 30969530 Free Books & Documents

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  1. Cardiac implantable electronic devices in adults with tetralogy of Fallot.

Egbe AC, Miranda WR, Madhavan M, Ammash NM, Missula VR, Al-Otaibi M, Fatola A, Kothapalli S, Connolly HM.

Heart. 2019 Apr;105(7):538-544. doi: 10.1136/heartjnl-2018-314072. Epub 2018 Oct 30.

PMID: 30377259

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  1. Predictive value of P-wave and QT interval dispersion in children with congenital heart disease and pulmonary arterial hypertension for the occurrence of arrhythmias.

Saleh A, Shabana A, El Amrousy D, Zoair A.

J Saudi Heart Assoc. 2019 Apr;31(2):57-63. doi: 10.1016/j.jsha.2018.11.006. Epub 2018 Dec 1.

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PMID: 31000097 Free Article

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  1. Sinus node dysfunction in patients with Fontan circulation: could heart rate variability be a predictor for pacemaker implantation?

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Chen JCY, Corban MT, Wackel PL, Van Dorn CS, Dearani JA, Sinak LJ.

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PMID: 30298833 Free Article

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Rijnberg FM, Blom NA, Sojak V, Bruggemans EF, Kuipers IM, Rammeloo LAJ, Jongbloed MRM, Bouma BJ, Hazekamp MG.

Interact Cardiovasc Thorac Surg. 2019 Apr 30. pii: ivz111. doi: 10.1093/icvts/ivz111. [Epub ahead of print]

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  1. What is the optimal remote monitoring schedule in the pediatric patients with cardiovascular implantable electronic devices (CIEDs)?

Tsao S.

Pacing Clin Electrophysiol. 2019 Apr 29. doi: 10.1111/pace.13706. [Epub ahead of print] No abstract available.

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  1. Intramyocardial Hematoma After Radiofrequency Catheter Ablation.

Chen JCY, Corban MT, Wackel PL, Van Dorn CS, Dearani JA, Sinak LJ.

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BMC Pediatr. 2019 Apr 24;19(1):124. doi: 10.1186/s12887-019-1494-7.

PMID: 31018851 Free PMC Article

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Circulation. 2019 Apr 22. doi: 10.1161/CIRCULATIONAHA.118.039048. [Epub ahead of print]

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  1. Increased QT and P-wave dispersion during attack-free period in pediatric patients with migraine attacks.

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Biendarra-Tiegs SM, Li X, Ye D, Brandt EB, Ackerman MJ, Nelson TJ.

Stem Cells Dev. 2019 Apr 17. doi: 10.1089/scd.2019.0030. [Epub ahead of print]

PMID: 30892143

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  1. Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome.

Michowitz Y, Milman A, Andorin A, Sarquella-Brugada G, Gonzalez Corcia MC, Gourraud JB, Conte G, Sacher F, Juang JJM, Kim SH, Leshem E, Mabo P, Postema PG, Hochstadt A, Wijeyeratne YD, Denjoy I, Giustetto C, Mizusawa Y, Huang Z, Jespersen CH, Maeda S, Takahashi Y, Kamakura T, Aiba T, Arbelo E, Mazzanti A, Allocca G, Brugada R, Casado-Arroyo R, Champagne J, Priori SG, Veltmann C, Delise P, Corrado D, Brugada J, Kusano KF, Hirao K, Calo L, Takagi M, Tfelt-Hansen J, Yan GX, Gaita F, Leenhardt A, Behr ER, Wilde AAM, Nam GB, Brugada P, Probst V, Belhassen B.

J Am Coll Cardiol. 2019 Apr 16;73(14):1756-1765. doi: 10.1016/j.jacc.2019.01.048.

PMID: 30975291

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  1. Incidentally identified genetic variants in arrhythmogenic right ventricular cardiomyopathy-associated genes among children undergoing exome sequencing reflect healthy population variation.

Headrick AT, Rosenfeld JA, Yang Y, Tunuguntla H, Allen HD, Penny DJ, Kim JJ, Landstrom AP.

Mol Genet Genomic Med. 2019 Apr 15:e593. doi: 10.1002/mgg3.593. [Epub ahead of print]

PMID: 30985088 Free Article

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  1. Utility and safety of adenosine challenge for subtle ventricular pre-excitation in the pediatric population.

Follansbee CW, Beerman LB, Wu L, Bertolet M, Arora G.

J Cardiovasc Electrophysiol. 2019 Apr 2. doi: 10.1111/jce.13935. [Epub ahead of print]

PMID: 30938913

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  1. [A long-term follow-up study of cardiac resynchronization therapy for children with right ventricle-paced heart failure].

Liu T, Liang DP, Zhang ZW, Wang SS, Li YF, Zhong CY, Zeng SY.

Zhonghua Er Ke Za Zhi. 2019 Apr 2;57(4):281-285. doi: 10.3760/cma.j.issn.0578-1310.2019.04.010. Chinese.

PMID: 30934201

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  1. [Clinical characteristics and treatment of congenital long QT syndrome in 58 children].

Ge HY, Li XM, Jiang H, Li MT, Zhang Y, Liu HJ.

Zhonghua Er Ke Za Zhi. 2019 Apr 2;57(4):272-276. doi: 10.3760/cma.j.issn.0578-1310.2019.04.008. Chinese.

PMID: 30934199

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  1. The low specificity of low voltage bridges associating atrioventricular nodal reentry in pediatric patients.

Marshall AM, Erickson CC, Danford DA, Kugler JD, Thomas VC.

J Interv Card Electrophysiol. 2019 Apr;54(3):277-281. doi: 10.1007/s10840-018-0382-7. Epub 2018 Jul 21.

PMID: 30032471

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  1. CAP2 mutation leads to impaired actin dynamics and associates with supraventricular tachycardia and dilated cardiomyopathy.

Aspit L, Levitas A, Etzion S, Krymko H, Slanovic L, Zarivach R, Etzion Y, Parvari R.

J Med Genet. 2019 Apr;56(4):228-235. doi: 10.1136/jmedgenet-2018-105498. Epub 2018 Dec 5.

PMID: 30518548

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  1. Localization of Accessory Pathways in Pediatric Patients With Wolff-Parkinson-White Syndrome Using 3D-Rendered Electromechanical Wave Imaging.

Melki L, Grubb CS, Weber R, Nauleau P, Garan H, Wan E, Silver ES, Liberman L, Konofagou EE.

JACC Clin Electrophysiol. 2019 Apr;5(4):427-437. doi: 10.1016/j.jacep.2018.12.001. Epub 2019 Jan 30.

PMID: 31000096

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  1. Electroanatomic mapping-guided catheter ablation of atrial tachycardia in children with limited/zero fluoroscopy.

Elkiran O, Akdeniz C, Karacan M, Tuzcu V.

Pacing Clin Electrophysiol. 2019 Apr;42(4):453-457. doi: 10.1111/pace.13619. Epub 2019 Feb 21.

PMID: 30740745

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  1. Ambulatory Arrhythmia Detection with ZIO® XT Patch in Pediatric Patients: A Comparison of Devices.

Pradhan S, Robinson JA, Shivapour JK, Snyder CS.

Pediatr Cardiol. 2019 Apr 1. doi: 10.1007/s00246-019-02089-0. [Epub ahead of print]

PMID: 30937502

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  1. Sinus node dysfunction in patients with Fontan circulation: could heart rate variability be a predictor for pacemaker implantation?

Dahlqvist JA, Wiklund U, Karlsson M, Hanséus K, Strömvall-Larsson E, Nygren A, Eliasson H, Rydberg A.

Pediatr Cardiol. 2019 Apr;40(4):685-693. doi: 10.1007/s00246-019-02092-5. Epub 2019 Mar 27. Review.

PMID: 30918992 Free PMC Article

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  1. Computational Identification of Ventricular Arrhythmia Risk in Pediatric Myocarditis.

Cartoski MJ, Nikolov PP, Prakosa A, Boyle PM, Spevak PJ, Trayanova NA.

Pediatr Cardiol. 2019 Apr;40(4):857-864. doi: 10.1007/s00246-019-02082-7. Epub 2019 Mar 6.

PMID: 30840104

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  1. Transseptal Puncture for Catheter Ablation in Children.

Koca S, Akdeniz C, Tuzcu V.

Pediatr Cardiol. 2019 Apr;40(4):799-804. doi: 10.1007/s00246-019-02069-4. Epub 2019 Feb 7.

PMID: 30729261

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  1. Usefulness of Electroanatomical Mapping with Contact Force Monitoring for Accessory Pathways Ablation in Pediatric Population.

Gulletta S, Vergara P, Gigli L, D’Angelo G, Radinovic A, Melania Barbaro C, Trevisi N, Della Bella P.

Pediatr Cardiol. 2019 Apr;40(4):713-718. doi: 10.1007/s00246-019-02051-0. Epub 2019 Jan 21.

PMID: 30666358

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ACHD Interventional Cardiology Featured Articles

ACHD Interventions

 

CHD Interventions April 2019

 

  1. Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics.

Silva-Sepulveda JA, Fonseca Y, Vodkin I, Vaughn G, Newbury R, Vavinskaya V, Dwek J, Perry JC, Reshamwala P, Baehling C, Lyon J, Davis C, Lee JW, El-Sabrout H, Shahbah D, Alshawabkeh L, Moore JW, El-Said H.

Congenit Heart Dis. 2019 Apr 30. doi: 10.1111/chd.12770. [Epub ahead of print]

PMID: 31038848

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Select item 31038380

 

  1. Congenital Coronary Aneurysm: Unusual Presentation of Myocardial Ischemia in Children.

Simry W, Al Kindi H, Sedky Y, Kaml H, Romaih S, Van Doorn C.

World J Pediatr Congenit Heart Surg. 2019 Apr 25:2150135118809078. doi: 10.1177/2150135118809078. [Epub ahead of print]

PMID: 31023146

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Select item 31023735

 

  1. Percutaneous axillary artery approach for ductal stenting in critical right ventricular outflow tract lesions in the neonatal period.

Breatnach CR, Aggarwal V, Al-Alawi K, McMahon CJ, Franklin O, Prendiville T, Oslizlok P, Walsh K, Qureshi AM, Kenny D.

Catheter Cardiovasc Interv. 2019 Apr 24. doi: 10.1002/ccd.28302. [Epub ahead of print]

PMID: 31020799

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Select item 31017617

 

  1. Medium- and long-term follow-up of transcatheter closure of ruptured sinus of Valsalva aneurysm in Central Europe population.

Galeczka M, Glowacki J, Yashchuk N, Ditkivskyy I, Rojczyk D, Knop M, Smerdzinski S, Cherpak B, Szkutnik M, Bialkowski J, Fiszer R, Lazoryshynets V.

J Cardiol. 2019 Apr 22. pii: S0914-5087(19)30084-X. doi: 10.1016/j.jjcc.2019.03.012. [Epub ahead of print]

PMID: 31023567

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Select item 31007148

 

  1. Acute and midterm outcomes of the post-approval MELODY Registry: a multicentre registry of transcatheter pulmonary valve implantation.

Nordmeyer J, Ewert P, Gewillig M, AlJufan M, Carminati M, Kretschmar O, Uebing A, Dähnert I, Röhle R, Schneider H, Witsenburg M, Benson L, Gitter R, Bökenkamp R, Mahadevan V, Berger F; MELODY Registry investigators .

Eur Heart J. 2019 Apr 21. pii: ehz201. doi: 10.1093/eurheartj/ehz201. [Epub ahead of print]

PMID: 31005985

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Select item 30451687

 

  1. Intravascular assessment of coronary arteries in patients with cyanotic congenital heart disease.

Tarp JB, Radu MD, De Backer O, Jensen AS, Celermajer D, Sørensen K, Holstein-Rathlou NH, Engstrøm T, Søndergaard L.

EuroIntervention. 2019 Apr 20;14(17):1744-1750. doi: 10.4244/EIJ-D-18-00882.

PMID: 30451687 Free Article

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Select item 30910324

 

  1. First use and limitations of Magmaris® bioresorbable stenting in a low birth weight infant with native aortic coarctation.

Sallmon H, Berger F, Cho MY, Opgen-Rhein B.

Catheter Cardiovasc Interv. 2019 Apr 19. doi: 10.1002/ccd.28300. [Epub ahead of print]

PMID: 31001884

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Select item 31006669

 

  1. Personalized Three-Dimensional Printed Models in Congenital Heart Disease.

Sun Z, Lau I, Wong YH, Yeong CH.

J Clin Med. 2019 Apr 16;8(4). pii: E522. doi: 10.3390/jcm8040522. Review.

PMID: 30995803 Free Article

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Select item 30995723

 

  1. Hybrid approach to ventricular septal defect enlargement.

Patel ND, Justino H, Ing FF.

Catheter Cardiovasc Interv. 2019 Apr 14. doi: 10.1002/ccd.28227. [Epub ahead of print]

PMID: 30983081

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Select item 30983537

 

  1. The aorta after coarctation repair – effects of calibre and curvature on arterial haemodynamics.

Quail MA, Segers P, Steeden JA, Muthurangu V.

J Cardiovasc Magn Reson. 2019 Apr 11;21(1):22. doi: 10.1186/s12968-019-0534-7.

PMID: 30975162 Free PMC Article

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Select item 30977251

 

  1. Novel use of a 3D printed heart model to guide simultaneous percutaneous repair of severe pulmonary regurgitation and right ventricular outflow tract aneurysm.

Jivanji SGM, Qureshi SA, Rosenthal E.

Cardiol Young. 2019 Apr 10:1-4. doi: 10.1017/S1047951119000106. [Epub ahead of print]

PMID: 30968796

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Select item 31024935

 

 

  1. Sedation in the Pediatric and Congenital Catheterization Lab: Is It Time We Wake Them Up?

Jayaram N, Kaine S, Divekar AA.

JACC Cardiovasc Interv. 2019 Apr 5. pii: S1936-8798(19)30466-2. doi: 10.1016/j.jcin.2019.02.001. [Epub ahead of print] No abstract available.

PMID: 30981569

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Select item 20301630

 

  1. Correction to: Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

[No authors listed]

Circulation. 2019 Apr 2;139(14):e835. doi: 10.1161/CIR.0000000000000685. No abstract available.

PMID: 30933623

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Select item 30933622

 

  1. Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Oster M, Bhatt AB, Zaragoza-Macias E, Dendukuri N, Marelli A.

Circulation. 2019 Apr 2;139(14):e814-e830. doi: 10.1161/CIR.0000000000000605. Erratum in: Circulation. 2019 Apr 2;139(14):e835.

PMID: 30586769

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Select item 30586768

 

  1. Interventional Therapy Versus Medical Therapy for Secundum Atrial Septal Defect: A Systematic Review (Part 2) for the 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

Oster M, Bhatt AB, Zaragoza-Macias E, Dendukuri N, Marelli A.

J Am Coll Cardiol. 2019 Apr 2;73(12):1579-1595. doi: 10.1016/j.jacc.2018.08.1032. Epub 2018 Aug 16.

PMID: 30121241

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  1. SCAI expert consensus statement on operator and institutional requirements for PFO closure for secondary prevention of paradoxical embolic stroke: The American Academy of Neurology affirms the value of this statement as an educational tool for neurologists.

Horlick E, Kavinsky CJ, Amin Z, Dean Boudoulas K, Carroll JD, Hijazi ZM, Leifer D, Lutsep HL, Rhodes JF, Tobis JM.

Catheter Cardiovasc Interv. 2019 Apr 1;93(5):859-874. doi: 10.1002/ccd.28111. Epub 2019 Mar 21.

PMID: 30896894

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  1. Classification scheme for ductal morphology in cyanotic patients with ductal dependent pulmonary blood flow and association with outcomes of patent ductus arteriosus stenting.

Qureshi AM, Goldstein BH, Glatz AC, Agrawal H, Aggarwal V, Ligon RA, McCracken C, McDonnell A, Buckey TM, Whiteside W, Metcalf CM, Petit CJ.

Catheter Cardiovasc Interv. 2019 Apr 1;93(5):933-943. doi: 10.1002/ccd.28125. Epub 2019 Feb 21.

PMID: 30790426

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  1. Congenital Aortic Valve Stenosis: To Dilate or Operate?

Donald JS, Wallace FRO, d’Udekem Y, Konstantinov IE.

Heart Lung Circ. 2019 Apr;28(4):519-520. doi: 10.1016/j.hlc.2019.02.001. No abstract available.

PMID: 30871758

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Select item 30392984

 

  1. Is Fractional Flow Reserve Useful in Assessing Coronary Artery Fistula: A Case-Based Discussion and Review.

Hayman S, Goela A, Lavi S.

Heart Lung Circ. 2019 Apr;28(4):e51-e53. doi: 10.1016/j.hlc.2018.04.281. Epub 2018 Apr 19. Review.

PMID: 29735395

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  1. Balloon Aortic Valvuloplasty for Congenital Aortic Stenosis: A 14-Year Single Centre Review.

Auld B, Carrigan L, Ward C, Justo R, Alphonso N, Anderson B.

Heart Lung Circ. 2019 Apr;28(4):632-636. doi: 10.1016/j.hlc.2018.02.014. Epub 2018 Mar 1.

PMID: 29625867

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  1. First-in-human, off-label use of BeGraft® stenting of non-conduit, large right ventricular outflow tract for transcatheter valve landing zone preparation.

Egidy Assenza G, Mariucci ME, Chiarabelli M, Donti A.

Int J Cardiol. 2019 Apr 1;280:43-45. doi: 10.1016/j.ijcard.2019.01.036. Epub 2019 Jan 14.

PMID: 30665803

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Select item 30651192

 

  1. Aerobic capacity in adolescence is associated with time to intervention in adult men with atrial septal defects.

Larsson L, Rinnström D, Sandberg C, Högström G, Thilén U, Nordström P, Johansson B.

Int J Cardiol. 2019 Apr 1;280:57-60. doi: 10.1016/j.ijcard.2018.12.073. Epub 2018 Dec 28.

PMID: 30651192

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Select item 30477927

 

  1. 3D-assessment of RVOT dimensions prior percutaneous pulmonary valve implantation: comparison of contrast-enhanced magnetic resonance angiography versus 3D steady-state free precession sequence.

Ebel S, Gottschling S, Buzan MTA, Grothoff M, Dähnert I, Wagner R, Gräfe D, Lurz P, Gutberlet M, Lücke C.

Int J Cardiovasc Imaging. 2019 Apr 1. doi: 10.1007/s10554-019-01578-w. [Epub ahead of print]

PMID: 30937683

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  1. Tricuspid Valve Imaging and Intervention in Pediatric and Adult Patients With Congenital Heart Disease.

Qureshi MY, Sommer RJ, Cabalka AK.

JACC Cardiovasc Imaging. 2019 Apr;12(4):637-651. doi: 10.1016/j.jcmg.2018.10.036. Review.

PMID: 30947906

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Select item 31000097

 

  1. Early to Mid-Term Follow-Up Outcomes of Percutaneous Closure of Atrial Septal Defects Using Recent Generation Devices: a Single-Center Experience.

Kim AY, Jung SY, Chang JY, Jung JW, Choi JY.

Korean Circ J. 2019 Apr;49(4):326-335. doi: 10.4070/kcj.2018.0278. Epub 2018 Dec 17.

PMID: 30808079 Free PMC Article

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  1. Preliminary results of the CeraflexTM PDA occluder and device behaviour during releasing.

Epçaçan S, Bulut MO, Yücelİ İK, Ceviz N, Çelebi A.

Cardiol Young. 2019 Apr 29:1-5. doi: 10.1017/S1047951119000349. [Epub ahead of print]

PMID: 31032755

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  1. Ductal stenting to improve pulmonary blood flow in pulmonary atresia with intact ventricular septum and critical pulmonary stenosis after balloon valvuloplasty.

Haddad RN, Hanna N, Charbel R, Daou L, Chehab G, Saliba Z.

Cardiol Young. 2019 Apr 29:1-7. doi: 10.1017/S1047951119000118. [Epub ahead of print]

PMID: 31030705

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  1. Percutaneous device embolization of an aneurysmal pulmonary artery in an infant weighing <3 kilograms.

Reynolds K, Stapleton G, Herbert C, Mauriello D.

Catheter Cardiovasc Interv. 2019 Apr 29. doi: 10.1002/ccd.28292. [Epub ahead of print]

PMID: 31033192

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Select item 31036959

 

  1. Left Subclavian Artery Test Balloon Occlusion Before Covered Stent for Recoarctation and Aneurysm.

Rothman A, Ciccolo ML, Galindo A, Evans WN.

World J Pediatr Congenit Heart Surg. 2019 Apr 22:2150135118817309. doi: 10.1177/2150135118817309. [Epub ahead of print]

PMID: 31010397

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Select item 31005985

 

  1. Follow-up of percutaneous transcatheter closure of pulmonary arteriovenous fistulas.

Xi SB, Xie YM, Qian MY, Shi JJ, Li YF, Zhang ZW.

Chin Med J (Engl). 2019 Apr 20;132(8):980-983. doi: 10.1097/CM9.0000000000000182. No abstract available.

PMID: 30958441

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Select item 31001884

 

  1. “Treat-to-close”: Non-repairable ASD-PAH in the adult: Results from the North American ASD-PAH (NAAP) Multicenter Registry.

Bradley EA, Ammash N, Martinez SC, Chin K, Hebson C, Singh HS, Aboulhosn J, Grewal J, Billadello J, Chakinala MM, Daniels CJ, Zaidi AN.

Int J Cardiol. 2019 Apr 17. pii: S0167-5273(18)34660-6. doi: 10.1016/j.ijcard.2019.03.056. [Epub ahead of print]

PMID: 31031077

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  1. Sudden cardiac death associated with cardiac catheterization in Williams syndrome: a case report and review of literature.

Abu-Sultaneh S, Gondim MJ, Alexy RD, Mastropietro CW.

Cardiol Young. 2019 Apr 5:1-5. doi: 10.1017/S1047951119000295. [Epub ahead of print]

PMID: 30950363

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  1. Operator-Directed Procedural Sedation in the Congenital Cardiac Catheterization Laboratory.

O’Byrne ML, Millenson ME, Steven JM, Gillespie MJ, Dori Y, Glatz AC, Rome JJ.

JACC Cardiovasc Interv. 2019 Apr 5. pii: S1936-8798(19)30313-9. doi: 10.1016/j.jcin.2019.01.224. [Epub ahead of print]

PMID: 30981573

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  1. Prospective concurrent head-to head comparison of three different types of nitinol occluder devices for transcatheter closure of secundum atrial septal defects.

Bhattacharjya S, Pillai LS, Doraiswamy V, Satyanarayana RM, Chandrasekaran R, Pavithran S, Sivakumar K.

EuroIntervention. 2019 Apr 2. pii: EIJ-D-18-01016. doi: 10.4244/EIJ-D-18-01016. [Epub ahead of print]

PMID: 30946015 Free Article

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Select item 30940174

 

  1. Ultrasound-Guided Femoral Arterial Cannulation in Neonates Undergoing Cardiac Surgery or Catheterization: Comparison of 0.014-Inch Floppy Versus 0.019-Inch Straight Guidewire.

Polat TB.

Pediatr Crit Care Med. 2019 Apr 2. doi: 10.1097/PCC.0000000000001916. [Epub ahead of print]

PMID: 31013264

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  1. Endovascular repair in extra-anatomic bypass after hypoplastic aortic arch repair.

Careddu L, Petridis FD, Angeli E, Balducci A, Buia F, Lovato L, Gargiulo GD.

J Cardiovasc Med (Hagerstown). 2019 Apr;20(4):261-263. doi: 10.2459/JCM.0000000000000762. No abstract available.

PMID: 30664070

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Select item 30865105

 

  1. Abernethy malformation type 2: varied presentation, management and outcome.

Jain V, Sangdup T, Agarwala S, Bishoi AK, Chauhan S, Dhua A, Jana M, Kandasamy D, Malik R, Kothari SS, Patcharu R, Varshney A, Bhatnagar V.

J Pediatr Surg. 2019 Apr;54(4):760-765. doi: 10.1016/j.jpedsurg.2018.08.053. Epub 2018 Sep 5.

PMID: 30262201

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Select item 29796843

 

  1. Palliative emergency stenting of the vertical vein in obstructive supracardiac pulmonary venous drainage prior to surgical repair.

Al-Muhaya MA, Najjar AHA, Jelly AA, Alkodami AA, Mofeed M, Al-Mutairi M.

J Saudi Heart Assoc. 2019 Apr;31(2):64-68. doi: 10.1016/j.jsha.2018.12.001. Epub 2018 Dec 12.

PMID: 30618482 Free PMC Article

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Select item 30618480

 

  1. Early to Mid-Term Follow-Up Outcomes of Percutaneous Closure of Atrial Septal Defects Using Recent Generation Devices: a Single-Center Experience.

Kim AY, Jung SY, Chang JY, Jung JW, Choi JY.

Korean Circ J. 2019 Apr;49(4):326-335. doi: 10.4070/kcj.2018.0278. Epub 2018 Dec 17.

PMID: 30808079 Free PMC Article

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Select item 30947827

 

  1. Outcomes of Reinterventions for Children with Postoperative Pulmonary Venous Restenosis.

Kai L, Xiaoyang Z, Jinghao Z, Zhongqun Z, Qi S, Xiaomin H, Zhiwei X, Jinfen L.

Pediatr Cardiol. 2019 Apr 1. doi: 10.1007/s00246-019-02098-z. [Epub ahead of print]

PMID: 30937500

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Select item 30929066

 

  1. Umbilical Vein Catheter Protruding Through a Pulmonary Vein in a Patient with an Infracardiac Type Total Abnormal Pulmonary Venous Drainage.

Bruyndonckx L, Kroft LJM, Bekker V, Roest AAW, van der Palen RLF.

Pediatr Cardiol. 2019 Apr;40(4):878-879. doi: 10.1007/s00246-019-02094-3. Epub 2019 Mar 30. No abstract available.

PMID: 30929066 Free PMC Article

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Select item 30918992

 

  1. Catheterization Performed in the Early Postoperative Period After Congenital Heart Surgery in Children.

Eraso-Díaz Del Castillo AM, Escobar-Díaz MC, Lince Varela R, Díaz Medina LH, Cañas Arenas EM.

Pediatr Cardiol. 2019 Apr;40(4):827-833. doi: 10.1007/s00246-019-02078-3. Epub 2019 Mar 4.

PMID: 30830282

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Select item 30830281

 

  1. Balloon atrial septostomy in hypoplastic left heart syndrome with restrictive atrial septum.

Fukushima Y, Baba K, Kondo M, Kurita Y, Eitoku T, Shigemitsu Y, Hirai K, Tsukahara H, Iwasaki T, Kasahara S, Kotani Y, Otsuki S.

Pediatr Int. 2019 Apr;61(4):339-344. doi: 10.1111/ped.13716. Epub 2019 Mar 6.

PMID: 30339315

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Select item 30406366

 

  1. Transesophageal echocardiography-assisted transcatheter device closure of apical muscular ventricular septal defect.

Maddali MM, Al-Maskari SN, Kandachar PS, Arora NR, Lacour-Gayet F.

Saudi J Anaesth. 2019 Apr-Jun;13(2):148-150. doi: 10.4103/sja.SJA_625_18. No abstract available.

PMID: 31007665 Free PMC Article

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Select item 30031947

ACHD Surgery

ACHD Surgery

 

Congenital Heart Surgery Featured Manuscripts of April 2019 Manuscripts

 

Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve-sparing repair.

Annavajjhala V, Punn R, Tacy TA, Hanley FL, McElhinney DB.

Congenit Heart Dis. 2019 Apr 15. doi: 10.1111/chd.12772. [Epub ahead of print]

PMID: 30989806

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Select item 30732866

 

Take-Home Points:

 

  • This study utilized certain echocardiographic parameters to assess ventricular function and mechanics following tetralogy of Fallot repair with either a transannular patch or valve-preserving technique with follow-up of up to 7 years.
  • Global longitudinal strain and RV:LV basal dimension ratio parameters may be helpful in following ventricular dimensions and mechanics, especially when other typical parameters are within normal limits.
  • Early ventricular dilatation was observed after both repairs though was more significant after transannular patch repair. However, ventricular dilatation improved over time but did not reach normal parameters.
  • Significant pulmonary regurgitation was associated with worse LV global longitudinal strain and was more pronounced after transannular patch repair across all time points.
  • How these parameters may be practically applied to clinical decision-making (i.e., timing of pulmonary valve replacement) remains to be seen.

 

Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  The authors evaluated various right ventricular mechanics following tetralogy of Fallot (TOF) repair using echocardiographic methods with the aim of trying to understand the trajectory of ventricular function and mechanics over time beyond the early period after repair. Specifically, they compared RV parameters for patients who underwent a valve-sparing (VS) versus transannular patch (TAP) repair of TOF. Patients who underwent complete TOF repair <12 years at their center between January 2002 and September 2015 were included. Overall, 150 echo studies for 42 patients were analyzed. Of these patients, 45% underwent VS repair and 55% underwent TAP repair at a mean age of 2.1 months.

Concomitant RVOT procedures during VS repair included infundibular myectomy 89%), pulmonary valvotomy 42%, patch augmentation of the main pulmonary artery (37%), and a subvalvar RVOT patch (21%).

Moderate or severe PR was present in nearly all TAP patients across all time points, though the proportion of patients with severe PR following VS repair increased over time. There was gradual RV dilation over time in the TAP group but not the VS group. LV EF was lower in the VS group early but improved over time. LV and RV dysfunction were present postoperatively in both groups and did not fully recover during the study period. Significant PR was associated with lower LV global longitudinal strain.

A total of 10 patients underwent surgical or transcatheter reintervention (8 in the TAP group, 2 in the VS group). Of these, 7 involved surgical PVR and 1 transcatheter PVR.​ However, it is not clear how the echocardiographic parameters utilized in the study were applied to these specific patients.

The authors conclude that ventricular function tends to improve over time after TOF repair. TAP repair tends to be associated with more RV dilatation, and global longitudinal strain correlated inversely with the presence of pulmonary regurgitation. Importantly, ventricular parameters improved over time, but did not reach normal limits. Certain parameters such as and RV:LV basal dimension ratio may provide objective data for following patients, especially before LV function is impacted.

How these parameters may factor into further decision-making (i.e., timing of pulmonary valve replacement), is not yet clear. Perhaps more importantly, long-term trends in these parameters may help clinicians weigh initial surgical options for TOF repair.

 

  1. Extracorporeal membrane oxygenation use in the first 24 hours following pediatric heart transplantation: Incidence, risk factors, and outcomes.

Godown J, Bearl DW, Thurm C, Hall M, Feingold B, Soslow JH, Mettler BA, Smith AH, Profita EL, Singh TP, Dodd DA.

Pediatr Transplant. 2019 Apr 11:e13414. doi: 10.1111/petr.13414. [Epub ahead of print]

PMID: 30973190

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Select item 30973305

 

Take Home Points:

 

  • Primary graft dysfunction (requiring ECMO) after pediatric heart transplant is not uncommon and can lead to significant morbidity or mortality.
  • This retrospective study was designed using large administrative databases to find the incidence of, risk factors for, and outcomes of primary graft dysfunction and ECMO utilization after heart transplant. The results indicate that primary dysfunction is not rare (7.9% of transplants) and that outcomes are strongly associated with the duration of ECMO.

 

Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch:  Primary graft dysfunction after heart transplant is often described as a “black box” of unknown etiology that requires extracorporeal membrane oxygenation (ECMO) support to allow the heart to recover, which may not occur. It constitutes 40% of mortality after transplant and often a single factor is not identified as the precipitating cause. The authors of this study sought to examine the incidence of, factors for and outcomes from the use of ECMO within 24 hours after heart transplant in children. They sought to examine two large administrative databases to utilize a unique data linkage to examine their questions.

The SPTR database includes information from every organ transplant since 1987. The PHIS database is an administrative/billing database that captures clinical and resource utilization data, including ICD-9 and -10 codes, from over 50 children’s hospitals. The researchers queried all heart transplants in children under 18 years old between 2002 and 2016 from the linked database and found 2820 patients. Primary graft dysfunction was then examined by using the PHIS to obtain billing codes for ECMO initiation within 24 hours of heart transplant. This was also the method to delineate the duration of ECMO therapy for each of these patients. Baseline demographics and data regarding the heart transplant were obtained. Analyses of this data using multivariate logistic regression models were used to assess risk factors for primary graft dysfunction. The authors also looked at each major transplant group (cardiomyopathy vs. congenital heart disease) to evaluate if there are separate risk factors for each. Outcomes evaluated included ECMO decannulation, re-transplantation and death.

The first key finding from this report is that 7.9% (n=224) of heart transplant recipients required ECMO within the first 24 hours after transplant. The median time on ECMO was 2 days with a maximum duration of 50 days. An evaluation of the multivariable logistic regression of patient demographics and primary graft dysfunction is summarized in table 2. It is not surprising that the authors found that younger patients and patients already on ECMO at time of transplant were independently associated with primary graft dysfunction. The risk factors for ECMO are also evaluated when the transplant recipients are separated into cardiomyopathy vs. congenital heart disease subgroups (Table 3.) Interestingly, only 14.3% (n=32) of the patients placed on ECMO died while on ECMO as seen in Figure 1. Of the 191 patients who were decannulated, 167 (87.4%) survived until discharge. The study also highlights that the survival to discharge decreases as the duration of the ECMO course increases, from 89% survival for 1-3 day ECMO runs to 18.8% survival for more than 10 days. This data is also presented graphically in Figure 2. The risks factors for in-hospital mortality for patients requiring post-transplant ECMO are presented in Table 5. Unsurprisingly, long ischemic times, length of ECMO and post-transplant dialysis were associated with in-hospital mortality. The need for post-transplant ECMO was associated with inferior in-house patient survival compared to those who did not need ECMO. However, for those who survived to discharge after ECMO, there was no long-term difference in survival when compared to those who did not require ECMO (Figures 3 and 4).

This unique method of assessing ECMO utilization in the first 24 hours after transplant by using large administrative databases gives a more thorough understanding of which patients are at risk for the need for post-transplant ECMO and which patients who do need ECMO will survive to discharge. The cynic in me points to the fact that hospitals are not going to forget to bill for ECMO and thus this data should be more complete than other voluntary collaborative databases. Still this data comes from just 28 hospitals and there is obviously much variability among transplant programs’ practices. The findings here will allow transplant programs to help guide their patients’ families into the short- and long-term outcomes from a post-transplant ECMO run. The results seem to be more favorable than expected. It is clear that this study does not allow for a better understanding of what the indication for ECMO was for these patients. And there is obviously the risks of errors and missing information in this data, but the summary of findings should give both practitioners and families hope that the need for post-transplant ECMO support is not a death sentence.

Tables and Figures.

 

 

 

CHD Surgery April 2019

 

  1. Unilateral interruption of pulmonary artery with pulmonary hypertension: a case for heart lung transplantation?

Singhal R.

Cardiol Young. 2019 Apr 30:1-4. doi: 10.1017/S1047951119000271. [Epub ahead of print]

PMID: 31036098

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Select item 31036097

 

  1. Repair of tricuspid valve regurgitation due to a tricuspid valve cleft.

Saku K, Inoue H, Yamamoto K, Ueno M.

Asian Cardiovasc Thorac Ann. 2019 Apr 29:218492319847365. doi: 10.1177/0218492319847365. [Epub ahead of print]

PMID: 31032618

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Select item 31030704

 

  1. The surgical treatment of anomalous origin of one pulmonary artery from the ascending aorta.

Dong S, Yan J, Xu H, Duan Y, Liu C.

J Cardiothorac Surg. 2019 Apr 27;14(1):82. doi: 10.1186/s13019-019-0904-0.

PMID: 31029153 Free Article

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Select item 31032647

 

  1. The efficiency of systemic-to-pulmonary shunts in older children with hypoplastic pulmonary arteries.

Zhang H, Fan X, Su J, Liu Y, Zhao L, Li G.

J Card Surg. 2019 Apr 26. doi: 10.1111/jocs.14063. [Epub ahead of print]

PMID: 31025765

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Select item 31030195

 

  1. Neurodevelopmental and health-related quality-of-life outcomes in adolescence after surgery for congenital heart disease in infancy.

Wotherspoon JM, Eagleson KJ, Gilmore L, Auld B, Hirst A, Johnson S, Stocker C, Heussler H, Justo RN.

Dev Med Child Neurol. 2019 Apr 25. doi: 10.1111/dmcn.14251. [Epub ahead of print]

PMID: 31025336

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Select item 31033906

 

  1. We Can Learn From the Past, but We Must Pave the Future of Congenital Heart Disease Research.

Hussain S, Belley-Côté EP, Whitlock RP.

JAMA Cardiol. 2019 Apr 24. doi: 10.1001/jamacardio.2019.1066. [Epub ahead of print] No abstract available.

PMID: 31017617

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Select item 31017614

 

  1. We Can Learn From the Past, but We Must Pave the Future of Congenital Heart Disease Research-Reply.

Smith CA Jr, Oster M, Kochilas L.

JAMA Cardiol. 2019 Apr 24. doi: 10.1001/jamacardio.2019.1076. [Epub ahead of print] No abstract available.

PMID: 31017614

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Select item 31019028

 

  1. Autologous Tissue Technique to Repair Unilateral Isolated Absence of a Pulmonary Artery.

von Stumm M, Biermann D, Reichenspurner H, Gottschalk U, Müller G, Kozlik-Feldmann R, Riso A, Sachweh JS.

World J Pediatr Congenit Heart Surg. 2019 Apr 24:2150135119825588. doi: 10.1177/2150135119825588. [Epub ahead of print]

PMID: 31018755

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Select item 31015364

 

  1. Long-term results of atrial maze surgery in patients with congenital heart disease.

Gonzalez Corcia MC, Walsh EP, Emani S.

Europace. 2019 Apr 19. pii: euz056. doi: 10.1093/europace/euz056. [Epub ahead of print]

PMID: 31004150

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Select item 30998304

 

  1. Valve-Sparing Repair of Tetralogy of Fallot with Intraoperative Dilation of the Pulmonary Valve. Mid-Term Results.

Lozano-Balseiro M, Garcia-Vieites M, Martínez-Bendayán I, García-Hernández I, Cuenca-Castillo JJ, Rueda-Núñez F, Bautista-Hernandez V.

Semin Thorac Cardiovasc Surg. 2019 Apr 18. pii: S1043-0679(19)30088-7. doi: 10.1053/j.semtcvs.2019.04.007. [Epub ahead of print]

PMID: 31005576

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Select item 30997589

 

  1. Extracorporeal Membrane Oxygenation Mortality in High-Risk Populations: An Analysis of the Pediatric Health Information System Database.

Coleman RD, Goldman J, Moffett B, Guffey D, Loftis L, Thomas J, Shekerdemian LS.

ASAIO J. 2019 Apr 15. doi: 10.1097/MAT.0000000000001002. [Epub ahead of print]

PMID: 31045920

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Select item 30989806

 

  1. Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve-sparing repair.

Annavajjhala V, Punn R, Tacy TA, Hanley FL, McElhinney DB.

Congenit Heart Dis. 2019 Apr 15. doi: 10.1111/chd.12772. [Epub ahead of print]

PMID: 30989806

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Select item 30732866

 

  1. Right ventricular remodeling after conduit replacement in patients with corrected tetralogy of Fallot – evaluation by cardiac magnetic resonance.

Guné H, Sjögren J, Carlsson M, Gustafsson R, Sjöberg P, Nozohoor S.

J Cardiothorac Surg. 2019 Apr 15;14(1):77. doi: 10.1186/s13019-019-0899-6.

PMID: 30987651 Free PMC Article

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Select item 30983081

 

  1. Diagnosis and Management of the Unligated Vertical Vein in Repaired Total Anomalous Pulmonary Venous Connection.

Akam-Venkata J, Turner DR, Joshi A, Aggarwal S, Gupta P.

World J Pediatr Congenit Heart Surg. 2019 Apr 14:2150135118817491. doi: 10.1177/2150135118817491. [Epub ahead of print]

PMID: 30983537

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Select item 30980591

 

  1. Left thorax approach to repair doubly committed juxta-arterial ventricular septal defect with Da Vinci robotic system.

Hu Y, Deng J, Zhao S, Zhong Q.

J Card Surg. 2019 Apr 13. doi: 10.1111/jocs.14036. [Epub ahead of print]

PMID: 30981213

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Select item 30982075

 

  1. Morbidity During Adult Congenital Heart Surgery Admissions.

Setton M, He W, Benavidez OJ.

Pediatr Cardiol. 2019 Apr 11. doi: 10.1007/s00246-019-02103-5. [Epub ahead of print]

PMID: 30976885

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Select item 30976884

 

  1. Readmissions Following Congenital Heart Surgery in Infants and Children.

Benavidez OJ, He W, Lahoud-Rahme M.

Pediatr Cardiol. 2019 Apr 11. doi: 10.1007/s00246-019-02104-4. [Epub ahead of print]

PMID: 30976884

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Select item 30973305

 

  1. Comparison of outcomes of pulmonary valve replacement in adult versus paediatric hospitals: institutional influence†.

Fuller S, Ramachandran A, Awh K, Faerber JA, Patel PA, Nicolson SC, O’Byrne ML, Mascio CE, Kim YY.

Eur J Cardiothorac Surg. 2019 Apr 8. pii: ezz102. doi: 10.1093/ejcts/ezz102. [Epub ahead of print]

PMID: 30957859

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Select item 31024343

 

  1. Cystic Fibrosis: From Qualification to Lung Transplantation, a Single Center Experience.

Ochman M, Latos M, Urlik M, Stącel T, Nęcki M, Tatoj Z, Zawadzki F, Wajda-Pokrontka M, Przybyłowski P, Zembala M.

Ann Transplant. 2019 Apr 5;24:185-190. doi: 10.12659/AOT.914328.

PMID: 30948702 Free PMC Article

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Select item 30789367

 

  1. MicroRNAs as potential biomarkers in congenital heart surgery.

Stoica SC, Dorobantu DM, Vardeu A, Biglino G, Ford KL, Bruno DV, Zakkar M, Mumford A, Angelini GD, Caputo M, Emanueli C.

J Thorac Cardiovasc Surg. 2019 Apr 4. pii: S0022-5223(19)30740-8. doi: 10.1016/j.jtcvs.2019.03.062. [Epub ahead of print]

PMID: 31043318

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Select item 30957182

 

  1. Reduced biventricular volumes and myocardial dysfunction long-term after pediatric heart transplantation assessed by CMR.

Latus H, Hachmann P, Voges I, Mueller S, Gummel K, Reich B, Sarikouch S, Peters B, Mazhari N, Behnke-Hall K, Jux C, Apitz C, Thul J, Akintuerk H, Bauer J, Schranz D.

Transplantation. 2019 Apr 4. doi: 10.1097/TP.0000000000002738. [Epub ahead of print]

PMID: 30964835

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Select item 30942148

 

  1. Contegra versus pulmonary homograft for right ventricular outflow tract reconstruction in newborns.

Falchetti A, Demanet H, Dessy H, Melot C, Pierrakos C, Wauthy P.

Cardiol Young. 2019 Apr 3:1-6. doi: 10.1017/S1047951119000143. [Epub ahead of print]

PMID: 30942148

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Select item 30950974

 

  1. ‘Off pump’ self-expanding injectable tissue valves (IPVR) versus ‘on pump’ conventional tissue valves (PVR) for replacement of the pulmonary valve: trial protocol for a randomised controlled trial (InVITe trial).

Heys R, Angelini G, Caputo M, Culliford L, Pufulete M, Reeves BC, Rogers CA, Stoica S, Parry A.

BMJ Open. 2019 Apr 2;9(4):e026221. doi: 10.1136/bmjopen-2018-026221.

PMID: 30944136 Free Article

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Select item 30933623

 

  1. [Non-cardiac surgery in adults with congenital heart defects : Most important parameters in anesthesia management].

Massoth C, Zarbock A, Wenk M.

Anaesthesist. 2019 Apr;68(4):245-258. doi: 10.1007/s00101-019-0575-1. German.

PMID: 30911773

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Select item 29324491

 

  1. Third Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report: Preimplant Characteristics and Outcomes.

Morales DLS, Rossano JW, VanderPluym C, Lorts A, Cantor R, St Louis JD, Koeh D, Sutcliffe DL, Adachi I, Kirklin JK, Rosenthal DN, Blume ED; Pedimacs Investigators.

Ann Thorac Surg. 2019 Apr;107(4):993-1004. doi: 10.1016/j.athoracsur.2019.01.038. Epub 2019 Feb 26.

PMID: 30817920

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Select item 30395857

 

  1. Impact of Heparin- or Nonheparin-Coated Circuits on Platelet Function in Pediatric Cardiac Surgery.

Giorni C, Pezzella C, Bojan M, Ricci Z, Pouard P, Raisky O, Tourneur L, La Salvia O, Favia I, Borgel D, Cogo P, Carotti A, Lasne D.

Ann Thorac Surg. 2019 Apr;107(4):1241-1247. doi: 10.1016/j.athoracsur.2018.09.032. Epub 2018 Nov 3.

PMID: 30395857

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Select item 30722979

 

  1. Health-related quality of life in congenital heart disease surgery in children and young adults: a systematic review and meta-analysis.

Ladak LA, Hasan BS, Gullick J, Gallagher R.

Arch Dis Child. 2019 Apr;104(4):340-347. doi: 10.1136/archdischild-2017-313653. Epub 2018 Mar 23.

PMID: 29572215

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Select item 31019643

 

  1. Combined heart and liver transplantation: State of knowledge and outlooks.

Lebray P, Varnous S.

Clin Res Hepatol Gastroenterol. 2019 Apr;43(2):123-130. doi: 10.1016/j.clinre.2018.08.009. Epub 2019 Jan 8. Review.

PMID: 30635235

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Select item 30770573

 

  1. Pulmonary arterial hypertension in adults with systemic right ventricles referred for cardiac transplantation.

Kim YY, Awh K, Acker M, Atluri P, Bermudez C, Crespo M, Diamond JM, Drajpuch D, Forde-Mclean R, Fuller S, Goldberg L, Mazurek J, Mascio C, Menachem JN, Rame E, Ruckdeschel E, Tobin L, Wald J.

Clin Transplant. 2019 Apr;33(4):e13496. doi: 10.1111/ctr.13496. Epub 2019 Mar 5.

PMID: 30770573

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Select item 30676534

 

  1. Dynamic changes and clinical significance of LXA4 in the perioperative period of cardiopulmonary bypass.

Wu M, Feng Z, Liao J, Zhou H, Wang J.

Exp Ther Med. 2019 Apr;17(4):3169-3173. doi: 10.3892/etm.2019.7304. Epub 2019 Feb 22.

PMID: 30936990 Free PMC Article

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Select item 30232381

 

  1. Minimally Invasive Surgery for Coronary-Pulmonary Artery Fistula Via Parasternal Minithoracotomy.

Liu H, Mei J, He Y, Jiang Z, Tang M.

Heart Lung Circ. 2019 Apr;28(4):e79-e82. doi: 10.1016/j.hlc.2018.04.307. Epub 2018 Sep 29.

PMID: 30318390

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Select item 29735395

 

  1. Durability of pulmonary homografts for reconstruction of the right ventricular outflow tract: how relevant are donor-related factors?

Dekens E, Van Damme E, Jashari R, Van Hoeck B, François K, Bové T.

Interact Cardiovasc Thorac Surg. 2019 Apr 1;28(4):503-509. doi: 10.1093/icvts/ivy316.

PMID: 30476047

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Select item 30376066

 

  1. Anomalous origin of both the right and left coronary arteries: a singular anomaly†.

Kostolny M, Kanakis M, Hsia TY.

Interact Cardiovasc Thorac Surg. 2019 Apr 1;28(4):640-641. doi: 10.1093/icvts/ivy301.

PMID: 30376066

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Select item 30939839

 

  1. Impact of additional tricuspid valve annuloplasty in TOF patients undergoing pulmonary valve replacement.

Lueck S, Bormann E, Rellensmann K, Martens S, Rukosujew A.

J Cardiovasc Surg (Torino). 2019 Apr;60(2):268-273. doi: 10.23736/S0021-9509.18.10385-5. Epub 2018 May 22.

PMID: 29786408

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Select item 30659708

 

  1. Ventricular assist device support for complex congenital heart disease: Inspiration from history of surgical evolution.

Adachi I.

J Heart Lung Transplant. 2019 Apr;38(4):431-432. doi: 10.1016/j.healun.2019.02.007. Epub 2019 Feb 16. No abstract available.

PMID: 30945638

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Select item 30459063

 

  1. Outcomes of children with congenital heart disease implanted with ventricular assist devices: An analysis of the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs).

Peng DM, Koehl DA, Cantor RS, McMillan KN, Barnes AP, McConnell PI, Jordan J, Andersen ND, St Louis JD, Maeda K, Kirklin JK, Kindel SJ.

J Heart Lung Transplant. 2019 Apr;38(4):420-430. doi: 10.1016/j.healun.2018.10.008. Epub 2018 Oct 31.

PMID: 30459063

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Select item 30391197

 

  1. Outcomes of children supported with an intracorporeal continuous-flow left ventricular assist system.

VanderPluym CJ, Adachi I, Niebler R, Griffiths E, Fynn-Thompson F, Chen S, O’Connor MJ, Machado D, Hawkins B, Bleiweis MS, Koehl DA, Cantor RS, Morales D, Lorts A.

J Heart Lung Transplant. 2019 Apr;38(4):385-393. doi: 10.1016/j.healun.2018.09.015. Epub 2018 Sep 25.

PMID: 30391197

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Select item 30966831

 

  1. The brain protective effect of dexmedetomidine during surgery for paediatric patients with congenital heart disease.

Gong J, Zhang R, Shen L, Xie Y, Li X.

J Int Med Res. 2019 Apr;47(4):1677-1684. doi: 10.1177/0300060518821272.

PMID: 30966831 Free PMC Article

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Select item 30350443

 

  1. S100B increases in cyanotic versus noncyanotic infants undergoing heart surgery and cardiopulmonary bypass (CPB).

Varrica A, Satriano A, Gavilanes ADW, Zimmermann LJ, Vles HJS, Pluchinotta F, Anastasia L, Giamberti A, Baryshnikova E, Gazzolo D.

J Matern Fetal Neonatal Med. 2019 Apr;32(7):1117-1123. doi: 10.1080/14767058.2017.1401604. Epub 2017 Nov 28.

PMID: 29183208

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Select item 30637595

 

  1. Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons’ Society Study.

Jegatheeswaran A, Jacobs ML, Caldarone CA, Kirshbom PM, Williams WG, Blackstone EH, DeCampli WM, Duncan KF, Lambert LM, Walters HL, Tchervenkov CI, McCrindle BW.

J Thorac Cardiovasc Surg. 2019 Apr;157(4):1577-1587.e10. doi: 10.1016/j.jtcvs.2018.11.152. Epub 2019 Jan 11.

PMID: 30770109

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Select item 30770106

 

  1. Centrifugal-flow ventricular assist device support in children: A single-center experience.

Adachi I, Zea-Vera R, Tunuguntla H, Denfield SW, Elias B, John R, Teruya J, Fraser CD Jr.

J Thorac Cardiovasc Surg. 2019 Apr;157(4):1609-1617.e2. doi: 10.1016/j.jtcvs.2018.12.045. Epub 2018 Dec 27.

PMID: 30770106

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Select item 30638615

 

  1. Commentary: Moving beyond survival: The long-term psychosocial impact of congenital heart disease.

Lamari-Fisher A, Mery CM.

J Thorac Cardiovasc Surg. 2019 Apr;157(4):1588-1589. doi: 10.1016/j.jtcvs.2018.11.086. Epub 2018 Dec 4. No abstract available.

PMID: 30638615

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Select item 30294866

 

  1. Outcomes of Prosthetic Valved Conduits for Right Ventricular Outflow Tract Reconstruction.

Chen H, Shi G, Qiu L, Wang S, Chen H, Xu Z.

Pediatr Cardiol. 2019 Apr;40(4):848-856. doi: 10.1007/s00246-019-02081-8. Epub 2019 Mar 18.

PMID: 30887063

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Select item 30879085

 

  1. Correlation Between Cardiopulmonary Exercise Test, Spirometry, and Congenital Heart Disease Severity in Pediatric Population.

Morales Mestre N, Reychler G, Goubau C, Moniotte S.

Pediatr Cardiol. 2019 Apr;40(4):871-877. doi: 10.1007/s00246-019-02084-5. Epub 2019 Mar 8.

PMID: 30850878

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Select item 30830281

 

  1. Characteristics and Outcomes of Heart Transplantation in DiGeorge Syndrome.

Woolman P, Bearl DW, Soslow JH, Dodd DA, Thurm C, Hall M, Feingold B, Godown J.

Pediatr Cardiol. 2019 Apr;40(4):768-775. doi: 10.1007/s00246-019-02063-w. Epub 2019 Feb 7.

PMID: 30729260

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Select item 30726509

 

  1. Impact of Time Interval Between Glenn and Fontan Procedures on Fontan Operative and Long-Term Follow-up Results.

Yi T, Fan G, Xing Y, Zhao W, Zhang L, Fan F, Jiang X, Ma Z, Yan J, Li S, Wang Q.

Pediatr Cardiol. 2019 Apr;40(4):705-712. doi: 10.1007/s00246-018-2049-7. Epub 2019 Jan 16.

PMID: 30652193

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Select item 30950987

 

  1. Fluid Management Practices After Surgery for Congenital Heart Disease: A Worldwide Survey.

Hanot J, Dingankar AR, Sivarajan VB, Sheppard C, Cave D, Garcia Guerra G.

Pediatr Crit Care Med. 2019 Apr;20(4):357-364. doi: 10.1097/PCC.0000000000001818.

PMID: 30950987

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Select item 30228371

 

  1. Risk factors for prolonged mechanical ventilation after surgical repair of congenital heart disease. Experience from a single cardiac center.

Alrddadi SM, Morsy MM, Albakri JK, Mohammed MA, Alnajjar GA, Fawaz MM, Alharbi AA, Alnajjar AA, Almutairi MM, Sayed AU, Khoshal SQ, Shihata MS, Salim SS, Almuhaya MA, Jelly AE, Alharbi KM, Alharbi IH, Abutaleb AR, Sandogji HI, Hussein MA.

Saudi Med J. 2019 Apr;40(4):367-371. doi: 10.15537/smj.2019.4.23682.

PMID: 30957130 Free Article

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Select item 30267752

 

  1. The Prevalence and Impact of Congenital Diaphragmatic Hernia Among Patients Undergoing Surgery for Congenital Heart Disease.

Fraser CD 3rd, Hill KD, Wallace A, Chiswell K, Zhou X, Jelin EB, Kays D, Jacobs JP, Hibino N, Jacobs ML, Vricella LA.

Semin Thorac Cardiovasc Surg. 2019 Spring;31(1):69-77. doi: 10.1053/j.semtcvs.2018.09.014. Epub 2018 Sep 27.

PMID: 30267752

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Select item 30104111

 

  1. From Multiple Organ Support Therapy to Extracorporeal Organ Support in Critically Ill Patients.

Ronco C, Ricci Z, Husain-Syed F.

Blood Purif. 2019 Apr 26:1-7. doi: 10.1159/000490694. [Epub ahead of print] Review.

PMID: 31030203 Free Article

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Select item 31023188

 

  1. Congenital Coronary Aneurysm: Unusual Presentation of Myocardial Ischemia in Children.

Simry W, Al Kindi H, Sedky Y, Kaml H, Romaih S, Van Doorn C.

World J Pediatr Congenit Heart Surg. 2019 Apr 25:2150135118809078. doi: 10.1177/2150135118809078. [Epub ahead of print]

PMID: 31023146

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Select item 31020347

 

  1. A Very Rare Case of Coronary Artery Bypass Grafting in a Progeria Child.

Cerejo RPS, Rodrigues RAN, Martins JD, Torres CGEC, Sousa LM, Pinto FF, Fragata JIG.

World J Pediatr Congenit Heart Surg. 2019 Apr 23:2150135118803596. doi: 10.1177/2150135118803596. [Epub ahead of print]

PMID: 31014187

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Select item 30689697

 

  1. Surgical Rescue After Failed Percutaneous Closure of an Aorto-Atrial Tunnel.

Caldaroni F, Lo Rito M, Saracino A, Varrica A, Giugno L, Giamberti A, Frigiola A.

World J Pediatr Congenit Heart Surg. 2019 Apr 21:2150135118802802. doi: 10.1177/2150135118802802. [Epub ahead of print]

PMID: 31006348

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Select item 30958441

 

  1. Serial assessment of postoperative ventricular mechanics in young children with tetralogy of Fallot: Comparison of transannular patch and valve-sparing repair.

Annavajjhala V, Punn R, Tacy TA, Hanley FL, McElhinney DB.

Congenit Heart Dis. 2019 Apr 15. doi: 10.1111/chd.12772. [Epub ahead of print]

PMID: 30989806

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Select item 30732866

 

  1. Reverse Remodeling of Pulmonary Arterioles After Pulmonary Artery Banding in Patients ≥ 2 Years Old with Severe Pulmonary Arterial Hypertension and Congenital Heart Disease.

Liu C, Cheng P, Liu A, Li B, Yang Y, Wang Z, Su J.

Pediatr Cardiol. 2019 Apr 13. doi: 10.1007/s00246-019-02097-0. [Epub ahead of print]

PMID: 30982075

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Select item 30978120

 

  1. Readmissions Following Congenital Heart Surgery in Infants and Children.

Benavidez OJ, He W, Lahoud-Rahme M.

Pediatr Cardiol. 2019 Apr 11. doi: 10.1007/s00246-019-02104-4. [Epub ahead of print]

PMID: 30976884

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Select item 30973190

 

  1. Extracorporeal membrane oxygenation use in the first 24 hours following pediatric heart transplantation: Incidence, risk factors, and outcomes.

Godown J, Bearl DW, Thurm C, Hall M, Feingold B, Soslow JH, Mettler BA, Smith AH, Profita EL, Singh TP, Dodd DA.

Pediatr Transplant. 2019 Apr 11:e13414. doi: 10.1111/petr.13414. [Epub ahead of print]

PMID: 30973190

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Select item 30973305

 

  1. A Retrospective Comparison of the Supported and Unsupported Bovine Jugular Vein Conduit in Children.

Lueth ET, Gist KM, Burkett DA, Landeck BF, Brinton JT, Meier MR, Coffin JM, Schafer M, Jaggers J, Mitchell MB.

Ann Thorac Surg. 2019 Apr 8. pii: S0003-4975(19)30498-9. doi: 10.1016/j.athoracsur.2019.03.021. [Epub ahead of print]

PMID: 30974093

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Select item 30957737

 

  1. Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease.

Xi SB, Wang SS, Qian MY, Xie YM, Li JJ, Zhang ZW.

Chin Med J (Engl). 2019 Apr 5;132(7):811-818. doi: 10.1097/CM9.0000000000000145.

PMID: 30789367

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Select item 30950302

 

  1. The black swan: Unique coronary arterial anatomy observed in a patient with transposition.

Michalak KW, Wernovsky G, Moll M, Anderson RH.

J Thorac Cardiovasc Surg. 2019 Apr 4. pii: S0022-5223(19)30771-8. doi: 10.1016/j.jtcvs.2019.03.087. [Epub ahead of print] No abstract available.

PMID: 31036358

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Select item 30964835

 

  1. International Survey on Extracorporeal Membrane Oxygenation Transport.

Broman LM, Dirnberger DR, Malfertheiner MV, Aokage T, Morberg P, Næsheim T, Pappalardo F, Di Nardo M, Preston T, Burrell AJC, Daly I, Harvey C, Mason P, Philipp A, Bartlett RH, Lynch W, Belliato M, Taccone FS.

ASAIO J. 2019 Apr 2. doi: 10.1097/MAT.0000000000000997. [Epub ahead of print]

PMID: 30946060

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Select item 30586764

 

  1. Converting Fontan-Björk to 1.5- or 2-Ventricle Circulation.

Hopkins KA, Brown JW, Darragh RK, Kay WA.

Ann Thorac Surg. 2019 Apr;107(4):e259-e261. doi: 10.1016/j.athoracsur.2018.07.097. Epub 2018 Oct 10.

PMID: 30315794

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Select item 30912153

 

  1. Welcome to the Fifteenth International conference on Pediatric Mechanical Circulatory Support Systems and Pediatric Cardiopulmonary Perfusion.

Law S, Chai P, Ündar A.

Artif Organs. 2019 Apr;43(4):329-333. doi: 10.1111/aor.13437. Epub 2019 Mar 25. No abstract available.

PMID: 30912153

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Select item 30935634

 

  1. Outcomes After Extracorporeal Cardiopulmonary Resuscitation of Pediatric In-Hospital Cardiac Arrest: A Report From the Get With the Guidelines-Resuscitation and the Extracorporeal Life Support Organization Registries.

Bembea MM, Ng DK, Rizkalla N, Rycus P, Lasa JJ, Dalton H, Topjian AA, Thiagarajan RR, Nadkarni VM, Hunt EA; American Heart Association’s Get With The Guidelines – Resuscitation Investigators.

Crit Care Med. 2019 Apr;47(4):e278-e285. doi: 10.1097/CCM.0000000000003622.

PMID: 30747771

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Select item 30730315

 

  1. Virtual reality for preoperative planning in large ventricular septal defects.

Mendez A, Hussain T, Hosseinpour AR, Valverde I.

Eur Heart J. 2019 Apr 1;40(13):1092. doi: 10.1093/eurheartj/ehy685. No abstract available.

PMID: 30329041

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Select item 30239653

 

  1. Long-term results of ventricular septation for double-inlet left ventricle†.

Shimada M, Hoashi T, Nakata T, Kurosaki K, Kanzaki S, Shiraishi I, Ichikawa H.

Eur J Cardiothorac Surg. 2019 Apr 1. pii: ezz097. doi: 10.1093/ejcts/ezz097. [Epub ahead of print]

PMID: 30932159

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Select item 30622330

 

  1. Aortic angle is associated with neo-aortic root dilatation and regurgitation following arterial switch operation.

Martins D, Khraiche D, Legendre A, Boddaert N, Raisky O, Bonnet D, Raimondi F.

Int J Cardiol. 2019 Apr 1;280:53-56. doi: 10.1016/j.ijcard.2019.01.042. Epub 2019 Jan 11.

PMID: 30660585

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  1. Echocardiographic Assessment of Right Ventricular Function in Clinically Well Pediatric Heart Transplantation Patients and Comparison With Normal Control Subjects.

White BR, Katcoff H, Faerber JA, Lin KY, Rossano JW, Mercer-Rosa L, O’Connor MJ.

J Am Soc Echocardiogr. 2019 Apr;32(4):537-544.e3. doi: 10.1016/j.echo.2019.01.015.

PMID: 30954122

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  1. Robotic repair of left-sided partial anomalous pulmonary venous connection to the coronary sinus.

Onan B, Aydin U, Kadirogullari E, Ozturk E.

J Robot Surg. 2019 Apr;13(2):319-323. doi: 10.1007/s11701-018-0825-2. Epub 2018 May 23.

PMID: 29796843

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  1. Infective endocarditis in children with normal heart: Indication for surgical intervention.

Nasser BA, Al Qwaee A, Almesned AR, Akhfash A, Mohamad T, Chaikhouni F, Alhabshan F, Kabbani MS.

J Saudi Heart Assoc. 2019 Apr;31(2):51-56. doi: 10.1016/j.jsha.2018.11.003. Epub 2018 Nov 22.

PMID: 30618480 Free PMC Article

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  1. Self-reported functional health status following interrupted aortic arch repair: A Congenital Heart Surgeons’ Society Study.

Jegatheeswaran A, Jacobs ML, Caldarone CA, Kirshbom PM, Williams WG, Blackstone EH, DeCampli WM, Duncan KF, Lambert LM, Walters HL, Tchervenkov CI, McCrindle BW.

J Thorac Cardiovasc Surg. 2019 Apr;157(4):1577-1587.e10. doi: 10.1016/j.jtcvs.2018.11.152. Epub 2019 Jan 11.

PMID: 30770109

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  1. Centrifugal-flow ventricular assist device support in children: A single-center experience.

Adachi I, Zea-Vera R, Tunuguntla H, Denfield SW, Elias B, John R, Teruya J, Fraser CD Jr.

J Thorac Cardiovasc Surg. 2019 Apr;157(4):1609-1617.e2. doi: 10.1016/j.jtcvs.2018.12.045. Epub 2018 Dec 27.

PMID: 30770106

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  1. Outcomes related to immediate extubation after stage 1 Norwood palliation for hypoplastic left heart syndrome.

Varghese J, Hammel JM, Ibrahimiye AN, Siecke R, Bisselou Moukagna KS, Kutty S.

J Thorac Cardiovasc Surg. 2019 Apr;157(4):1591-1598. doi: 10.1016/j.jtcvs.2018.10.153. Epub 2018 Nov 24.

PMID: 30665762

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Select item 30294866

 

  1. Transthoracic Echocardiographic Evaluation of Pulmonary Valve Anomalies in Pediatric Patients.

Wang SS, Xu MG, Zhuang J, Li WB, Zhang ZW, Xu G.

J Ultrasound Med. 2019 Apr;38(4):1091-1096. doi: 10.1002/jum.14780. Epub 2018 Oct 7.

PMID: 30294866

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  1. Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease.

Foulks MG, Meyer RML, Gold JI, Herrington CS, Kallin K, Menteer J.

Pediatr Cardiol. 2019 Apr 1. doi: 10.1007/s00246-019-02093-4. [Epub ahead of print]

PMID: 30937501

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  1. Outcomes of Prosthetic Valved Conduits for Right Ventricular Outflow Tract Reconstruction.

Chen H, Shi G, Qiu L, Wang S, Chen H, Xu Z.

Pediatr Cardiol. 2019 Apr;40(4):848-856. doi: 10.1007/s00246-019-02081-8. Epub 2019 Mar 18.

PMID: 30887063

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  1. Does Coronary Reimplantation After Neoaortic Reconstruction Increase Aortic Regurgitation?

Choi KH, Sung SC, Kim H, Lee HD, Kim G, Ko H, Byun JH, Lee YS.

Pediatr Cardiol. 2019 Apr;40(4):813-819. doi: 10.1007/s00246-019-02075-6. Epub 2019 Feb 19.

PMID: 30778636

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  1. Size and Stiffness of the Pulmonary Autograft after the Ross Procedure in Children.

Ando Y, Ochiai Y, Tokunaga S, Hisahara M, Baba H, Miyagi C, Takigawa T.

Pediatr Cardiol. 2019 Apr;40(4):776-783. doi: 10.1007/s00246-019-02064-9. Epub 2019 Feb 7.

PMID: 30734094

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  1. Characteristics and Outcomes of Heart Transplantation in DiGeorge Syndrome.

Woolman P, Bearl DW, Soslow JH, Dodd DA, Thurm C, Hall M, Feingold B, Godown J.

Pediatr Cardiol. 2019 Apr;40(4):768-775. doi: 10.1007/s00246-019-02063-w. Epub 2019 Feb 7.

PMID: 30729260

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Select item 30726509

 

  1. Pulmonary Artery Banding in Post-tricuspid Congenital Cardiac Shunting Defects with High Pulmonary Vascular Resistance.

Kulik TJ, McSweeney JE, Tella J, Mullen MP.

Pediatr Cardiol. 2019 Apr;40(4):719-725. doi: 10.1007/s00246-019-02054-x. Epub 2019 Jan 23.

PMID: 30673799

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Select item 30671646

 

  1. Timing of Fontan Completion in Children with Functionally Univentricular Hearts and Isomerism: The Impact of Age, Weight, and Pre-Fontan Arterial Oxygen Saturation.

Loomba RS, Frommelt PC, Anderson RH, Flores S, Tweddell JS.

Pediatr Cardiol. 2019 Apr;40(4):753-761. doi: 10.1007/s00246-019-02060-z. Epub 2019 Jan 22.

PMID: 30671646

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Select item 30666358

 

  1. How Could Aortic Atresia With Interrupted Aortic Arch Survive? About a Neonatal Repair on Two Ventricles.

Lacour-Gayet F, Kandachar P, Al-Bulushi A, Maddali MM.

Semin Thorac Cardiovasc Surg. 2019 Spring;31(1):118-121. doi: 10.1053/j.semtcvs.2018.07.009. Epub 2018 Jul 20.

PMID: 30031947

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  1. The Congenital Heart Surgeon’s Society Complete Atrioventricular Septal Defect Cohort: Baseline, Preintervention Echocardiographic Characteristics.

Meza JM, Devlin PJ, Overman DM, Gremmels D, Baffa G, Cohen MS, Quartermain MD, Caldarone CA, Pourmoghadam K, DeCampli WM, Fackoury CT, Mertens L.

Semin Thorac Cardiovasc Surg. 2019 Spring;31(1):80-86. doi: 10.1053/j.semtcvs.2018.02.004. Epub 2018 Feb 8.

PMID: 29428621

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Select item 30726517

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Fetal Cardiology Featured Articles

Fetal Cardiology

 

Fetal Cardiology Featured Manuscripts of April 2019

 

Myocardial strain abnormalities in fetuses with pulmonary atresia and intact ventricular septum.

Cohen J, Binka E, Woldu K, Levasseur S, Glickstein J, Freud LR, Chelliah A, Chiu JS, Shah A.

Ultrasound Obstet Gynecol. 2019 Apr;53(4):512-519. doi: 10.1002/uog.19183. Epub 2019 Mar 12.

PMID: 30043402

 

Take Home Points:

 

  • Myocardial strain is a technique to objectively assess fetal ventricular function compared with standard 2D echocardiography.
  • Fetuses with pulmonary atresia/intact ventricular septum have decreased global longitudinal strain of both the left and right ventricles compared with controls
  • Combining LV and RV global longitudinal strain assessments with other. echocardiographic parameters, listed in Table 3, may allow development of a scoring system to predict presence of RV dependent coronary circulation and may provide valuable prognostic information for prenatal counseling.

  

Shelby White

Comment from Dr. Shelby White (Tucson AZ), section editor of Fetal Cardiology Journal Watch:  Pulmonary atresia with intact ventricular septum (PA/IVS) has a wide range of potential outcomes, based in part on the presence of abnormal communications between the right ventricle and the coronary arteries, most severely the presence of right ventricle-dependent coronary circulation (RVDCC).  Myocardial strain analysis has been used in fetal echocardiography as a sensitive way to identify ventricular dysfunction compared with more subjective measures.  This technique uses speckle-tracking to assess myocardial deformation and is reported as a negative number, with a lower absolute value indicating worse strain/worse ventricular function. This study sought to identify differences in myocardial strain in fetuses with PA/IVS compared with controls and in PA/IVS fetuses with and without RVDCC.

57 fetuses with PA/IVS were analyzed, mean gestational age of 26.3 + 5 weeks.  Left ventricular global longitudinal strain (LV-GLS) was significantly decreased in PA/IVS compared with controls, P < 0.001.  Right ventricular global longitudinal strain (RV-GLS) was also significantly decreased in PA/IVS, P < 0.0001; it was also more difficult to measure as only 42 fetuses had images sufficient for speckle tracking.

Among fetuses with PA/IVS, LV-GLS was decreased in those with RVDCC (Table 3).  The only difference in outcomes that was identified was a decreased RV-GLS in fetuses that had single ventricle palliation compared with biventricular repair.

Fetal cardiology and genetics April 2019

 

  1. Modulation of ADAR mRNA expression in patients with congenital heart defects.

Altaf F, Vesely C, Sheikh AM, Munir R, Shah STA, Tariq A.

PLoS One. 2019 Apr 30;14(4):e0200968. doi: 10.1371/journal.pone.0200968. eCollection 2019.

PMID: 31039163 Free Article

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  1. DNAH11 variants and its association with congenital heart disease and heterotaxy syndrome.

Liu S, Chen W, Zhan Y, Li S, Ma X, Ma D, Sheng W, Huang G.

Sci Rep. 2019 Apr 30;9(1):6683. doi: 10.1038/s41598-019-43109-6.

PMID: 31040315

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Select item 31032618

 

  1. Head Biometry in Fetuses with Isolated Congenital Heart Disease.

Graupner O, Koch J, Enzensberger C, Götte M, Wolter A, Müller V, Kawecki A, Herrmann J, Axt-Fliedner R.

Ultraschall Med. 2019 Apr 25. doi: 10.1055/a-0893-7484. [Epub ahead of print] No abstract available.

PMID: 31022734

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  1. Intronic Polymorphisms in Gene of Second Heart Field as Risk Factors for Human Congenital Heart Disease in a Chinese Population.

Wang E, Nie Y, Fan X, Zheng Z, Hu S.

DNA Cell Biol. 2019 Apr 23. doi: 10.1089/dna.2018.4254. [Epub ahead of print]

PMID: 31013439

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Select item 31026592

 

  1. Compound heterozygous Pkd1l1 variants in a family with two fetuses affected by heterotaxy and complex Chd.

Le Fevre A, Baptista J, Ellard S, Overton T, Oliver A, Gradhand E, Scurr I.

Eur J Med Genet. 2019 Apr 23. pii: S1769-7212(18)30904-2. doi: 10.1016/j.ejmg.2019.04.014. [Epub ahead of print]

PMID: 31026592

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  1. Three-dimensional visualisation of the fetal heart using prenatal MRI with motion-corrected slice-volume registration: a prospective, single-centre cohort study.

Lloyd DFA, Pushparajah K, Simpson JM, van Amerom JFP, van Poppel MPM, Schulz A, Kainz B, Deprez M, Lohezic M, Allsop J, Mathur S, Bellsham-Revell H, Vigneswaran T, Charakida M, Miller O, Zidere V, Sharland G, Rutherford M, Hajnal JV, Razavi R.

Lancet. 2019 Apr 20;393(10181):1619-1627. doi: 10.1016/S0140-6736(18)32490-5. Epub 2019 Mar 22.

PMID: 30910324 Free Article

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  1. The Accuracy and Cost-Effectiveness of Selective Fetal Echocardiography for the Diagnosis of Congenital Heart Disease in Patients with Pregestational Diabetes Stratified by Hemoglobin A1c.

Finneran MM, Ware CA, Kiefer MK, Buschur EO, Foy PM, Thung SF, Landon MB, Gabbe SG.

Am J Perinatol. 2019 Apr 16. doi: 10.1055/s-0039-1685490. [Epub ahead of print]

PMID: 30991442

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  1. Loss of function of Kmt2d, a gene mutated in Kabuki syndrome, affects heart development in Xenopus laevis.

Schwenty-Lara J, Nürnberger A, Borchers A.

Dev Dyn. 2019 Apr 13. doi: 10.1002/dvdy.39. [Epub ahead of print]

PMID: 30980591

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Select item 30981213

 

  1. Response to the letter about the article “Prognosis of severe congenital heart diseases: Do we overestimate the impact of prenatal diagnosis?”

Amedro P, Vincenti M.

Arch Cardiovasc Dis. 2019 Apr 11. pii: S1875-2136(19)30073-7. doi: 10.1016/j.acvd.2019.04.001. [Epub ahead of print] No abstract available.

PMID: 30982719

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Select item 30770395

 

  1. Letter in response to the article entitled “Prognosis of severe congenital heart diseases: Do we overestimate the impact of prenatal diagnosis?” by Vincenti et al.

Séguéla PE, Thomas J, Thambo JB.

Arch Cardiovasc Dis. 2019 Apr 9. pii: S1875-2136(19)30071-3. doi: 10.1016/j.acvd.2019.03.002. [Epub ahead of print] No abstract available.

PMID: 30979689

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Select item 30957737

 

  1. Homeobox Genes and Homeodomain Proteins: New Insights into Cardiac Development, Degeneration and Regeneration.

Miksiunas R, Mobasheri A, Bironaite D.

Adv Exp Med Biol. 2019 Apr 4. doi: 10.1007/5584_2019_349. [Epub ahead of print]

PMID: 30945165

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  1. Role of lncRNA uc.457 in the differentiation and maturation of cardiomyocytes.

Zhang Q, Cheng Z, Yu Z, Zhu C, Qian L.

Mol Med Rep. 2019 Apr 4. doi: 10.3892/mmr.2019.10132. [Epub ahead of print]

PMID: 30957182

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Select item 30964835

 

  1. Prognosis of severe congenital heart diseases: Do we overestimate the impact of prenatal diagnosis?

Vincenti M, Guillaumont S, Clarivet B, Macioce V, Mura T, Boulot P, Cambonie G, Amedro P.

Arch Cardiovasc Dis. 2019 Apr;112(4):261-269. doi: 10.1016/j.acvd.2018.11.013. Epub 2019 Feb 2.

PMID: 30722979

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Select item 29572215

 

  1. The Role of Non-Coding RNA in Congenital Heart Diseases.

Dueñas A, Expósito A, Aranega A, Franco D.

J Cardiovasc Dev Dis. 2019 Apr 1;6(2). pii: E15. doi: 10.3390/jcdd6020015. Review.

PMID: 30939839 Free Article

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Select item 30993945

 

  1. Ankyrin repeat domain 1: A novel gene for cardiac septal defects.

Yang Y, Xia Y, Wu Y, Huang S, Teng Y, Liu X, Li P, Chen J, Zhuang J.

J Gene Med. 2019 Apr;21(4):e3070. doi: 10.1002/jgm.3070. Epub 2019 Feb 19. Review.

PMID: 30659708

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Select item 30945638

 

  1. Persistent truncus arteriosus with absent semilunar valve in first trimester.

Yang SH, Li XQ, Yang ZJ, Tian XX, Wei HW.

J Med Ultrason (2001). 2019 Apr;46(2):273-275. doi: 10.1007/s10396-018-00926-y. Epub 2019 Jan 14.

PMID: 30637595

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Select item 30591407

 

  1. Rationale and Design of the FREQUENCY Study: The Fetal Cardiac Registry of Québec to Improve Resource Utilization in Fetal Cardiology.

Noël C, Gagnon MH, Cardinal MP, Guertin O, Déry A, Têtu C, Vanasse A, Roy-Lacroix MÈ, Poder TG, Marelli AJ, Cavallé-Garrido T, Vaujois L, Bigras JL, Dallaire F.

J Obstet Gynaecol Can. 2019 Apr;41(4):459-465.e12. doi: 10.1016/j.jogc.2018.10.009. Epub 2018 Dec 25.

PMID: 30591407

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  1. It’s All About the Foot Pedal: One Small Step for the Obstetric Sonographer, One Big Step for the Prenatal Detection of Congenital Heart Disease.

Sklansky MS, Satou GM, DeVore GR.

J Ultrasound Med. 2019 Apr;38(4):1097-1099. doi: 10.1002/jum.14784. Epub 2018 Aug 31. No abstract available.

PMID: 30171623

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  1. Identifying pathogenic variants in the Follistatin-like 1 gene (FSTL1) in patients with skeletal and atrioventricular valve disorders.

Prakash S, Mattiotti A, Sylva M, Mulder BJM, Postma AV, van den Hoff MJB.

Mol Genet Genomic Med. 2019 Apr;7(4):e00567. doi: 10.1002/mgg3.567. Epub 2019 Feb 5.

PMID: 30722102 Free PMC Article

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Select item 30720078

 

  1. Altered in utero kidney development in newborns with congenital heart disease.

Scholes GB, Zannino D, Kausman JY, Cheung MMH.

Pediatr Res. 2019 Apr;85(5):644-649. doi: 10.1038/s41390-018-0163-0. Epub 2018 Sep 11.

PMID: 30228371

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  1. Fetal heart rhabdomyomatosis: a single-center experience.

Pavlicek J, Klaskova E, Kapralova S, Prochazka M, Vrtel R, Gruszka T, Kacerovsky M.

J Matern Fetal Neonatal Med. 2019 Apr 28:1-231. doi: 10.1080/14767058.2019.1613365. [Epub ahead of print]

PMID: 31032681

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  1. Fetal descending aortic tortuosity with ductal aneurysm.

Karmegaraj B, Rajeshkannan R, Kappanayil M, Vaidyanathan B.

Ultrasound Obstet Gynecol. 2019 Apr 25. doi: 10.1002/uog.20303. [Epub ahead of print]

PMID: 31021025

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  1. Prenatal diagnosis, associated findings and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center.

Gottschalk I, Abel JS, Menzel T, Herberg U, Breuer J, Gembruch U, Geipel A, Brockmeier K, Berg C, Strizek B.

J Perinat Med. 2019 Apr 24;47(3):354-364. doi: 10.1515/jpm-2018-0316.

PMID: 30676006

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Select item 31019217

 

  1. Fetal echocardiographic prediction score for perinatal mortality of tricuspid valve malformation and Ebstein anomaly.

Torigoe F, Ishida H, Ishii Y, Ishii R, Narita J, Kawazu Y, Kayatani F, Inamura N.

Ultrasound Obstet Gynecol. 2019 Apr 22. doi: 10.1002/uog.20302. [Epub ahead of print]

PMID: 31008542

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  1. Prenatal diagnosis of left pulmonary artery sling and review of literature.

Sezer S, Acar DK, Ekiz A, Kaya B, Bornaun H, Aslan H.

Echocardiography. 2019 Apr 9. doi: 10.1111/echo.14325. [Epub ahead of print]

PMID: 30968436

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Select item 31024933

 

  1. A Novel Somatic Variant in HEY2 Unveils an Alternative Splicing Isoform Linked to Ventricular Septal Defect.

Fardon M, Dehaini H, Kamar A, Bitar F, Majdalani M, El-Rassi I, Nemer G, Arabi M.

Pediatr Cardiol. 2019 Apr 6. doi: 10.1007/s00246-019-02099-y. [Epub ahead of print]

PMID: 30955100

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  1. Prenatal Diagnosis and Successful Palliation of Absent Aortic Valve with Hypoplastic Left Heart Syndrome: A Case Report and Review of Literature.

Qasim A, Johnson CB, Aly MA, Aly AM.

AJP Rep. 2019 Apr;9(2):e121-e126. doi: 10.1055/s-0038-1677480. Epub 2019 Apr 9.

PMID: 30972226 Free PMC Article

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  1. Genetic architecture of laterality defects revealed by whole exome sequencing.

Li AH, Hanchard NA, Azamian M, D’Alessandro LCA, Coban-Akdemir Z, Lopez KN, Hall NJ, Dickerson H, Nicosia A, Fernbach S, Boone PM, Gambin T, Karaca E, Gu S, Yuan B, Jhangiani SN, Doddapaneni H, Hu J, Dinh H, Jayaseelan J, Muzny D, Lalani S, Towbin J, Penny D, Fraser C, Martin J, Lupski JR, Gibbs RA, Boerwinkle E, Ware SM, Belmont JW.

Eur J Hum Genet. 2019 Apr;27(4):563-573. doi: 10.1038/s41431-018-0307-z. Epub 2019 Jan 8.

PMID: 30622330

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  1. Comprehensive Evaluation of Fetal Cardiac Ventricular Widths and Ratios Using a 24-Segment Speckle Tracking Technique.

DeVore GR, Cuneo B, Klas B, Satou G, Sklansky M.

J Ultrasound Med. 2019 Apr;38(4):1039-1047. doi: 10.1002/jum.14792. Epub 2018 Oct 2.

PMID: 30280404

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  1. Left pulmonary artery in 22q11.2 deletion syndrome. Echocardiographic evaluation in patients without cardiac defects and role of Tbx1 in mice.

Mastromoro G, Calcagni G, Versacci P, Putotto C, Chinali M, Lambiase C, Unolt M, Pelliccione E, Anaclerio S, Caprio C, Cioffi S, Bilio M, Baban A, Drago F, Digilio MC, Marino B, Baldini A.

PLoS One. 2019 Apr 1;14(4):e0211170. doi: 10.1371/journal.pone.0211170. eCollection 2019.

PMID: 30933971 Free PMC Article

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  1. Myocardial strain abnormalities in fetuses with pulmonary atresia and intact ventricular septum.

Cohen J, Binka E, Woldu K, Levasseur S, Glickstein J, Freud LR, Chelliah A, Chiu JS, Shah A.

Ultrasound Obstet Gynecol. 2019 Apr;53(4):512-519. doi: 10.1002/uog.19183. Epub 2019 Mar 12.

PMID: 30043402

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Congenital Cardiovascular Anesthesia and Critical Care Featured Articles

Congenital Cardiovascular Anesthesia

 

Congenital Heart Anesthesia and Intensive Care Featured Manuscripts January-May 2019

 

Anesthesia for high-risk procedures in the catheterization laboratory.

Daaboul DG, DiNardo JA, Nasr VG.

Paediatr Anaesth. 2018 Dec 28. doi: 10.1111/pan.13571. [Epub ahead of print]

PMID:  30592354

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Take Home Points:

 

  • While reported adverse events in the catheterization lab range between 4-10%, the prevalence of adverse events (4.2%) and all cause-mortality (12%) during the hospital stay is highest for neonates. In addition to the risk of the procedure itself, young age, pulmonary artery hypertension, diastolic dysfunction, low cardiac-output, cyanosis, and low mixed venous saturation may further increase the risk.
  • Safe induction and maintenance of general anesthesia for high-risk cardiac catheterization require an understanding of the pathophysiology of each disease process, the planned procedure, its impact on the hemodynamics, the impact of the various anesthetic and pharmacologic interventions as well as experienced personnel and good communication between team members.

 

Commentary by Laura A. Downey, a pediatric cardiac anesthesiologist at Children’s Healthcare of Atlanta/Emory University:  Recent advances in technology allow for more complex procedures to be performed in smaller patients in the cardiac catheterization laboratory. Multiple cardiology registries have been created to assess adverse events and assign risk scores to these patients.  However, none of the developed scores specifically address anesthetic risk.  Therefore, these authors discuss perioperative procedure and anesthetic-related risk assessment as well as the anesthetic approach for the management of neonates undergoing specific high-risk procedures in the cardiac catheterization laboratory.

 

The authors discuss several registries that cardiologists have used to risk stratify patients presenting to the catheterization lab, including two different risk scores. One such prediction model for high-severity adverse events is The Catheterization for Congenital Heart Disease Adjustment of Risk Method (CHARM), which uses procedure risk category, number of variables of hemodynamic vulnerability, and age <1 year. Incorporating the procedure risk group and hemodynamic variable in CHARM, the large IMPACT dataset was used to create a more generalizable model to predict major adverse events that included: procedure risk group, four hemodynamic vulnerability variables, age, the presence of renal insufficiency, and single ventricle physiology.   Concurrent with the development of the CHARM model, the Congenital Cardiac Interventional Study Consortium (CCISC) group developed the Catheterization Risk Score for Pediatrics (CRISP).  This scoring system assigns patients into one of five CRISP categories that assigns points based on weight, age, inotropic support/extracorporeal membrane oxygenation (ECMO), systemic illness/organ failure, physiological status, pre-catheterization diagnosis, procedure type, and the procedure performed.  The risk of serious adverse events ranges from 1% for CRISP 1 and 36.8% for CRISP 5.

Despite the above models, neither of these developed scores specifically address anesthetic risk.  Recently the C3PO database determined a 1.83% risk of overall sedation/airway related events and only 0.14% category 4 life threatening events and 0.014% category 5 catastrophic events (two cardiac arrests). Low patient weight (<4kg), lowed mixed venous saturation (single ventricles <50%, two ventricles < 60%), and the presence of additional non-cardiac comorbidities were correlated with high severity of sedation or airway anesthesia related complications.  In light of these statistics and a paper that demonstrated the involvement of cardiac anesthesiologists lower the incidence of cardiac arrest from 3.5% to 0.7%, a 2016 expert consensus statement recommended that anesthesiologists with advanced skills and understanding of the pathophysiology and expertise in management of patients with CHD provide care for high-risk patients.

The authors discuss the importance of preparing for the unique challenges that anesthesiologists face in the cardiac catheterization laboratory: limited access to patients due to small workspaces and equipment, poor lighting, radiation exposure, offsite location, and potential difficulty in getting help fast. Low ambient temperatures and the frequent flushing of catheters and sheaths may result in hypothermia and volume load, especially in infants. Due to catheters crossing through the heart, risks include dysrhythmias, bleeding from perforation of heart structures or vessels, damage to valves, stroke and risk of air embolism.  In order to prepare for a variety of potential complications, the authors recommend adequate IV access, readily available resuscitation drugs and defibrillation pads, easily accessible blood (the authors recommend cross-matched blood, however other institutions may use O-negative blood), and ECMO support if necessary.

Based on factors in the CRISP and CHARM models that predict adverse events, these authors specifically discuss perioperative concerns and anesthesia technique for interventional procedures performed in patients <1 year of age that present the highest risk of adverse events due to procedure and anesthesia-related risks: 1) Closure of a patent ductus arteriosus (PDA); 2) Ductal stent; 3) right ventricular outflow tract (RVOT) stenting; 4) Pulmonary atresia with intact ventricular septum (PA/IVS); 5) Valvotomy in critical aortic stenosis (AS).

 

  1. Patent Ductus Arteriosus (PDA) Closure

 

These authors summarize the recent literature regarding PDA device closure, specifically focusing on recent studies that demonstrate up to 88% success of PDA occlusion in preterm and low birth weight infants (mean age 30 days, mean weight 1249g) with very low complication rates.  Although relatively safe, important complications include device embolization, ductal spasm, residual shunts, left pulmonary artery obstruction, development of aortic coarctation, and vascular injury. Ductal spasm, occurring in 2.1%-3.7% of closures, may result in failure of the procedure due to undersizing of the device and risk of embolization.  For anesthesiologists, device embolization and retrieval may present the most challenging complication of this procedure, including possible hemodynamic instability, valvular damage, vascular injury, or emergent surgical intervention with cardiopulmonary bypass.

 

  1. Ductal Stenting

 

Traditionally achieved surgically with a Blalock Taussig (BT) shunt, creating a reliable source of pulmonary blood can now be achieved in the cardiac catheterization laboratory with ductal stenting. When ductal stenting is compared to a BT shunt, patients receiving a ductal stent had lower complication rates, shorter length of stay (LOS) in the intensive care unit (ICU), and more symmetrical pulmonary artery (PA) growth (Glatz et al), while a similar study found shorter mechanical ventilation times, shorter ICU LOS, and less ECMO.  Both studies found that ductal stent patients had a higher rate of reintervention, unrelated to cyanosis. Important anesthetic considerations for patients receiving ductal stents, include appropriate discontinuation of prostaglandin E2 to allow the ductus to be small enough to wedge the stent in place, but not so small that pulmonary blood flow (PBF) is compromised.  During the procedure, anesthesiologists should be prepared to support hemodynamic changes during complete or partial occlusion of the ductus due to catheter manipulation or ductus spasm.  Occasionally, the stent may not be able to be deployed or hypoxemia persists despite stent deployment and surgical intervention to place a shunt may be required.

 

  1. RVOT Stenting

 

Advances in the catheterization lab make RVOT stenting and balloon dilation of the pulmonary valve a reasonable alternative for patients with RVOT obstruction (i.e. TOF patients).  This approach is most beneficial in premature or low birth weight patients, who have the highest operative morbidity and mortality. When compared to a BT shunt, the RVOT stent group had shorter time to surgical repair (232 days versus 428 days), lower intensive care admission rate (22% versus 100%) and shorter length of stay (median 7 days versus 14 days). Procedure-related complications include perforation of the pulmonary artery or RVOT leading to hemothorax and pericardial effusion, stent embolization, or tricuspid valve regurgitation. Anesthesiologists should be prepared for possible hemorrhage due to RVOT or PA perforation with immediate access to red blood cells and adequate IV access. Transient pulmonary edema may occur during the procedure due to the sudden increase in pulmonary blood flow.  Pulmonary edema should be treated with positive end-expiratory pressure (PEEP) and diuretics and admission to the ICU should be considered.

 

  1. Pulmonary Atresia with Intact Ventricular Septum

 

Patients with PA/IVS require PGE2 to maintain ductal patency and pulmonary blood flow.  These patients are often taken to the catheterization laboratory early to determine if the patient has RV-dependent coronary circulation (RV-DCC).  In patients without RV-DCC, the pulmonary valve can be opened with radiofrequency (RF) perforation and stented.  RF of the pulmonary valve is contraindicated in patients with RV-DCC who require PDA stent placement, BT shunt placement, or transplantation.  Mortality is estimated at 6.69%.  As these patients are at risk for sub-endocardial ischemia, RV preload and systemic diastolic pressure must be adequate to maintain coronary perfusion.  Common complications are similar to patients undergoing RVOT stenting, including perforation of the PA/RVOT. Atrial and ventricular arrhythmias and heart block are likely related to coronary ischemia. Anesthesiologists must, therefore, be prepared for emergent transfusion or cardioversion in these procedures.  In the setting of borderline RV function, pulmonary blood flow may not be sufficient and PGE2 will need to be started.

 

  1. Critical Aortic Stenosis

 

Of the 6% of patients with left ventricular outflow obstruction (LVOTO), aortic stenosis accounts for approximately 80% of these cases. Neonates with critical AS usually present with signs of hypoperfusion, cyanosis, and lethargy as the ductus begins to close.  Thus, prostaglandins are started in order to maintain ductal patency and systemic perfusion. Balloon valvotomy has been shown to decrease the aortic gradient by 50%, but with a 15% risk of aortic regurgitation (AR).  Many of these patients will present for surgical intervention for AR or re-intervention for recurrent stenosis.  Procedure risks include arrhythmias and perforation.  During balloon dilation, cardiac arrest may occur due to cessation of cardiac output. In addition to preparation for hemorrhage, arrhythmias, or cardiac arrest, the goal of the anesthetic is to maintain coronary perfusion and cardiac output.  The authors recommend an opioid-based anesthetic with readily available inotropic support, such as epinephrine.

 

As demonstrated by the above discussion, safe induction and maintenance of general anesthesia for high-risk cardiac catheterization require an understanding of the pathophysiology of each disease process, the planned procedure, its impact on the hemodynamics, the impact of the various anesthetic and pharmacologic interventions as well as experienced personnel and good communication between team members.

 

Positive Airway Pressure Versus High-Flow Nasal Cannula for Prevention of Extubation Failure in Infants After Congenital Heart Surgery.

Richter RP, Alten JA, King RW, Gans AD, Rahman AF, Kalra Y, Borasino S.

Pediatr Crit Care Med. 2019 Feb;20(2):149-157. doi: 10.1097/PCC.0000000000001783.

PMID:  30407954

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Take Home Points:

 

  • Prevention of post-extubation failure after congenital cardiac surgery in neonates and infants should be a high priority as it is associated with significant morbidity and mortality.
  • The optimal strategy for mitigating post-extubation failure [high flow nasal cannula (HFNC) versus non-invasive positive airway pressure (PAP)ventilation] is not well established.
  • In this retrospective, single institution, propensity score matched cohort study comparing extubation to HFNC versus PAP, there was no difference in post-extubation failure rates. However, extubation to PAP was associated with greater resource utilization, longer times to transition to low-flow nasal cannula and then room air, and longer postsurgical hospital stay.

Commentary from Dr. Sana Ullah, a pediatric cardiac anesthesiologist at UT Southwestern Medical Center in Dallas, Texas:  Extubation failure rate is around 6-9% for children and around 17% for neonates after cardiac surgery, and is associated with increased morbidity and mortality. There is no consensus or evidence-based optimal strategy for preventing extubation failure in this setting. Typically, patients are extubated to nasal cannula (high flow nasal cannula, HFNC, low-flow nasal cannula, LFNC), or some form of non-invasive ventilation (NIV), such as nasal CPAP with or without pressure support. The exact modality is based on clinician choice, with the NIV method being perceived as providing more support for higher risk patients. There is very little prospective evidence-based data to help decision-making.

In this single-institution, retrospective, matched cohort study, the authors describe the impact of post-extubation respiratory support on patient outcomes and resource utilization. Patients under 6 months of age admitted to the cardiac  intensive care unit (ICU) at Children’s of Alabama hospital after cardiac surgery using cardiopulmonary bypass were included, with those requiring pre-operative tracheostomy, post-operative extracorporeal membrane oxygenation (ECMO),  limited life-sustaining therapy, and/or post-operative death prior to extubation being excluded.

The respiratory modalities were as follows: Positive airway pressure (PAP) included CPAP with or without pressure support or BiPAP. HFNC oxygen support was given at 3 L/min or greater. LFNC was defined as less than 2 L/min of oxygen.  The choice of support was at the discretion of the ICU attending. HFNC was started at 3-6 L/min for neonates and 3-8 L/min for infants, increasing to 8 L/min for neonates and 12 l/min for infants before declaring failure. PAP was initiated with BiPAP, starting with an inspired (iPAP) of 20 cmH2O, expiratory PAP (ePAP) of 10 cmH2O and a rate of 20-30. This was increased to iPAP of 25, ePAP of 15, and rate of 30 before declaring failure. The FiO2 was titrated for all modalities as deemed appropriate by the ICU clinician. Treatment failure was determined by clinical signs (tachypnea, grunting, accessory muscle use, irritability) and evidence of low cardiac output (declining renal NIRS, increasing arteriovenous oxygen and/or CO2 gradient, and/or increasing serum lactate).

There was no set protocol for weaning support. HFNC was typically weaned by the bedside nurse, and PAP was weaned based on respiratory assessment by the respiratory therapist and the clinician according to clinical progression. PAP was generally weaned in a step-wise fashion from BiPAP to CPAP, then to HFNC. Extubation failure was defined as need for reintubation within 48 hours of extubation.

The results are interesting: Out of 245 patients, overall extubation failure rate was 12%, and in the matched cohort, there was no significant difference between the HFNC and PAP groups. Fifteen of the HFNC patients (31%) required rescue PAP at a median of 10 hours after extubation. Three of the patients who received rescue PAP failed extubation at a median of 28 hours. The PAP group (not surprisingly) took longer to wean to both LFNC and then room air, and feeding was delayed by almost 4 days. Perhaps because of this, the PAP group also had a higher rate of G-tube insertions for feeding. There was also increased post-surgical hospital length of stay. And not surprisingly, the PAP group had greater resource utilization in terms of equipment, personnel, lab draws, and sedation.

A major problem with studies like this is selection bias for a particular treatment. In this study, there would be clear selection bias towards clinicians choosing PAP technique in patients they perceived to be at a higher risk of extubation failure. To reduce this bias, the investigators used propensity scores to generate 49 matched pairs where there was no significant difference in patient demographics or treatment factors between the two groups.

Based on this study, despite using propensity scores to minimize selection bias, it suggests that extubating to PAP ventilation using non-invasive techniques may be disadvantageous compared with the much simpler HFNC. However, the decision to select a particular strategy for respiratory support after extubation is quite complex. There are objective parameters such as the respiratory and hemodynamic data, but more so, there are many subjective criteria that the ICU clinician will consider in deciding the optimal support strategy. These include the post-surgical progress and the perceived clinical trajectory of the patient, age and complexity of the operation, possible complications that may have an impact on respiratory function such as vocal cord or diaphragm paresis, sedative and analgesic medications, the time of day as far as weaning support (aggressive weaning may be avoided at night), variability between clinicians, and the paucity of objective criteria on when exactly to intervene when extubation is deemed a failure. Once a more aggressive strategy is begun (i.e. PAP), there is a natural tendency to progress more cautiously as far as weaning support. Extubation failure and subsequent reintubation is associated with more complications, and so there is a strong desire to avoid the “one step forward, two steps back” scenario. Ultimately, until more prospective data becomes available, the choice of post-extubation respiratory support strategy will depend mostly on the bedside clinician taking into account the myriad of patient factors that will have an impact on the eventual success or failure of that strategy.

 

High-energy nutrition in paediatric cardiac critical care patients: a randomized controlled trial.

Zhang H, Gu Y, Mi Y, Jin Y, Fu W, Latour JM.

Nurs Crit Care. 2019 Mar;24(2):97-102. doi: 10.1111/nicc.12400. Epub 2018 Dec 9.

PMID:  30548121

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Take Home Points:

 

  • Infants with congenital heart disease are at high risk for malnutrition. The immediate postoperative period can worsen malnutrition and increase mortality in infants and children due to increased energy requirements, inadequate calorie intake, intestinal malabsorption, and fluid restriction.
  • This study was aimed at assessing efficacy and safety of feeding high-energy formula (HF) to infants with congenital heart disease in the early postoperative period after cardiac surgery. The authors found that infants fed HF gained more weight but had higher rates of feeding intolerance. Feeding intolerance was relieved with medication and did not deter feed advancement.

Commentary from Dr. Anne Elisa Cossu, a practicing pediatric cardiac anesthesiologist at Riley Hospital for Children in Indianapolis, IN:  Several clinical studies have been designed to investigate the effects of feeding HF to infants with congenital heart disease (CHD).  The results have shown that HF increases caloric intake, however, there is not clear evidence as to the effect of HF on weight gain and gastrointestinal function.   Studies have suggested a resting energy expenditure of 40-60 kcal/kg/day in CHD patients.  The authors’ aim of the current study was to evaluate safety and efficacy of HF during the early postoperative period after cardiac surgery in infants with CHD.

The study was a randomized, controlled trial that divided patients into two groups—an intervention group and a control group.  Inclusion criteria were the following: diagnosis of CHD based on symptoms, ultrasound and imaging; < 1 year of age; and parent agreement to cardiac surgery. Exclusion criteria were the following: diseases that cause nutritional disorders, preoperative gastrointestinal intolerance, use of total parenteral nutrition after surgery, or length of stay in the CICU predicted to be < 5 days. Feeds were begun post-operatively, and patients in the intervention group received HF (51-89 kcal/kg/day) whereas the control group received standard-energy formula (SF) (44-56 kcal/kg/day) for an interval of 7 days. The primary outcome measures were weight gain in grams by the seventh day of feeding and feeding intolerance from the first day to the seventh day after starting enteral feeding. Feeding intolerance was considered to be present when infants had any of the following symptoms: vomiting three or more times per day, abdominal distension (abdominal circumference increased by more than 10%), formula volume decrease or lack of increase for 3 days, gastric residual volume greater than one third of the previous feed, greater than two unscheduled feeding breaks or diarrhea.  Secondary outcomes included the following: prealbumin (mg/L) level within 24 hours of surgery and at 3 and 7 days postop; enteral energy intake during feeding with HF or SF; and duration of mechanical ventilation, CICU length of stay, hospital length of stay and number of participants who developed necrotizing enterocolitis.

A total of 59 infants completed the trial (intervention group n=30 and control group n=29).  The two groups did not differ significantly in baseline characteristics. Infants who received HF had less weight loss as compared to the SF group.   The HF group experienced significantly more gastrointestinal intolerance in contrast to the SF group.  Nine infants in the HF group were administered drugs to improve gastrointestinal function versus 6 in the SF group. In terms of secondary outcomes, serum prealbumin levels gradually increased in the HF group but declined in the SF group. Enteral nutrition energy intake of both groups gradually increased, but more rapidly in the HF group.  There were no statistical differences in the other secondary outcomes between the HF and SF groups.

Previous studies have established improved clinical outcomes when enteral nutrition is provided in the early postoperative period.  Protocols for early feeding can promote these practices, which was a strength of the current study. Body weight decreased in all study participants, however, began to rebound 4-5 days after intervention due to adequate caloric intake.  The exact dose-response relationship between energy intake and weight gain remains unclear.  Infants in the intervention group (HF) had increased serum albumin levels compared to infants in the control (SF) group who had decreased albumin levels, which may indicate better nutritional status of the infants in the HF group. Limitations of the study included reporting bias in outcome indicators such as abdominal distension, short intervention time of 7 days, and limited time for collecting follow-up data. Additionally, weight gain may not be a reliable indicator of nutritional status as it may only indicate generalized edema. Ultimately, the authors drew the conclusion that HF enteral feeding might increase energy intake, reduce weight loss and improve nutritional status but may also cause gastrointestinal intolerance.  More studies are needed to confirm safety and efficacy of HF.

 

CHD Anesthesia and critical care April 2019

 

  1. Efficacy of different analgesic or sedative drug therapies in pediatric patients with congenital heart disease undergoing surgery: a network meta-analysis.

Liu RZ, Li BT, Zhao GQ.

World J Pediatr. 2019 Apr 23. doi: 10.1007/s12519-019-00252-4. [Epub ahead of print]

PMID: 31016566

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  1. Central Venous Catheter-Related Deep Vein Thrombosis in the Pediatric Cardiac Intensive Care Unit.

Steen EH, Lasa JJ, Nguyen TC, Keswani SG, Checchia PA, Anders MM.

J Surg Res. 2019 Apr 22;241:149-159. doi: 10.1016/j.jss.2019.03.052. [Epub ahead of print]

PMID: 31026793

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Select item 31005985

 

  1. Effects of Dexmedetomidine on Myocardial Repolarization in Children Undergoing General Anesthesia: A Randomized Controlled Trial.

Görges M, Sherwin ED, Poznikoff AK, West NC, Brodie SM, Whyte SD.

Anesth Analg. 2019 Apr 8. doi: 10.1213/ANE.0000000000004135. [Epub ahead of print]

PMID: 30985379

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Select item 30974093

 

  1. Comparison of chloral hydrate and pentobarbital sedation for pediatric echocardiography.

Ganigara M, Srivastava S, Malik P, Fong S, Ko H, Parness I, Shenoy R.

Echocardiography. 2019 Apr;36(4):766-769. doi: 10.1111/echo.14301. Epub 2019 Feb 25.

PMID: 30803010

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  1. Complications of Transthoracic Intracardiac and Central Venous Lines in Neonates Undergoing Cardiac Surgery.

Stein ML, Quinonez LG, DiNardo JA, Brown ML.

Pediatr Cardiol. 2019 Apr;40(4):733-737. doi: 10.1007/s00246-019-02057-8. Epub 2019 Jan 30.

PMID: 30701276

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The CHIP Network Journal Watch Team

Konstantin Averin, MD, MS is an Associate Professor of Pediatrics at the University of Alberta and an Interventional Pediatric Cardiologist at the Stollery Children’s Hospital in Edmonton, Alberta. He received his medical degree from the Feinberg School of Medicine at Northwestern University in Chicago, IL. After medical school, he completed pediatrics residency, pediatric cardiology fellowship and sub-specialty training in pediatric and adult interventional cardiology at the Cincinnati Children’s Hospital in Cincinnati, OH. His clinical and research interests are focused on the percutaneous treatment of pediatric patients with congenital and acquired heart disease with a focus on patients with single ventricle physiology, transcatheter pulmonary valves, and pulmonary hypertension.

Dr. Leong Ming Chern “MC” is an adult congenital heart disease specialist at the National Heart Institute, Kuala Lumpur, Malaysia. He received his medical training at the University of Malaya and pediatric cardiology training at the National Heart Institute. His area of interest includes treatment of adult patients with congenital heart disease and pulmonary hypertension in congenital heart disease.

Dr. Damien Cullington, MBChB MRCP MD FESC is a consultant adult congenital cardiologist who works at Leeds General Infirmary, UK. In summer 2019, he will move over to the newly commissioned North West ACHD Service based in Liverpool, UK. Damien qualified in 2002 from the University of Liverpool and became a substantive consultant in 2016. Prior to this, he worked throughout the North of England and Yorkshire as a cardiology trainee. Damien was awarded a doctorate in medicine (MD) from the University of Hull in 2013 for his work in heart rate in patients with heart failure. He is a member of the Royal College of Physicians (London), Fellow of the European Society of Cardiology and honorary senior lecturer for the University of Leeds. His ACHD subspecialty clinical interests are heart failure, imaging and palliative care. He is regional organiser for ACHD training at Leeds and clinical governance lead for the Leeds congenital cardiac unit. His research interests and wide and far but particularly epidemiology, chronic heart failure in ACHD patients in all its guises, the systemic RV and the univentricle

Dr. Blanche Cupido is a consultant adult cardiologist working at Groote Schuur Hospital, UCT Private Academic Hospital and the University of Cape Town(UCT), South Africa. She completed her physician training in 2009 and her cardiology subspecialist training in 2013. She recently returned to SA after doing a Fellowship in Adult Congenital Heart Disease in Leeds, United Kingdom under the guidance of Dr Kate English. She has established a dedicated unit for Grown Up Congenital Heart Disease in Cape Town, South Africa. Her aim is to grow ACHD services in Sub-Saharan Africa and embark on GUCH research on the African continent.

Dr. Jeremy L. Herrmann is an Assistant Professor of Surgery in the Division of Thoracic and Cardiovascular Surgery at Indiana University. He specializes in pediatric and adult congenital cardiac surgery, and his clinical interests also include heart transplantation and mechanical circulatory support. His hospital affiliations include Indiana University Health Riley Hospital for Children and Methodist Hospital as well as Peyton Manning Children’s Hospital at St. Vincent Hospital in Indianapolis

Maan Jokhadar is a cardiologist and associate professor of medicine at Emory University in Atlanta, Georgia. He is board certified in internal medicine, cardiovascular disease, advanced heart failure/transplantation, adult congenital heart disease, and echocardiography. He is fellowship director for the Emory Adult Congenital Heart Disease training program and Core Curriculum Director for the Emory general cardiology fellowship program. Dr Jokhadar graduated from the University Damascus School of Medicine in Syria and then went to Mayo Clinic in Rochester, Minnesota for internal medicine residency. He then completed cardiology and subspecialty training at Emory University, where he currently on faculty. Dr. Jokhadar has been the recipient of numerous teaching awards. He is married with 3 children.

Michael Ma, MD is an Assistant Professor in Cardiothoracic Surgery in the Division of Pediatric Cardiac Surgery at Stanford University. He specializes in pediatric and adult congenital cardiac surgery, with an emphasis on neonates, complex biventricular repair, and pulmonary artery reconstruction. His research lab investigates ex and in vivo translational models for complex congenital heart disease, to optimize future surgical and endovascular repair strategies.

Jeremy P. Moore MD MS FHRS is the Director of Clinical Research and faculty in Pediatric Cardiology and Adult Congenital Heart Disease at the University of California, Los Angeles. Dr. Moore received his medical degree from the Medical College of Virginia in 2003. He completed residency and fellowship at UCLA before pursuing his subspecialty training in Pediatric Electrophysiology at Vanderbilt University in 2009. Dr. Moore has been faculty at UCLA since 2010 and has since published numerous research manuscripts dealing with electrophysiologic aspects pertinent to congenital heart disease. Dr. Moore’s primary interest is the study of mechanisms of arrhythmia, and the development of novel electrophysiologic techniques for management of the adult patient with congenital heart disease

Dr. Mehul Patel MD, is a structural and adult congenital heart disease specialist and an interventional cardiologist. Dr. Patel earned his undergraduate and medical education from the Mumbai University, India. After completing his post-graduate training in Internal Medicine and Cardiology, he further trained in interventional cardiology at the Mount Sinai Medical Center, NYC, NY and Adult Congenital Heart Disease at the Texas Children’s Hospital, Texas Heart Institute, Baylor College of Medicine, Houston, TX. Dr. Patel worked as Chief of adult congenital heart disease, Assistant Professor at the Michigan State University, Grand Rapids, MI where he not only expanded the program, performing complex interventions and device implantations but also established the percutaneous pulmonary valve implantation (Melody Valve) Program. Due to his passion for treating structurally abnormal hearts, he did a dedicated year of Structural Heart Disease fellowship at Henry Ford Hospital, Detroit, MI where he worked with pioneers in this field before moving to North Carolina. He is proficient in performing transcatheter aortic valve replacement (TAVR), MitraClip, Watchman device implantation, percutaneous Mitral, Tricuspid and Pulmonary valve replacements along with a variety of interventions on congenital heart disease and pulmonary hypertension. Dr. Patel is ABIM board certified in Internal Medicine, Cardiology, Interventional cardiology and Adult Congenital Heart Disease. Dr. Patel has more than 50 peer reviewed publications and numerous abstracts to his credit. He serves as a Co-Editor-in-Chief for the CHiP Network journal watch. His wife, Khyati is a pediatric cardiac Electrophysiologist and they have a 9-year-old daughter. Dr. Patel is also an artist, likes music, yoga and plays badminton. His clinical areas of expertise and interests are: 1) Transcatheter therapies for Structural and Adult Congenital heart disease. 2) Complex device implantations.

Dr. Timothy Pirolli is an Assistant Professor of Surgery in the Division of Pediatric Cardiothoracic Surgery at University of Texas Southwestern Medical Center. He specializes in pediatric and adult congenital cardiac surgery. His hospital affiliations include Children’s Medical Center in Dallas, Parkland Memorial Hospital, and Clements University Hospital (UTSW).

Dr. Inga Voges, M.D. Consultant in Pediatric Cardiology, Lead Consultant Cardiovascular MRI. I trained in General Pediatrics and Pediatric Cardiology in Rostock (Germany) and Kiel (Germany) from 2002-2010 and did additional training in Adult Congenital Heart Disease (ACHD) in Kiel (Germany) which I finished in 2014. I specialized in Cardiovascular Magnetic Resonance Imaging (CMR) and have a further special interest in cardiomyopathies. Currently, I am working as a consultant pediatric and ACHD cardiologist at the University Hospital Schleswig-Holstein (Kiel, Germany) where I am responsible for the CMR imaging program in pediatric and ACHD patients and contribute to the care of patients with acquired and congenital heart disease. I received my MD from the Medical University Luebeck in 2006 (Germany) and finished my “Habilitation” (PhD equivalent; University Hospital Kiel, Germany) in 2014. Since 2016, I am the secretary of the Association for European Paediatric and Congenital Cardiology Imaging Working Group.

Dr. Gary Webb, M.D. is an Emeritus Professor of Pediatrics and Internal Medicine at the University of Cincinnati College of Medicine and,
from 2009-2016, the Director of the Adult Congenital Heart Program at Cincinnati Children’s Hospital Heart Institute. A graduate of McGill
University in Montréal, he interned at the Royal Victoria Hospital, and then trained in internal medicine and cardiology at the University of Toronto. From 1980-2004, he was co-director and then director of the Toronto Congenital Cardiac Center for Adults at Toronto General Hospital. He is a Fellow of the Royal College of Physicians and Surgeons of Canada in both internal medicine and cardiology. From 2004-2009, he was director of the Philadelphia Adult Congenital Heart Center at the University of Pennsylvania. Since 2016, he has been a consultant to Cincinnati Children’s Hospital, and has been responsible for the ACHD Learning Center, the Cardiology Fellow Testing Center, and the Congenital Heart Professionals International (CHIP) Network. Since 2017, he has resumed seeing patients on a part-time basis at the Toronto Congenital Cardiac Centre for Adults.

Wendy Whiteside, MD Wendy is Assistant Professor of Pediatrics and Associate Director of Interventional Pediatric Cardiology at the University of Michigan Congenital Heart Center, C.S. Mott Children’s Hospital. She obtained her medical degree from Albert Einstein College of Medicine in 2006 then completed pediatric residency at Children’s Hospital Oakland in Oakland, CA in 2009. She received
both her categorical and interventional pediatric cardiology training at the University of Michigan in Ann Arbor, MI. Her clinical and research interests include single ventricle physiology, transcatheter
pulmonary valves, and quality improvement within the cardiac catheterization laboratory.

About the Congenital Heart and Pediatric Cardiac Journal Watch

Congenital heart and pediatric cardiac Journal Watch was designed to make it easier for congenital heart and pediatric cardiac professionals to keep up with the literature in 6 subsections of congenital heart disease abstracts on a monthly basis: pediatric cardiology; congenital heart surgery; congenital  heart interventions; congenital/pediatric electrophysiology; fetal cardiology; and adult congenital heart disease.

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Sincerely,

Dr. Gary Webb, MD and Dr. Mehul Patel
CHiP Network
Congenital Heart and Pediatic Cardiac Journal Watch Editorial Board

The Chip Network, the Congenital Heart International Professionals Network, aims to develop a single global list of all congenital and pediatric cardiac professionals.

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