CHiP Network Congenital Heart Journal Watch – June / July 2020

Pediatric Cardiology Featured Articles

Pediatric Cardiology Reviews of April 2020 Manuscripts

 

  1. Pediatric Cardiology Reviews of April 2020

     

    1. Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy.

    El Assaad I, Gauvreau K, Rizwan R, Margossian R, Colan S, Chen MH.

    J Am Soc Echocardiogr. 2020 Apr 9. pii: S0894-7317(20)30067-5. doi: 10.1016/j.echo.2020.01.020. [Epub ahead of print]

    PMID: 32279939

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    Take Home Points

     

    • Exercise stress echocardiography (ESE) is a safe and feasible modality in children with hypertrophic cardiomyopathy.
    • In children without rest LVOT gradient 44% develop gradients > 30 mm Hg with exercise.
    • ESE-derived rest and exercise gradients correlated with risk of cardiac outcome.
      • Children with LVOT gradients <30 mm Hg have lowest risk of CV events.
      • Children with LVOT gradients ≥30 mm Hg have 5 times the risk of CV events.

     

     

    Comment from Dr. Jennifer Johnson (Pittsburg, PA), Section Editor of Pediatric Cardiology Journal Watch:   This is a single center retrospective chart review of all pediatric hypertrophic cardiomyopathy patients who underwent exercise stress echocardiography to determine if exercise stress echocardiography can be useful in risk stratifying hypertrophic cardiomyopathy subgroups and if LVOT obstruction occurs in the patients with nonobstructive hypertrophic cardiomyopathy.

     

    Methods:  Data was collected on all pediatric hypertrophic cardiomyopathy patient who underwent exercise stress echocardiography from 2007-2018 at Boston Children’s Hospital.  Subjects were assigned to one of three categories based on left ventricular outflow tract gradients: group 1: <30 mm Hg at rest and exercise; group 2: <30 mm Hg at rest and ≥30 mm Hg with exercise; and group 3: ≥ 30 mm Hg at rest and exercise. The composite adverse endpoints on follow-up included heart transplant, aborted cardiac arrest, and sudden cardiac death.

     

    Results:  A total of 91 (61% male), median age 12 years (6-24 years) with hypertrophic cardiomyopathy underwent exercise stress echocardiography; baseline patient characteristics are described in table 1.  Median left ventricle wall thickness was 20 mm and median follow-up duration was 3 years. During ESE, only one child experienced an event and was resuscitated. Of the 91 children, 25 were classified as group 1, 40 as group 2, and 26 as group 3. Twenty-six patients met the composite endpoint, including two heart transplant, one aborted cardiac arrest, and one sudden cardiac death. Group 3 patients had a 5 times higher risk of developing symptoms and/or serious clinical outcome at any age (hazard ratio = 5.18; 95% CI, [1.39-19.2]; P = .014). During our short follow-up time, group 2 patients had a higher risk of outcome, but this did not achieve statistical significance (hazard ratio = 1.95; 95% CI, [0.5-7.6]; P = .33).

     

    Exercise stress echocardiography data; table 2.  Of the 40 patients in group 2 (resting LVOT <30 mmHg, exercise LVOT gradient ³ 30 mmHg) 20 had obstruction due to septal hypertrophy/systolic anterior motion, 13 patients mid cavitary obstruction and other 7 patients had a mixed obstruction etiology.

     

     

     

     

    Of the total cohort, 73 (80%) subjects  were free of cardiac events prior to the first exercise stress echocardiogram study.  In the 3 year follow up data 26 patients had 31 cardiac events.

     

     

    Discussion: In this cohort, 90 (99%) patients had an event-free exercise stress echocardiogram with one group 3 patient experiencing a fast-polymorphic ventricular fibrillation arrest requiring resuscitation during exercise stress test.  This data showed pediatric exercise stress echocardiogram for hypertrophic cardiomyopathy can be performed safely with low risk to the patients.

     

     

     

    Environmental and Socioeconomic Factors Influence the Live-Born Incidence of Congenital Heart Disease: A Population-Based Study in California.

    Peyvandi S, Baer RJ, Chambers CD, Norton ME, Rajagopal S, Ryckman KK, Moon-Grady A, Jelliffe-Pawlowski LL, Steurer MA.

    J Am Heart Assoc. 2020 Apr 21;9(8):e015255. doi: 10.1161/JAHA.119.015255. Epub 2020 Apr 19.

     

    Take Home Points

     

    • Adverse social and environmental factors at the neighborhood level may play an important role in the development of congenital heart disease (CHD).
    • Worse social deprivation index (SDI) (6 measures of wealth and income) and environmental exposure index (EEI) (levels of exposure to pollutants) quartiles had higher odds of CHD.
    • Maternal comorbidities explain some, but not all, of this socio-environmental relationship with development of CHD.

     

     

    Commentary from Dr. Clifford Cua (Columbus, OH), Section Editor of Pediatric Cardiology Journal Watch:   The California Office of Statewide Health Planning and Development database was used to obtain data on newborn patients born between 2007 – 2012.  Maternal and infant data are linked in this database and ICD-9 codes were used for diagnostic classification.  Significant congenital heart disease (CHD) was defined as a defect that would require surgery within the 1st year of life.  A social deprivation index (SDI) (6 measures of wealth and income) and environmental exposure index (EEI) (levels of exposure to pollutants) were determined at the neighborhood level.  Z-scores for both indexes were obtained for each patient and scores were categorized into 4 quartiles, with 1st quartile being the most ideal situation and 4th quartile being the least ideal situation.

     

    Hierarchical logistic regression was used to determine the association between the main predictors and the primary outcome after adjustment for maternal factors and age of cohort.  Sensitivity analysis was conducted to determine the relationship after excluding single ventricle diagnosis in regions with high prenatal diagnosis.  A mediation analysis was used to test if maternal comorbidities may be in the causal pathway of SDI and EEI.

     

    During the time period studied, over 2 million live births were included in the study.  All individual sociodemographic and environmental factor Z-scores were worse for in the CHD group (7698 infants with CHD studied) compared to the controls.  The odds of live-born CHD were significantly higher among those with increasing SDI and EEI quartile.  Odds were also significantly higher in mothers with comorbidities to have a child with CHD.  These odds still were significant when excluding infants with known chromosomal abnormalities/syndromes (6120 infants with CHD studied).  Odds were also still significant when excluding single ventricle patients to take into account possible bias when evaluating just live-born births.  Odds were 1.48 (1.32 – 1.66) higher for having a child with CHD for those in the 4th quartile versus those in the 1st quartile.  Causal mediation analysis showed that 13% (95% CI, 10 – 20%) of the total effect of SDI/EEI on the incidence of CHD is mediated through the presence of maternal comorbidities with race/ethnicity as confounders in the relationship between maternal conditions and incidence of CHD.

     

    As the authors stated, this study is limited by the use of an administrative database where errors in entry may occur, ~20% of data were excluded due to incomplete data, only live-born infants were evaluated thus true incidence of CHD may be under-reported due to fetal demise/termination, misclassification of pollutants, and no data on timing of exposure to pollutants.

     

    Despite these limitations, this is a large database study that provides intriguing data suggesting how multifactorial variables may play a role in the development of CHD.  Improving the socioeconomic welfare and decreasing pollutant exposure may be another method to decrease the incidence of CHD in the overall population.

     

     

     

    Pediatric Cardiology Abstracts of April 2020

     

    1. Aortic arch geometry predicts outcome in patients with Loeys-Dietz syndrome independent of the causative gene.

    Mariucci E, Spinardi L, Stagni S, Graziano C, Lovato L, Pacini D, Di Marco L, Careddu L, Angeli E, Ciuca C, Wischmeijer A, Gargiulo G, Donti A.

    Am J Med Genet A. 2020 Apr 30. doi: 10.1002/ajmg.a.61608. [Epub ahead of print]

    PMID: 32352226

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    1. Pulmonary Artery Aneurysm Associated with Bicuspid Pulmonary Valve.

    Izumida S, Kawano H, Tsuneto A, Doi Y, Maemura K.

    Intern Med. 2020 Apr 30. doi: 10.2169/internalmedicine.4415-20. [Epub ahead of print]

    PMID: 32350197 Free Article

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    1. 4D flow cardiovascular magnetic resonance for monitoring of aortic valve repair in bicuspid aortic valve disease.

    Lenz A, Petersen J, Riedel C, Weinrich JM, Kooijman H, Schoennagel BP, Adam G, von Kodolitsch Y, Reichenspurner H, Girdauskas E, Bannas P.

    J Cardiovasc Magn Reson. 2020 Apr 30;22(1):29. doi: 10.1186/s12968-020-00608-0.

    PMID: 32354361 Free PMC Article

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    1. Psychosocial interventions in families with a child with congenital heart disease.

    van der Mheen M, Utens EMWJ.

    J Pediatr. 2020 Apr 30. pii: S0022-3476(20)30346-2. doi: 10.1016/j.jpeds.2020.03.018. [Epub ahead of print] No abstract available.

    PMID: 32362410

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    1. Clinical heterogeneity of Pulmonary Arterial Hypertension associated with variants in TBX4.

    Hernandez-Gonzalez I, Tenorio J, Palomino-Doza J, Martinez Meñaca A, Morales Ruiz R, Lago-Docampo M, Valverde Gomez M, Gomez Roman J, Enguita Valls AB, Perez-Olivares C, Valverde D, Gil Carbonell J, Garrido-Lestache Rodríguez-Monte E, Del Cerro MJ, Lapunzina P, Escribano-Subias P.

    PLoS One. 2020 Apr 29;15(4):e0232216. doi: 10.1371/journal.pone.0232216. eCollection 2020.

    PMID: 32348326 Free PMC Article

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    1. Angiotensin-converting enzyme insertion/deletion (ACE I/D) gene polymorphism in Egyptian children with congenital heart disease.

    Saleh NY, Salem SS, Abo-El Fotoh WM, Soliman SE, Abo-Haded HM.

    Birth Defects Res. 2020 Apr 28. doi: 10.1002/bdr2.1700. [Epub ahead of print]

    PMID: 32343056

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    1. Mature and immature platelets during the first week after birth and incidence of patent ductus arteriosus.

    Sallmon H, Metze B, Koehne P, Opgen-Rhein B, Weiss K, Will JC, Franke CV, Hansmann G, Koestenberger M, Bührer C, Berger F, Weber SC, Cremer M.

    Cardiol Young. 2020 Apr 28:1-5. doi: 10.1017/S1047951120000943. [Epub ahead of print]

    PMID: 32340633

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    1. Deep learning in congenital heart disease imaging: hope but not haste.

    Davies R, Babu-Narayan SV.

    Heart. 2020 Apr 27. pii: heartjnl-2019-316496. doi: 10.1136/heartjnl-2019-316496. [Epub ahead of print] No abstract available.

    PMID: 32341135

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    1. Symptom frequency in children with congenital heart disease and parental care burden in predicting the quality of life of parents in Turkey.

    Bektas İ, Kır M, Yıldız K, Genç Z, Bektas M, Ünal N.

    J Pediatr Nurs. 2020 Apr 27. pii: S0882-5963(20)30165-2. doi: 10.1016/j.pedn.2020.04.012. [Epub ahead of print]

    PMID: 32354457

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    1. Atrial Function and Its Role in the Non-invasive Evaluation of Diastolic Function in Congenital Heart Disease.

    Ta HT, Alsaied T, Steele JM, Truong VT, Mazur W, Nagueh SF, Kutty S, Tretter JT.

    Pediatr Cardiol. 2020 Apr 27. doi: 10.1007/s00246-020-02351-w. [Epub ahead of print] Review.

    PMID: 32342149

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    1. De novo damaging variants associated with congenital heart diseases contribute to the connectome.

    Ji W, Ferdman D, Copel J, Scheinost D, Shabanova V, Brueckner M, Khokha MK, Ment LR.

    Sci Rep. 2020 Apr 27;10(1):7046. doi: 10.1038/s41598-020-63928-2.

    PMID: 32341405 Free PMC Article

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    1. An ontology-based classification of Ebstein’s anomaly and its implications in clinical adverse outcomes.

    Tang X, Chen W, Zeng Z, Ding K, Zhou Z.

    Int J Cardiol. 2020 Apr 26. pii: S0167-5273(19)34800-4. doi: 10.1016/j.ijcard.2020.04.073. [Epub ahead of print]

    PMID: 32348812

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    1. Moving beyond two-dimensional screens to interactive three-dimensional visualization in congenital heart disease.

    Byl JL, Sholler R, Gosnell JM, Samuel BP, Vettukattil JJ.

    Int J Cardiovasc Imaging. 2020 Apr 25. doi: 10.1007/s10554-020-01853-1. [Epub ahead of print] Review.

    PMID: 32335820

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    1. Anomalous lateral course of the right subclavian artery through the lung: A hitherto undescribed variation.

    Ojha V, Ganga KP, Chandrashekhara SH, Kumar S.

    J Card Surg. 2020 Apr 25. doi: 10.1111/jocs.14543. [Epub ahead of print] No abstract available.

    PMID: 32333429

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    1. [Risk factors for death and the clinical features of different subtypes of patients with pulmonary arterial hypertension related to congenital heart disease].

    Xu ZY, Li QQ, Zhang C, Zhang HS, Gu H.

    Zhonghua Xin Xue Guan Bing Za Zhi. 2020 Apr 24;48(4):315-322. doi: 10.3760/cma.j.cn112148-20190628-00364. Chinese.

    PMID: 32370483

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    1. Further delineation of Basel-Vanagaite-Smirin-Yosef syndrome: Report of three patients.

    Haynes D, Pollack L, Prasad C, Goobie S, Colaiacovo S, Wolfinger T, Lacassie Y.

    Am J Med Genet A. 2020 Apr 23. doi: 10.1002/ajmg.a.61603. [Epub ahead of print]

    PMID: 32324310

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    1. Image diagnosis: Eisenmenger’s syndrome in patients with simple congenital heart disease.

    Bu H, Gong X, Zhao T.

    BMC Cardiovasc Disord. 2020 Apr 23;20(1):194. doi: 10.1186/s12872-020-01489-y.

    PMID: 32326907 Free PMC Article

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    1. The role of multimodality imaging in the diagnosis of left ventricular noncompaction.

    Rao K, Bhaskaran A, Choudhary P, Tan TC.

    Eur J Clin Invest. 2020 Apr 23:e13254. doi: 10.1111/eci.13254. [Epub ahead of print] Review.

    PMID: 32329049

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    1. Telediagnosis system for congenital heart disease in a Japanese prefecture.

    Mabuchi A, Waratani M, Tanaka Y, Mori T, Kitawaki J.

    J Med Ultrason (2001). 2020 Apr 23. doi: 10.1007/s10396-020-01020-y. [Epub ahead of print]

    PMID: 32328807

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    1. Respiratory Syncytial Virus-related Death in Children With Down Syndrome: The RSV GOLD Study.

    Löwensteyn YN, Phijffer EWEM, Simons JVL, Scheltema NM, Mazur NI, Nair H, Bont LJ; RSV GOLD Study Group.

    Pediatr Infect Dis J. 2020 Apr 23. doi: 10.1097/INF.0000000000002666. [Epub ahead of print]

    PMID: 32332221

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    1. Aerococcus urinae – A potent biofilm builder in endocarditis.

    Yaban B, Kikhney J, Musci M, Petrich A, Schmidt J, Hajduczenia M, Schoenrath F, Falk V, Moter A.

    PLoS One. 2020 Apr 23;15(4):e0231827. doi: 10.1371/journal.pone.0231827. eCollection 2020.

    PMID: 32325482 Free PMC Article

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    1. Differences in morbidity and mortality in Down syndrome are related to the type of congenital heart defect.

    Baban A, Olivini N, Cantarutti N, Calì F, Vitello C, Valentini D, Adorisio R, Calcagni G, Alesi V, Di Mambro C, Villani A, Dallapiccola B, Digilio MC, Marino B, Carotti A, Drago F.

    Am J Med Genet A. 2020 Apr 22. doi: 10.1002/ajmg.a.61586. [Epub ahead of print]

    PMID: 32319738

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    1. Trajectories of care in congenital heart disease – the long arm of disease in the womb.

    Marelli A.

    J Intern Med. 2020 Apr 22. doi: 10.1111/joim.13048. [Epub ahead of print]

    PMID: 32323405

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    1. Role of the ADCY9 gene in cardiac abnormalities of the Rubinstein-Taybi syndrome.

    Wu Y, Xia Y, Li P, Qu HQ, Liu Y, Yang Y, Lin J, Zheng M, Tian L, Wu Z, Huang S, Qin X, Zhou X, Chen S, Liu Y, Wang Y, Li X, Zeng H, Hakonarson H, Zhuang J.

    Orphanet J Rare Dis. 2020 Apr 22;15(1):101. doi: 10.1186/s13023-020-01378-9.

    PMID: 32321550 Free PMC Article

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    1. Maternal Obesity and Diabetes Mellitus as Risk Factors for Congenital Heart Disease in the Offspring.

    Helle E, Priest JR.

    J Am Heart Assoc. 2020 Apr 21;9(8):e011541. doi: 10.1161/JAHA.119.011541. Epub 2020 Apr 20.

    PMID: 32308111 Free Article

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    1. Environmental and Socioeconomic Factors Influence the Live-Born Incidence of Congenital Heart Disease: A Population-Based Study in California.

    Peyvandi S, Baer RJ, Chambers CD, Norton ME, Rajagopal S, Ryckman KK, Moon-Grady A, Jelliffe-Pawlowski LL, Steurer MA.

    J Am Heart Assoc. 2020 Apr 21;9(8):e015255. doi: 10.1161/JAHA.119.015255. Epub 2020 Apr 19.

    PMID: 32306820 Free Article

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    1. Prevalence of Continuous Pulse Oximetry Monitoring in Hospitalized Children With Bronchiolitis Not Requiring Supplemental Oxygen.

    Bonafide CP, Xiao R, Brady PW, Landrigan CP, Brent C, Wolk CB, Bettencourt AP, McLeod L, Barg F, Beidas RS, Schondelmeyer A; Pediatric Research in Inpatient Settings (PRIS) Network.

    JAMA. 2020 Apr 21;323(15):1467-1477. doi: 10.1001/jama.2020.2998.

    PMID: 32315058

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    1. Contrast enhanced computed tomography findings of persistent truncus arteriosus; A rare congenital heart disease.

    Turkoglu S, Batur A, Yokuş A, Dündar İ, Akinci MB.

    Radiol Case Rep. 2020 Apr 21;15(6):795-798. doi: 10.1016/j.radcr.2020.04.007. eCollection 2020 Jun.

    PMID: 32346456 Free PMC Article

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    1. Baring all: The impact of the hospital gown on patient well-being.

    Morton L, Cogan N, Kornfält S, Porter Z, Georgiadis E.

    Br J Health Psychol. 2020 Apr 20. doi: 10.1111/bjhp.12416. [Epub ahead of print]

    PMID: 32314508

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    1. Continuous, complete and comparable NT-proBNP reference ranges in healthy children.

    Palm J, Hoffmann G, Klawonn F, Tutarel O, Palm H, Holdenrieder S, Ewert P.

    Clin Chem Lab Med. 2020 Apr 18. pii: /j/cclm.ahead-of-print/cclm-2019-1185/cclm-2019-1185.xml. doi: 10.1515/cclm-2019-1185. [Epub ahead of print]

    PMID: 32305952

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    1. Maternal occupational exposure to polycyclic aromatic hydrocarbons and the risk of isolated congenital heart defects among offspring.

    Patel J, Nembhard WN, Politis MD, Rocheleau CM, Langlois PH, Shaw GM, Romitti PA, Gilboa SM, Desrosiers TA, Insaf T, Lupo PJ; National Birth Defects Prevention Study.

    Environ Res. 2020 Apr 18;186:109550. doi: 10.1016/j.envres.2020.109550. [Epub ahead of print]

    PMID: 32335433

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    1. Identification and analysis of KLF13 variants in patients with congenital heart disease.

    Li W, Li B, Li T, Zhang E, Wang Q, Chen S, Sun K.

    BMC Med Genet. 2020 Apr 15;21(1):78. doi: 10.1186/s12881-020-01009-x.

    PMID: 32293321 Free PMC Article

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    1. Insights from circulating microRNAs in cardiovascular entities in turner syndrome patients.

    Abu-Halima M, Oberhoffer FS, El Rahman MA, Jung AM, Zemlin M, Rohrer TR, Kahraman M, Keller A, Meese E, Abdul-Khaliq H.

    PLoS One. 2020 Apr 9;15(4):e0231402. doi: 10.1371/journal.pone.0231402. eCollection 2020.

    PMID: 32271829 Free PMC Article

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    1. “Treat and repair” strategy for shunt lesions: a critical review.

    Arvind B, Relan J, Kothari SS.

    Pulm Circ. 2020 Apr 9;10(2):2045894020917885. doi: 10.1177/2045894020917885. eCollection 2020 Apr-Jun. Review.

    PMID: 32313642 Free PMC Article

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    1. Neuropathology of Congenital Heart Disease in an Inpatient Autopsy Cohort 2000-2017.

    Rettenmaier LA, Kirby PA, Reinking BE, Viaene AN, Hefti MM.

    J Am Heart Assoc. 2020 Apr 7;9(7):e013575. doi: 10.1161/JAHA.119.013575. Epub 2020 Mar 23.

    PMID: 32200729 Free Article

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    1. The membranous septum revisited: A glimpse of our anatomical past.

    De Almeida MC, Sanchez-Quintana D, Anderson RH.

    Clin Anat. 2020 Apr 5. doi: 10.1002/ca.23599. [Epub ahead of print] Review.

    PMID: 32249445

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    1. Bi-allelic Loss-of-Function Variants in NUP188 Cause a Recognizable Syndrome Characterized by Neurologic, Ocular, and Cardiac Abnormalities.

    Muir AM, Cohen JL, Sheppard SE, Guttipatti P, Lo TY, Weed N, Doherty D, DeMarzo D, Fagerberg CR, Kjærsgaard L, Larsen MJ, Rump P, Löhner K, Hirsch Y, Zeevi DA, Zackai EH, Bhoj E, Song Y, Mefford HC.

    Am J Hum Genet. 2020 Apr 4. pii: S0002-9297(20)30085-9. doi: 10.1016/j.ajhg.2020.03.009. [Epub ahead of print]

    PMID: 32275884

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    1. Challenges in the handover process of the new-born with congenital heart disease.

    Hansson L, Wrigstad J, Wangel AM.

    Intensive Crit Care Nurs. 2020 Apr 3:102855. doi: 10.1016/j.iccn.2020.102855. [Epub ahead of print]

    PMID: 32253120

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    1. Epidemiological, clinical, and echocardiographic features, and outcome of dogs with Ebstein’s anomaly: 32 cases (2002-2016).

    Chetboul V, Poissonnier C, Bomassi E, Jamin C, Pouchelon JL, Tissier R, Desquilbet L.

    J Vet Cardiol. 2020 Apr 3;29:11-21. doi: 10.1016/j.jvc.2020.03.003. [Epub ahead of print]

    PMID: 32348933

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    1. Value of emergent pediatric cardiac computed tomographic angiography service: initial experience at a large children’s hospital.

    May LA, More SR, Masand PM, Ketwaroo PD, Krishnamurthy R, Jadhav SP.

    Pediatr Radiol. 2020 Apr 3. doi: 10.1007/s00247-020-04659-4. [Epub ahead of print]

    PMID: 32242280

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    1. Right-sided Aortic Arch Associated with Congenital Heart Disease.

    Arita Y, Tanaka K, Kitabayashi K, Hasegawa S.

    Intern Med. 2020 Apr 2. doi: 10.2169/internalmedicine.4575-20. [Epub ahead of print] No abstract available.

    PMID: 32238728 Free Article

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    1. Early angiogenic proteins associated with high risk for bronchopulmonary dysplasia and pulmonary hypertension in preterm infants.

    Arjaans S, Wagner BD, Mourani PM, Mandell EW, Poindexter BB, Berger RMF, Abman SH.

    Am J Physiol Lung Cell Mol Physiol. 2020 Apr 1;318(4):L644-L654. doi: 10.1152/ajplung.00131.2019. Epub 2020 Jan 22.

    PMID: 31967847

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    1. Genetic characterisation of 22q11.2 variations and prevalence in patients with congenital heart disease.

    Hou HT, Chen HX, Wang XL, Yuan C, Yang Q, Liu ZG, He GW.

    Arch Dis Child. 2020 Apr;105(4):367-374. doi: 10.1136/archdischild-2018-316634. Epub 2019 Oct 30.

    PMID: 31666243

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    1. The Creation of a Pediatric Health Care Learning Network: The ACTION Quality Improvement Collaborative.

    Lorts A, Smyth L, Gajarski RJ, VanderPluym CJ, Mehegan M, Villa CR, Murray JM, Niebler RA, Almond CS, Thrush P, O’Connor MJ, Conway J, Sutcliffe DL, Lantz JE, Zafar F, Morales DLS, Peng DM, Rosenthal DN.

    ASAIO J. 2020 Apr;66(4):441-446. doi: 10.1097/MAT.0000000000001133.

    PMID: 32224822

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    1. Disappearance of the shunt and lower cardiac index during exercise in small, unrepaired ventricular septal defects.

    Maagaard M, Eckerström F, Heiberg J, Asschenfeldt B, Ringgaard S, Hjortdal VE.

    Cardiol Young. 2020 Apr;30(4):526-532. doi: 10.1017/S1047951120000505. Epub 2020 Mar 25.

    PMID: 32209161

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    1. Repair of isolated atrial septal defect in infants less than 12 months improves symptoms of chronic lung disease or shunt-related pulmonary hypertension.

    Charisopoulou D, Bini RM, Riley G, Janagarajan K, Moledina S, Marek J.

    Cardiol Young. 2020 Apr;30(4):511-520. doi: 10.1017/S1047951120000463. Epub 2020 Mar 16.

    PMID: 32172702

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    1. Epidemiology and Outcomes of Acute Decompensated Heart Failure in Children.

    Lasa JJ, Gaies M, Bush L, Zhang W, Banerjee M, Alten JA, Butts RJ, Cabrera AG, Checchia PA, Elhoff J, Lorts A, Rossano JW, Schumacher K, Shekerdemian LS, Price JF.

    Circ Heart Fail. 2020 Apr;13(4):e006101. doi: 10.1161/CIRCHEARTFAILURE.119.006101. Epub 2020 Apr 17.

    PMID: 32301336

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    1. Pediatric Heart Failure: Apples and Oranges and the Way Forward.

    Hsu DT.

    Circ Heart Fail. 2020 Apr;13(4):e006516. doi: 10.1161/CIRCHEARTFAILURE.120.006516. Epub 2020 Apr 17. No abstract available.

    PMID: 32301335

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    Select item 31769098

     

    1. Identification of FOXH1 mutations in patients with sporadic conotruncal heart defect.

    Wei W, Li B, Li F, Sun K, Jiang X, Xu R.

    Clin Genet. 2020 Apr;97(4):576-585. doi: 10.1111/cge.13710. Epub 2020 Feb 12.

    PMID: 32003456

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    Select item 31302712

     

    1. Congenital Portosystemic Shunts in Children: Associations, Complications, and Outcomes.

    DiPaola F, Trout AT, Walther AE, Gupta A, Sheridan R, Campbell KM, Tiao G, Bezerra JA, Bove KE, Patel M, Nathan JD.

    Dig Dis Sci. 2020 Apr;65(4):1239-1251. doi: 10.1007/s10620-019-05834-w. Epub 2019 Sep 23.

    PMID: 31549332

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    1. Efficacy of phosphodiesterase type 5 inhibitors in univentricular congenital heart disease: the SV-INHIBITION study design.

    Amedro P, Gavotto A, Abassi H, Picot MC, Matecki S, Malekzadeh-Milani S, Levy M, Ladouceur M, Ovaert C, Aldebert P, Thambo JB, Fraisse A, Humbert M, Cohen S, Baruteau AE, Karsenty C, Bonnet D, Hascoet S; SV-INHIBITION study investigators.

    ESC Heart Fail. 2020 Apr;7(2):747-756. doi: 10.1002/ehf2.12630. Epub 2020 Mar 9.

    PMID: 32147955 Free PMC Article

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    1. Double-chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome.

    Yamamoto M, Takashio S, Nakashima N, Hanatani S, Arima Y, Sakamoto K, Yamamoto E, Kaikita K, Aoki Y, Tsujita K.

    ESC Heart Fail. 2020 Apr;7(2):721-726. doi: 10.1002/ehf2.12650. Epub 2020 Feb 20.

    PMID: 32078254 Free PMC Article

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    Select item 31740945

     

    1. Parenting stress mediates the association between cyanotic congenital heart disease and internalising problems in children and adolescents.

    Chang LY, Chiu SN, Wang CC, Weng WC, Chang HY.

    Eur J Cardiovasc Nurs. 2020 Apr;19(4):301-309. doi: 10.1177/1474515119881871. Epub 2019 Oct 28.

    PMID: 31656087

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    Select item 31999620

     

    1. Prospective evaluation of autoimmune and non-autoimmune subclinical hypothyroidism in Down syndrome children.

    Pepe G, Corica D, De Sanctis L, Salerno M, Faienza MF, Tessaris D, Tuli G, Scala I, Penta L, Alibrandi A, Pajno GB, Aversa T, Wasniewska M; Thyroid Study Group of the Italian Society of Pediatric Endocrinology and Diabetology (ISPED).

    Eur J Endocrinol. 2020 Apr;182(4):385-392. doi: 10.1530/EJE-19-0823.

    PMID: 31999620

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    Select item 31570783

     

    1. A de novo splicing variant supports the candidacy of TLL1 in ASD pathogenesis.

    Alanzi T, Alhashem A, Dagriri K, Alzahrani F, Alkuraya FS.

    Eur J Hum Genet. 2020 Apr;28(4):525-528. doi: 10.1038/s41431-019-0524-0. Epub 2019 Sep 30.

    PMID: 31570783

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    1. A single nucleotide deletion resulting in a frameshift in exon 4 of TAB2 is associated with a polyvalular syndrome.

    Permanyer E, Laurie S, Blasco-Lucas A, Maldonado G, Amador-Catalan A, Ferrer-Curriu G, Fuste B, Perez ML, Gonzalez-Alujas T, Beltran S, Comas-Riu J, Bardají A, Evangelista A, Galiñanes M.

    Eur J Med Genet. 2020 Apr;63(4):103854. doi: 10.1016/j.ejmg.2020.103854. Epub 2020 Jan 23.

    PMID: 31981616

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    Select item 31654754

     

    1. Small 7p22.3 microdeletion: Case report of Snx8 haploinsufficiency and neurological findings.

    Mastromoro G, Capalbo A, Guido CA, Torres B, Fabbretti M, Traversa A, Giancotti A, Ventriglia F, Bernardini L, Spalice A, Pizzuti A.

    Eur J Med Genet. 2020 Apr;63(4):103772. doi: 10.1016/j.ejmg.2019.103772. Epub 2019 Sep 27.

    PMID: 31568860

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    Select item 31865429

     

    1. Approach to a Child with Congestive Heart Failure.

    Rohit M, Budakoty S.

    Indian J Pediatr. 2020 Apr;87(4):312-320. doi: 10.1007/s12098-020-03255-6. Review.

    PMID: 32162150

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    1. Coping with Caregiving Stress in Families of Children with Congenital Heart Disease: A Qualitative Study.

    Dalir Z, Heydari A, Kareshki H, Manzari ZS.

    Int J Community Based Nurs Midwifery. 2020 Apr;8(2):127-139. doi: 10.30476/IJCBNM.2020.83029.1113.

    PMID: 32309454 Free PMC Article

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    Select item 31968274

     

    1. Congenital heart disease in children: Orofacial myofunctional aspects, eating behavior and facial temperature.

    Barbosa MDG, Castelo PM, Ferreira CLP, Haddad DS, Chiari BM, Santana MV, Bommarito S.

    Int J Pediatr Otorhinolaryngol. 2020 Apr;131:109883. doi: 10.1016/j.ijporl.2020.109883. Epub 2020 Jan 15. Review.

    PMID: 31968274

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    Select item 32236557

     

    1. The Pediatric and Congenital Heart Disease Track at ASE 2020 Explores the Current State-of-the-Art and the Future of Cardiac Imaging.

    Sachdeva R, Srivastava S.

    J Am Soc Echocardiogr. 2020 Apr;33(4):A22. doi: 10.1016/j.echo.2020.02.006. No abstract available.

    PMID: 32248910

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    Select item 31980926

     

    1. A novel de novo dominant mutation of NOTCH1 gene in an Iranian family with non-syndromic congenital heart disease.

    Kalayinia S, Maleki M, Mahdavi M, Mahdieh N.

    J Clin Lab Anal. 2020 Apr;34(4):e23147. doi: 10.1002/jcla.23147. Epub 2019 Dec 22.

    PMID: 31867804 Free PMC Article

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    Select item 32088108

     

    1. Bridging the Cardiac Needs of a Large, Underserved Immigrant and Resettled Refugee Population.

    Agrawal H, Dokania G, Allen HD, Acosta S, Caracostis A, Havemann LM, Lara A, Riley AF, Seery TJ.

    J Pediatr. 2020 Apr;219:83-88. doi: 10.1016/j.jpeds.2019.12.022. Epub 2020 Jan 24.

    PMID: 31987651

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    Select item 31704054

     

    1. Longitudinal Analysis of Ventilation Perfusion Mismatch in Congenital Diaphragmatic Hernia Survivors.

    Dao DT, Kamran A, Wilson JM, Sheils CA, Kharasch VS, Mullen MP, Rice-Townsend SE, Zalieckas JM, Morash D, Studley M, Staffa SJ, Zurakowski D, Becker RE, Smithers CJ, Buchmiller TL.

    J Pediatr. 2020 Apr;219:160-166.e2. doi: 10.1016/j.jpeds.2019.09.053. Epub 2019 Nov 5.

    PMID: 31704054

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    Select item 31793183

     

    1. Collaborative caregiving of parents who have an infant with congenital heart disease.

    Pridham KF, Harrison TM, Brown R, Mussatto K.

    J Spec Pediatr Nurs. 2020 Apr;25(2):e12283. doi: 10.1111/jspn.12283. Epub 2019 Dec 2.

    PMID: 31793183

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    Select item 31706555

     

    1. Addressing the rising burden of congenital heart disease in China.

    He Y, Xu W, Su Z, Liu K, Zhang H.

    Lancet Child Adolesc Health. 2020 Apr;4(4):e7. doi: 10.1016/S2352-4642(20)30061-4. Epub 2020 Mar 17. No abstract available.

    PMID: 32197102

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    Select item 32311999

     

    1. Wiedemann-steiner syndrome with a de novo mutation in KMT2A: A case report.

    Jinxiu L, Shuimei L, Ming X, Jonathan LC, Xiangju L, Wenyuan D.

    Medicine (Baltimore). 2020 Apr;99(16):e19813. doi: 10.1097/MD.0000000000019813.

    PMID: 32311999 Free Article

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    1. Two case reports of right atrial aneurysm.

    Li HP, Ye XW, Wang HT.

    Medicine (Baltimore). 2020 Apr;99(16):e19748. doi: 10.1097/MD.0000000000019748.

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    Select item 32059087

     

    1. Defining language disorders in children and adolescents with Noonan Syndrome.

    Lazzaro G, Caciolo C, Menghini D, Cumbo F, Digilio MC, Capolino R, Zampino G, Tartaglia M, Vicari S, Alfieri P.

    Mol Genet Genomic Med. 2020 Apr;8(4):e1069. doi: 10.1002/mgg3.1069. Epub 2020 Feb 14.

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    Select item 31985165

     

    1. Novel KLHL26 variant associated with a familial case of Ebstein’s anomaly and left ventricular noncompaction.

    Samudrala SSK, North LM, Stamm KD, Earing MG, Frommelt MA, Willes R, Tripathi S, Dsouza NR, Zimmermann MT, Mahnke DK, Liang HL, Lund M, Lin CW, Geddes GC, Mitchell ME, Tomita-Mitchell A.

    Mol Genet Genomic Med. 2020 Apr;8(4):e1152. doi: 10.1002/mgg3.1152. Epub 2020 Jan 27.

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    Select item 32265037

     

    1. Utilization of Diagnostic Testing for Renal Anomalies and Congenital Heart Disease in Patients with Microtia.

    Ramprasad VH, Shaffer AD, Jabbour N.

    Otolaryngol Head Neck Surg. 2020 Apr;162(4):554-558. doi: 10.1177/0194599820901351. Epub 2020 Jan 21.

    PMID: 31959051

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    1. Drug Treatment of Pulmonary Hypertension in Children.

    Avitabile CM, Vorhies EE, Ivy DD.

    Paediatr Drugs. 2020 Apr;22(2):123-147. doi: 10.1007/s40272-019-00374-2. Review.

    PMID: 31960361

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    1. Association between cardiovascular anomalies and karyotypes in Turner syndrome patients in Taiwan: A local cohort study.

    Chou YY, Wang CJ, Lin CH, Chung HT, Lo FS.

    Pediatr Neonatol. 2020 Apr;61(2):188-194. doi: 10.1016/j.pedneo.2019.10.001. Epub 2019 Oct 11.

    PMID: 31672476 Free Article

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    1. High-flow nasal cannula for the treatment of life-threatening plastic bronchitis.

    Vézina K, Dipchand AI, Narang I.

    Pediatr Pulmonol. 2020 Apr;55(4):E1-E2. doi: 10.1002/ppul.24695. Epub 2020 Feb 18.

    PMID: 32068971

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    Select item 31711070

     

    1. Pitfalls of using IQ short forms in neurodevelopmental disorders: a study in patients with congenital heart disease.

    Ehrler M, Latal B, Polentarutti S, von Rhein M, Held L, Wehrle FM.

    Pediatr Res. 2020 Apr;87(5):917-923. doi: 10.1038/s41390-019-0667-2. Epub 2019 Nov 11.

    PMID: 31711070

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    Select item 31953255

     

    1. Ppp1r1b-lncRNA inhibits PRC2 at myogenic regulatory genes to promote cardiac and skeletal muscle development in mouse and human.

    Kang X, Zhao Y, Van Arsdell G, Nelson SF, Touma M.

    RNA. 2020 Apr;26(4):481-491. doi: 10.1261/rna.073692.119. Epub 2020 Jan 17.

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    1. Audit of Childhood Death in a Tertiary Care Center in Niger Delta Region of Nigeria.

    Obiora UJ, Ekpebe PA, Okoye C, David-Idiapho CG.

    West Afr J Med. 2020 Apr-Jun;37(2):113-117.

    PMID: 32150628

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    Select item 31931249

     

    1. Bilateral Multilevel Cervical Rib and Bilateral Omovertebra in Klippel-Feil Syndrome.

    Satış S, Alparslan N, Tuna M, Dere O, Yetişgin A.

    World Neurosurg. 2020 Apr;136:62-65. doi: 10.1016/j.wneu.2020.01.010. Epub 2020 Jan 10.

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    1. Time-trend population analysis of the clinical and epidemiologic effect on pediatric infective endocarditis after change of antibiotic prophylaxis guidelines.

    Walter K, Katharina SS, Martin C, Roland W.

    Infection. 2020 Apr 30. doi: 10.1007/s15010-020-01433-4. [Epub ahead of print]

    PMID: 32356253

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    Select item 32356163

     

    1. Reference values of normal liver stiffness in healthy children by two methods: 2D shear wave and transient elastography.

    Mărginean CO, Meliţ LE, Ghiga DV, Săsăran MO.

    Sci Rep. 2020 Apr 29;10(1):7213. doi: 10.1038/s41598-020-64320-w.

    PMID: 32350349 Free PMC Article

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    Select item 32360389

     

    1. Mediastinal mass after a Blalock-Taussig shunt: utility of CT angiography.

    Peña-Trujillo V, Gallo-Bernal S, Forero Melo JF.

    Cardiol Young. 2020 Apr 28:1-2. doi: 10.1017/S1047951120000906. [Epub ahead of print]

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    Select item 32340633

     

    1. Children Born with Congenital Heart Defects and Growth Restriction at Birth: A Systematic Review and Meta-Analysis.

    Ghanchi A, Derridj N, Bonnet D, Bertille N, Salomon LJ, Khoshnood B.

    Int J Environ Res Public Health. 2020 Apr 28;17(9). pii: E3056. doi: 10.3390/ijerph17093056. Review.

    PMID: 32354021 Free Article

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    Select item 32343451

     

    1. Cardiac Function by Magnetic Resonance Imaging in Coronary Artery Occlusions After Kawasaki Disease.

    Nakaoka H, Tsuda E, Morita Y, Kurosaki K.

    Circ J. 2020 Apr 24;84(5):792-798. doi: 10.1253/circj.CJ-19-0511. Epub 2020 Mar 31.

    PMID: 32238692 Free Article

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    Select item 32238643

     

    1. A statistical comparison of reproducibility in current pediatric two-dimensional echocardiographic nomograms.

    Cantinotti M, Scalese M, Giordano R, Assanta N, Marchese P, Franchi E, Viacava C, Koestenberger M, Jani V, Kutty S.

    Pediatr Res. 2020 Apr 24. doi: 10.1038/s41390-020-0900-z. [Epub ahead of print]

    PMID: 32330930

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    Select item 32332807

     

    1. Missed or Delayed Diagnosis of Kawasaki Disease During the 2019 Novel Coronavirus Disease (COVID-19) Pandemic.

    Harahsheh AS, Dahdah N, Newburger JW, Portman MA, Piram M, Tulloh R, McCrindle BW, de Ferranti SD, Cimaz R, Truong DT, Burns JC.

    J Pediatr. 2020 Apr 23. pii: S0022-3476(20)30556-4. doi: 10.1016/j.jpeds.2020.04.052. [Epub ahead of print] No abstract available.

    PMID: 32370951 Free PMC Article

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    Select item 32361481

     

    1. [Functional Echocardiography for Neonatologists – Consensus based Recommendations of the German Association for Pediatric Cardiology and the German Association for Neonatology and Pediatric Intensive Care].

    Herberg U, Czernik C, Höhn R, Kececioglu D, Riede FT, Robel-Tillig E, von der Wense A.

    Z Geburtshilfe Neonatol. 2020 Apr 22. doi: 10.1055/a-1139-9470. [Epub ahead of print] German. No abstract available.

    PMID: 32323281

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    Select item 32320658

     

    1. Beta Blocker Therapy for Congenital Hepatic Arteriovenous Fistula in Two Neonates.

    Ba H, Xu L, Peng H, Lin Y, Li X, Qin Y, Wang H.

    Front Pediatr. 2020 Apr 21;8:163. doi: 10.3389/fped.2020.00163. eCollection 2020.

    PMID: 32373560 Free PMC Article

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    Select item 32299590

     

    1. A novel echocardiographic approach indicates disease severity in pediatric pulmonary hypertension.

    Koestenberger M, Avian A, Cantinotti M, Meinel K, Hansmann G.

    Pediatr Int. 2020 Apr 17. doi: 10.1111/ped.14163. [Epub ahead of print] No abstract available.

    PMID: 32304149

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    Select item 32299151

     

    1. Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy.

    El Assaad I, Gauvreau K, Rizwan R, Margossian R, Colan S, Chen MH.

    J Am Soc Echocardiogr. 2020 Apr 9. pii: S0894-7317(20)30067-5. doi: 10.1016/j.echo.2020.01.020. [Epub ahead of print]

    PMID: 32279939

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    Select item 32271829

     

    1. Understanding antibiotic prophylaxis prescribing in pediatric surgical specialties.

    Malone SM, Seigel NS, Newland JG, Saito JM, McKay VR.

    Infect Control Hosp Epidemiol. 2020 Apr 7:1-6. doi: 10.1017/ice.2020.71. [Epub ahead of print]

    PMID: 32252848

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    Select item 32223393

     

    1. Selexipag for the treatment of children with pulmonary arterial hypertension: First multicenter experience in drug safety and efficacy.

    Hansmann G, Meinel K, Bukova M, Chouvarine P, Wåhlander H, Koestenberger M; European Pediatric Pulmonary Vascular Disease Network (EPPVDN).

    J Heart Lung Transplant. 2020 Apr 7. pii: S1053-2498(20)31490-X. doi: 10.1016/j.healun.2020.03.029. [Epub ahead of print]

    PMID: 32362477 Free Article

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    Select item 32269016

     

    1. The role of a multidisciplinary team in a pediatric pulmonary hypertension center.

    Whalen E, Ely E, Brown A.

    Pediatr Pulmonol. 2020 Apr 6. doi: 10.1002/ppul.24761. [Epub ahead of print] Review.

    PMID: 32250064

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    Select item 32248201

     

    1. Rivaroxaban for treatment of pediatric venous thromboembolism. An Einstein-Jr Phase 3 dose-exposure-response evaluation.

    Young G, Lensing AWA, Monagle P, Male C, Thelen K, Willmann S, Palumbo JS, Kumar R, Nurmeev I, Hege K, Bajolle F, Connor P, Hooimeijer HL, Torres M, Chan AKC, Kenet G, Holzhauer S, Santamaría A, Amedro P, Beyer-Westendorf J, Martinelli I, Patricia Massicotte M, Smith WT, Berkowitz SD, Schmidt S, Price V, Prins MH, Kubitza D; EINSTEIN-Jr. Phase 3 Investigators.

    J Thromb Haemost. 2020 Apr 4. doi: 10.1111/jth.14813. [Epub ahead of print]

    PMID: 32246743

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    Select item 32248280

     

    1. Sedated Echocardiograms Better Characterize Branch Pulmonary Arteries Following Bidirectional Glenn Palliation with Minimal Risk of Adverse Events.

    Williams JL, Raees MA, Sunthankar S, Killen SAS, Bichell D, Parra DA, Soslow JH.

    Pediatr Cardiol. 2020 Apr 4. doi: 10.1007/s00246-020-02342-x. [Epub ahead of print]

    PMID: 32248280

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    Select item 32242738

     

    1. Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.

    Garcia AM, Beatty JT, Nakano SJ.

    Am J Physiol Heart Circ Physiol. 2020 Apr 1;318(4):H947-H965. doi: 10.1152/ajpheart.00518.2019. Epub 2020 Feb 28.

    PMID: 32108525

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    Select item 32159369

     

    1. Congenital giant right coronary artery.

    Raut MS, Hanjoora VM, Srivastava AR, Khangarot SS, Jyoti A, Mahajan V, Rawal N.

    Ann Card Anaesth. 2020 Apr-Jun;23(2):230-231. doi: 10.4103/aca.ACA_181_18.

    PMID: 32275044 Free Article

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    Select item 32246937

     

    1. Bidirectional coronary venous return in the partial unroofed coronary sinus syndrome complicated by coronary sinus orifice atresia.

    Asada D, Ito H.

    Cardiol Young. 2020 Apr;30(4):582-584. doi: 10.1017/S1047951120000475. Epub 2020 Mar 5.

    PMID: 32131919

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    Select item 32090726

     

    1. Paediatric cardiomyopathies: echocardiographic diagnosis, clinical profile, and demographic characteristics: the experience of a tertiary referral centre for Latin American paediatric cardiology.

    Huertas-Quiñones VM, Mestra CF, Peña-Trujillo V, Gallo-Bernal S, Villaveces M, Alarcón-Forero LC.

    Cardiol Young. 2020 Apr;30(4):462-467. doi: 10.1017/S1047951120000281. Epub 2020 Feb 17.

    PMID: 32063255

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    Select item 31393559

     

    1. Neonatal functional echocardiography.

    Tissot C, Singh Y.

    Curr Opin Pediatr. 2020 Apr;32(2):235-244. doi: 10.1097/MOP.0000000000000887.

    PMID: 32068595

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    Select item 32200576

     

    1. Isolated right superior vena cava drainage into the left atrium: An uncommon cause of persistent hypoxia in a neonate.

    Gulati R, Smith T, Siow M, Batten L.

    Echocardiography. 2020 Apr;37(4):641-645. doi: 10.1111/echo.14636. Epub 2020 Mar 22.

    PMID: 32200576

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    Select item 32147955

     

    1. Takotsubo (stress) cardiomyopathy in childhood.

    Topal Y, Topal H, Doğan C, Tiryaki SB, Biteker M.

    Eur J Pediatr. 2020 Apr;179(4):619-625. doi: 10.1007/s00431-019-03536-z. Epub 2019 Dec 21.

    PMID: 31865427

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    Select item 32091595

     

    1. Pediatric Cardiology: Updates for Pediatrician.

    Ahamed MZ, Rohit M.

    Indian J Pediatr. 2020 Apr;87(4):287-288. doi: 10.1007/s12098-020-03266-3. Epub 2020 Mar 13. No abstract available.

    PMID: 32166606

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    Select item 32162150

     

    1. Approach to a Child with Congestive Heart Failure.

    Rohit M, Budakoty S.

    Indian J Pediatr. 2020 Apr;87(4):312-320. doi: 10.1007/s12098-020-03255-6. Review.

    PMID: 32162150

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    Select item 31732966

     

    1. 4D flow vs. 2D cardiac MRI for the evaluation of pulmonary regurgitation and ventricular volume in repaired tetralogy of Fallot: a retrospective case control study.

    Jacobs KG, Chan FP, Cheng JY, Vasanawala SS, Maskatia SA.

    Int J Cardiovasc Imaging. 2020 Apr;36(4):657-669. doi: 10.1007/s10554-019-01751-1. Epub 2020 Jan 1.

    PMID: 31894524

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    Select item 31912893

     

    1. Effects of levosimendan on ventriculo-arterial coupling and cardiac efficiency in paediatric patients with single-ventricle physiology after surgical palliation: retrospective study.

    Iacobelli R, Ricci Z, Marinari E, Haiberger R, Di Molfetta A, Toscano A, Di Chiara L.

    Interact Cardiovasc Thorac Surg. 2020 Apr 1;30(4):623-629. doi: 10.1093/icvts/ivz319.

    PMID: 31986196

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    Select item 31821450

     

    1. Urinary NT-proBNP as a potential noninvasive biomarker for screening of pulmonary hypertension in preterm infants: a pilot study.

    Naeem B, Ayub A, Aly AM, Malloy MH, Okorodudu AO, Jain SK.

    J Perinatol. 2020 Apr;40(4):628-632. doi: 10.1038/s41372-019-0581-9. Epub 2020 Jan 7.

    PMID: 31911650

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    Select item 31740116

     

    1. MicroRNA as a Biomarker in Pediatric Pulmonary Hypertension: A Step Closer to the Holy Grail?

    Coleman RD, Chartan CA, Ivy DD.

    Pediatr Crit Care Med. 2020 Apr;21(4):393-394. doi: 10.1097/PCC.0000000000002219. No abstract available.

    PMID: 32251186

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    Select item 32084099

     

    099

ACHD Featured Articles in association with ISACHD

Adult Congenital Heart Reviews of April 2020 Manuscripts

 

Adult Congenital Heart Reviews of April 2020 (sponsored by ISACHD)

 

  1. Body Composition in Young Adults Living With a Fontan Circulation: The Myopenic Profile.

Tran D, D’Ambrosio P, Verrall CE, Attard C, Briody J, D’Souza M, Fiatarone Singh M, Ayer J, d’Udekem Y, Twigg S, Davis GM, Celermajer DS, Cordina R.

J Am Heart Assoc. 2020 Apr 21;9(8):e015639. doi: 10.1161/JAHA.119.015639. Epub 2020 Apr 15.

PMID: 32290749 Free Article

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Take Home Points:

 

  1. In this relatively healthy group of Fontan patients, low skeletal muscle mass was associated with reduced exercise capacity, ventricular dysfunction, and compensatory erythrocytosis as a marker of cyanosis.
  1. BMI overestimates skeletal muscle mass and underestimates adiposity in Fontan patients.

 

 

Commentary by Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:  The Fontan circulation is associated with elevated central venous pressure, low cardiac output, and cyanosis. These abnormalities increase the risk of heart failure, arrhythmias, thromboembolic events, hepatic fibrosis, and protein using the neuropathy, to name a few. Prior studies have described role of skeletal muscle as a “muscle pump” that increases venous return and augments pulmonary blood flow, which improves cardiac output and improves exercise capacity in Fontan patients.

Cardiac dysfunction can cause neurohormonal derangement and associated skeletal muscle loss and myopenia. There is also a complicated and often paradoxical interrelationship between obesity and heart failure (obesity paradox) with obese patients more likely to develop heart failure but obese patients with heart failure having improved survival. Data are mixed regarding the presence of the obesity paradox failure in Fontan patients.

Derek Tran and colleagues from Sydney, Australia performed a cross-sectional study of 28 Fontan patients who were prospectively recruited. The mean age was 26 with a near even split between male and female and 57% had a systemic left ventricle.  Extracardiac Fontan was present in 50%, lateral tunnel in 39%, and 11% (3 patients) with atriopulmonary Fontan. The median BMI was 22.4 kg/m2. Participants had dual energy x-ray absorptiometry (DXA) to assess Appendicular lean mass index (ALMI) Z score and total percent body fat (%BF). They also underwent cardiopulmonary stress testing, echocardiography, handgrip strength assessment, and biochemical assessments. This was a relatively healthy group with exclusion criteria that included NYHA class III-IV, major intellectual or physical disability, or current pregnancy.

 

Fontan associated myopenia ( Z score: -2 or lower) :  11 patients (39%)

– Less pronounced skeletal muscle mass deficit (Z score: between -2 and -1) : 8 patients (29%)

– Normal range muscle mass (Z score: higher than -1) was present in only 9 patients (32%)

All participants with normal range skeletal muscle mass had normal ventricular systolic function. Whereas 80% participants with ventricular dysfunction had skeletal myopenia. Males had lower %BF. High adiposity was present in 32%, moderate adiposity and 14%, 50% had normal range adiposity, and 4% (1) had low adiposity. There were 3 patients who had both Fontan associated myopenia and high adiposity.

Vitamin D deficiency was not associated with myopenia. Above normal range PTH was present in 40%, even though only 7 patients had low vitamin D. Blood leptin was increased and 70% of patients, reflecting elevated adiposity.

ALMI was strongly associated with exercise capacity as measured by peak VO2. Fontan associated myopenia was strongly associated with reduced peak handgrip. There was no difference in spirometry measures between normal and reduced muscle mass groups.

This is the first study to characterize body composition using DXA in Fontan patients. This clinically stable group showed low skeletal muscle mass and adiposity predisposition, which can be unrecognized when looking at BMI alone. BMI may overestimate skeletal muscle mass and underestimate adiposity in Fontan patients. Low skeletal muscle mass was associated with reduced exercise capacity, ventricular dysfunction, and compensatory erythrocytosis as a marker of cyanosis. About two thirds of participant had reduced muscle mass.  ALMI was independently associated with absolute peak VO2. Grip strength was positively associated with muscle mass and was lower in patients with Fontan associated myopenia.

Ventricular systolic dysfunction was associated with low muscle mass, which could be due to peripheral “muscle pump” impairment reducing venous return, pulmonary blood flow, and cardiac output. Ventricular dysfunction can also cause myopenia due to myriad physiologic and neurohormonal mechanisms.

Based in DXA analysis of Fontan patients, reduced muscle mass and increased adiposity is common.

Given that reduced muscle mass is associated with ventricular dysfunction and reduced exercise capacity, additional study is needed to determine the therapeutic strategies and potentially substantial benefits of building lean muscle mass in these patients.

 

  1. Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

Ghosh RM, Griffis HM, Glatz AC, Rome JJ, Smith CL, Gillespie MJ, Whitehead KK, O’Byrne ML, Biko DM, Ravishankar C, Dewitt AG, Dori Y.

J Am Heart Assoc. 2020 Apr 7;9(7):e015318. doi: 10.1161/JAHA.119.015318. Epub 2020 Mar 30.

PMID: 32223393 Free Article

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Take Home Points:

 

  • Nearly a third of patients developed complications in the first 6 months post Fontan completion.
  • This was mainly driven by prolonged pleural effusions, readmission or unplanned cardiac catheterization.
  • Prolonged cross-clamp time and prolonged bypass time emerged as risk factors for early Fontan morbidity.
  • The presence and severity of AV valve regurgitation did not influence early outcomes.
  • The presence of a type 3 or type 4 lymphatic drainage pattern on MRI (T2 -weighted) was associated with higher early failure rates (Odds ratio 6.28).

Commentary from Dr. Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch:  Recent studies implicated the role of lymphatic congestion in the pathogenesis of both protein-losing enteropathy and plastic bronchitis in patients with the Fontan circulation resulting in late Fontan failure. The role of lymphatic drainage in the development of early Fontan complications is not well described. This is a single center retrospective study at a tertiary paediatric center in the US, describing the prevalence and cause of early post-Fontan morbidity.

All patients who underwent a Fontan operation from June 2012 to July 2017 were included. Those presenting for a Fontan take-down, those for Fontan revision or those with a Kawashima operation, were excluded. Lymphatic patterns were characterized using T2-weighted images. Patterns included 4 types:

  • Type 1: Little or no abnormalities of the thoracic duct
  • Type 2: Enhancement of the supraclavicular area and dilatation and/or tortuosity of the thoracic duct
  • Type 3: Enhancement of mediastinal and supraclavicular area
  • Type 4: enhancement extending from the mediastinum into the lung parenchyma

 

The primary outcome was a composite of early Fontan complications (within 6 months of surgery) including death, Fontan take-down, ECMO, chest drain >14 days, cardiac catheterization, re-admission, heart transplant listing. Fontan failure is characterized into 4 groups: structural failure, pump failure, pleural (non-chylous) effusions despite lack of pump or structural failure, and lastly lymphatic failure – usually presenting with chylothorax or plastic bronchitis.

 

 

Two hundred and thirty-eight patients were included in the study; 58 developed early Fontan complications (25.7%) – only 2 deaths occurred. Mean age at surgery was 3.4±1.7 years. An extra-cardiac fenestrated Fontan was present in 81% of the cohort.

The presence of a systemic RV (81% vs 67%, p0.047), a longer bypass time (median time 69.5 vs 64 minutes, p=0.025) and a longer cross-clamp time (median time 29 vs 25 min, p=0.002) was associated with a higher rate of early post-Fontan complications.  The presence and severity of atrio-ventricular valve regurgitation did not have an effect on early outcomes.

Fontan failure was attributed to structural failure in 20 patients (35%), and to pump failure in 8 patients (14%). The presence of severe Fontan complications was only 4%.  Fifteen patients (21.5%) had prolonged effusions and 15 patients (21.5%) had lymphatic failure as evidenced by plastic bronchitis or chylous effusions.

One hundred and ninety-five patients (82%) had pre-Fontan MRI’s. The systemic-pulmonary arterial collateral (APC) burden was quantified and expressed as a percentage of the total aortic flow. Of the 51 patients with a >35%  APC burden, 43 had collateral embolization prior to Fontan completion. Only 126 patients had the correct T2 weighted sequences to establish lymphatic flow patterns: 39 had type 1, 41 had type 2, 35 had type 3. Only 7 had type 4 – thus rather rare.

Forty- three percent of those with type 3 pattern and all of those with the type 4 pattern developed early complications. Type 3 and type 4 patterns were combined and regarded as high-grade lymphatic abnormalities.

Type 3 and 4 combined subtended an odds-ratio of 6.05 (95% CI 2.10-17.46, p=0.001) for developing complications compared to those with a type 1 pattern. When controlling for a morphological RV, bypass and cross-clamp time, the odds ratio was 6.28 (95% CI 2.13 – 18.5, p=0.001) for developing early Fontan complications.

 

Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot.

Arroyo-Rodríguez C, Fritche-Salazar JF, Posada-Martínez EL, Arías-Godínez JA, Ortiz-León XA, Calvillo-Arguelles O, Ruiz-Esparza ME, Sandoval JP, Sierra-Lara D, Araiza-Garaygordobil D, Picano E, Rodríguez-Zanella H.

Int J Cardiovasc Imaging. 2020 Apr;36(4):595-604. doi: 10.1007/s10554-019-01753-z. Epub 2020 Jan 1.

PMID: 31894525

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Take home point:

 

  • Right ventricular free wall longitudinal strain may be useful as a surrogate marker for low functional capacity in patients with repaired tetralogy of Fallot with severe pulmonary regurgitation (PR).

Commentary from Dr. Helen Parry (Leeds UK), section editor of ACHD Journal Watch:  Functional capacity can be a useful guide to pulmonary valve replacement in patients with repaired tetralogy of Fallot who are on the borderline for intervention according to current guidelines.  However, many patients are unable to exercise well due to non-cardiac reasons. This study looked at surrogate markers for impaired functional capacity to help guide management in borderline cases for pulmonary valve replacement.

 

Method:

Thirty-three patients were included in the study.  Inclusion criteria were previous tetralogy of Fallot repair, NYHA class 1, no other significant cardiac abnormality such as AVSD and severe pulmonary regurgitation (PR).  Severe PR was defined according to the following criteria:

  • Jet: pulmonary annulus diameter >=0.7
  • Pressure half time <100ms
  • Diastolic flow reversal in the pulmonary branches
  • PR index <0.77

The level of PR was classed as severe if 3 of the above criteria were fulfilled, or 2 if PR index<0.77.

Exercise echocardiography was performed using a bike with initial workload of 25W, increasing by 25 W every 2 mins.  Patients who managed <7 METS were categorised as low functional capacity.

Strain imaging was performed using a GE machine and analysis was performed offline using the EchoPAC software.  Inter-observer variability was assessed by repeat analysis by the same operator with a 2 week difference period.

Statistical analysis was performed by dividing the patients into 2 groups: low and normal functional capacity.  Comparisons were made using the Students t-test for normally distributed variables and Wilcoxon sum rank test for non-parametric variables.

 

Results:

Twenty- two patients had normal functional capacity relative to 11 in the low functional capacity group.

The following variables were associated with low functional capacity, p value<0.05:

  • Female gender
  • Short stature
  • Previous shunt palliation
  • LV contractile reserve
  • Right ventricular free wall longitudinal strain <17%

Conclusions:

Right ventricular free wall longitudinal strain may be useful as a surrogate marker for low functional capacity in patients with repaired tetralogy of Fallot with severe PR.

 Strengths of the study:

  • Timings for pulmonary valve replacement are a continued subject of debate- many clinicians believe we should be doing this at an earlier stage.
  • Limited literature regarding the uses of newer echo techniques here, this study is an important contribution to the literature.
  • Suggests a useful alternative to exercise testing in these borderline patients.

Weaknesses of the study:

  • Very small sample size, only 11 patients in the low functional capacity group-can statistically meaningful associations genuinely be found in such a small sample?
  • Strain imaging is both machine specific and supplier specific- many departments do not use GE systems, the cut-off for right ventricular wall strain is likely to be different across manufacturers.
  • Many patients do not have adequate echo windows for reliable RV strain imaging.
  • Inter-observer variability was tested by the same reporter reviewing the scans with 2 weeks difference. Two individuals performing analysis and testing agreement between the 2 would be a better way of looking at inter-observer variability.

 

ACHD Abstracts of April 2020

 

  1. Quality of Life in Adults With Congenital Heart Disease: Function Over Form.

Greenway SC.

Can J Cardiol. 2020 Apr 24. pii: S0828-282X(20)30406-2. doi: 10.1016/j.cjca.2020.04.021. [Epub ahead of print] No abstract available.

PMID: 32339650

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  1. Body Composition in Young Adults Living With a Fontan Circulation: The Myopenic Profile.

Tran D, D’Ambrosio P, Verrall CE, Attard C, Briody J, D’Souza M, Fiatarone Singh M, Ayer J, d’Udekem Y, Twigg S, Davis GM, Celermajer DS, Cordina R.

J Am Heart Assoc. 2020 Apr 21;9(8):e015639. doi: 10.1161/JAHA.119.015639. Epub 2020 Apr 15.

PMID: 32290749 Free Article

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  1. The impact of the aortic cusps fusion pattern and valve disease severity on the aortic wall mechanics in patients with bicuspid aortic valve.

Kalinowski ME, Szulik M, Pawlak S, Rybus-Kalinowska B, Zembala M, Kalarus Z, Kukulski T.

Int J Cardiovasc Imaging. 2020 Apr 17. doi: 10.1007/s10554-020-01838-0. [Epub ahead of print]

PMID: 32303878

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  1. The diagnosis and treatment of adults with congenital heart disease.

Henning RJ.

Future Cardiol. 2020 Apr 16. doi: 10.2217/fca-2019-0061. [Epub ahead of print]

PMID: 32297523

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  1. Nationwide Frequency, Sequential Trends, and Impact of Co-morbid Mental Health Disorders on Hospitalizations, Outcomes, and Healthcare Resource Utilization in Adult Congenital Heart Disease.

Desai R, Patel K, Dave H, Shah K, DeWitt N, Fong HK, Varma Y, Varma K, Mansuri Z, Sachdeva R, Khanna A, Kumar G.

Am J Cardiol. 2020 Apr 15;125(8):1256-1262. doi: 10.1016/j.amjcard.2020.01.024. Epub 2020 Jan 30.

PMID: 32085866

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  1. Inspiratory muscle training did not improve exercise capacity and lung function in adult patients with Fontan circulation: A randomized controlled trial.

Fritz C, Müller J, Oberhoffer R, Ewert P, Hager A.

Int J Cardiol. 2020 Apr 15;305:50-55. doi: 10.1016/j.ijcard.2020.01.015. Epub 2020 Jan 9.

PMID: 31992463

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  1. Biomarker profile in stable Fontan patients.

Saraf A, De Staercke C, Everitt I, Haouzi A, Ko YA, Jennings S, Kim JH, Rodriguez FH, Kalogeropoulos AP, Quyyumi A, Book W.

Int J Cardiol. 2020 Apr 15;305:56-62. doi: 10.1016/j.ijcard.2020.01.012. Epub 2020 Jan 9.

PMID: 31959411

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  1. Subacute Bacterial Endocarditis (SBE) Prophylaxis.

Ibrahim AM, Siddique MS.

StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-.
2020 Apr 14.

PMID: 30422578 Free Books & Documents

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  1. A transition clinic intervention to improve follow-up rates in adolescents and young adults with congenital heart disease.

Gaydos SS, Chowdhury SM, Judd RN, McHugh KE.

Cardiol Young. 2020 Apr 13:1-8. doi: 10.1017/S1047951120000682. [Epub ahead of print]

PMID: 32279695

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  1. Pulmonary vasodilators can lead to various complications in pulmonary “arterial” hypertension associated with congenital heart disease.

Chida-Nagai A, Sagawa K, Tsujioka T, Fujimoto T, Taniguchi K, Sasaki O, Izumi G, Yamazawa H, Masaki N, Manabe A, Takeda A.

Heart Vessels. 2020 Apr 13. doi: 10.1007/s00380-020-01604-1. [Epub ahead of print]

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  1. The COngenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry: a descriptive study from single-center hospital registry of adult congenital heart disease and pulmonary hypertension in Indonesia.

Dinarti LK, Hartopo AB, Kusuma AD, Satwiko MG, Hadwiono MR, Pradana AD, Anggrahini DW.

BMC Cardiovasc Disord. 2020 Apr 7;20(1):163. doi: 10.1186/s12872-020-01434-z.

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  1. Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

Ghosh RM, Griffis HM, Glatz AC, Rome JJ, Smith CL, Gillespie MJ, Whitehead KK, O’Byrne ML, Biko DM, Ravishankar C, Dewitt AG, Dori Y.

J Am Heart Assoc. 2020 Apr 7;9(7):e015318. doi: 10.1161/JAHA.119.015318. Epub 2020 Mar 30.

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  1. Autopsy in adults with congenital heart disease (ACHD).

Angelini A, di Gioia C, Doran H, Fedrigo M, Henriques de Gouveia R, Ho SY, Leone O, Sheppard MN, Thiene G, Dimopoulos K, Mulder B, Padalino M, van der Wal AC; Association for European Cardiovascular Pathology (AECVP).

Virchows Arch. 2020 Apr 7. doi: 10.1007/s00428-020-02779-8. [Epub ahead of print] Review.

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  1. Diffuse enlargement of cerebral vasculature in an adult patient operated for cyanotic congenital heart disease.

Emekli AS, Ekizoglu E, Yesilot N.

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  1. Lymphopenia in adults after the Fontan operation: prevalence and associations.

Alsaied T, Possner M, Brown N, Almeneisi H, Szugye C, Trout AT, Niss O, Palermo JJ, Zafar F, Dillman JR, Veldtman GR, Opotowsky AR, Lubert AM.

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  1. Influence of long-standing pulmonary arterial hypertension and its severity on pulmonary artery aneurysm development.

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Heart Vessels. 2020 Apr 4. doi: 10.1007/s00380-020-01600-5. [Epub ahead of print]

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  1. Multi-detector computed tomography in the assessment of tetralogy of Fallot patients: is it a must?

Shaaban M, Tantawy S, Elkafrawy F, Haroun D, Romeih S, Elmozy W.

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  1. The globe on the spotlight: Coronavirus disease 2019 (Covid-19).

Brida M, Chessa M, Gu H, Gatzoulis MA.

Int J Cardiol. 2020 Apr 3. pii: S0167-5273(20)31727-7. doi: 10.1016/j.ijcard.2020.04.006. [Epub ahead of print] No abstract available.

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Sandoval J, Del Valle-Mondragón L, Masso F, Zayas N, Pulido T, Teijeiro R, Gonzalez-Pacheco H, Olmedo-Ocampo R, Sisniega C, Paez-Arenas A, Pastelin-Hernandez G, Gomez-Arroyo J, Voelkel NF.

Eur Respir J. 2020 Apr 2. pii: 1902416. doi: 10.1183/13993003.02416-2019. [Epub ahead of print]

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Rev Esp Cardiol (Engl Ed). 2020 Apr 2. pii: S1885-5857(20)30097-9. doi: 10.1016/j.rec.2019.09.032. [Epub ahead of print] English, Spanish.

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  1. Providing a framework of principles for conceptualising the Fontan circulation.

Gewillig M, Brown SC, van de Bruaene A, Rychik J.

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  1. Emergency department evaluation of chest pain among adult congenital heart disease patients.

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  1. Contraceptive methods of privately insured US women with congenital heart defects.

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  1. Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.

Garcia AM, Beatty JT, Nakano SJ.

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  1. Alteration of cardiolipin biosynthesis and remodeling in single right ventricle congenital heart disease.

Garcia AM, McPhaul JC, Sparagna GC, Jeffrey DA, Jonscher R, Patel SS, Sucharov CC, Stauffer BL, Miyamoto SD, Chatfield KC.

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  1. Assessment of right ventricular function in patients with pulmonary arterial hypertension-congenital heart disease and repaired and unrepaired defects: Correlation among speckle tracking, conventional echocardiography, and clinical parameters.

Kemal HS, Kayıkçıoğlu M, Nalbantgil S, Can LH, Moğulkoç N, Kültürsay H.

Anatol J Cardiol. 2020 Apr;23(5):277-287. doi: 10.14744/AnatolJCardiol.2020.01379.

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  1. Platypnea-Orthodeoxia Syndrome in Fontan Circulation: Is It Gravity, the Height Difference Between the Right and Left Pulmonary Arteries, or Both?

Tajiri Y, Miyazaki A, Miyake M, Higaki R, Miki N, Misaki T, Doi H.

Can J Cardiol. 2020 Apr;36(4):590.e1-590.e2. doi: 10.1016/j.cjca.2019.10.024. Epub 2019 Nov 1. No abstract available.

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  1. Results of an Expert Consensus Survey on the Treatment of Pulmonary Arterial Hypertension With Oral Prostacyclin Pathway Agents.

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  1. Insights from examination of hearts from adults dying suddenly to the understanding of congenital cardiac malformations.

Westaby JD, Cooper STE, Edwards KA, Anderson RH, Sheppard MN.

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  1. Validation of simple measures of aortic distensibility based on standard 4-chamber cine CMR: a new approach for clinical studies.

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  1. Nursing research in congenital heart disease has grown up.

Moons P.

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  1. Perceived health competence predicts anxiety and depressive symptoms after a three-year follow-up among adolescents and adults with congenital heart disease.

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  1. An adult female with 5q34-q35.2 deletion: A rare syndromic presentation of left ventricular non-compaction and congenital heart disease.

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  1. Clinical characteristics in patients with rest angina and hypoplastic right coronary artery.

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  1. Systematic and Multidisciplinary Evaluation of Fibromuscular Dysplasia Patients Reveals High Prevalence of Previously Undetected Fibromuscular Dysplasia Lesions and Affects Clinical Decisions: The ARCADIA-POL Study.

Warchol-Celinska E, Prejbisz A, Dobrowolski P, Klisiewicz A, Kadziela J, Florczak E, Michalowska I, Jozwik-Plebanek K, Kabat M, Kwiatek P, Nazarewski S, Madej K, Rowinski O, Swiatlowski L, Peczkowska M, Hanus K, Talarowska P, Smolski M, Kowalczyk K, Kurkowska-Jastrzebska I, Stefanczyk L, Wiecek A, Widecka K, Tykarski A, Stryczynski L, Litwin M, Hoffman P, Witkowski A, Szczerbo-Trojanowska M, Januszewicz M, Januszewicz A.

Hypertension. 2020 Apr;75(4):1102-1109. doi: 10.1161/HYPERTENSIONAHA.119.13239. Epub 2020 Mar 9.

PMID: 32148126

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  1. Transition to adult care for adolescents with congenital heart disease – Is there a light at the end of the tunnel?

Vonder Muhll I.

Int J Cardiol. 2020 Apr 1. pii: S0167-5273(20)30612-4. doi: 10.1016/j.ijcard.2020.03.073. [Epub ahead of print] No abstract available.

PMID: 32276772

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  1. Coronary artery disease in patients with adult congenital heart disease.

Toyoda S, Sakuma M, Abe S, Nakajima T, Inoue T.

Int J Cardiol. 2020 Apr 1;304:35-36. doi: 10.1016/j.ijcard.2020.01.011. Epub 2020 Jan 16. No abstract available.

PMID: 31982163

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  1. Risk of coronary artery disease in adults with congenital heart disease: A comparison with the general population.

Kuijpers JM, Vaartjes I, Bokma JP, van Melle JP, Sieswerda GT, Konings TC, Bakker-de Boo M, van der Bilt I, Voogel B, Zwinderman AH, Mulder BJM, Bouma BJ.

Int J Cardiol. 2020 Apr 1;304:39-42. doi: 10.1016/j.ijcard.2019.11.114. Epub 2019 Nov 18.

PMID: 31767384

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  1. Right ventricular free wall strain predicts functional capacity in patients with repaired Tetralogy of Fallot.

Arroyo-Rodríguez C, Fritche-Salazar JF, Posada-Martínez EL, Arías-Godínez JA, Ortiz-León XA, Calvillo-Arguelles O, Ruiz-Esparza ME, Sandoval JP, Sierra-Lara D, Araiza-Garaygordobil D, Picano E, Rodríguez-Zanella H.

Int J Cardiovasc Imaging. 2020 Apr;36(4):595-604. doi: 10.1007/s10554-019-01753-z. Epub 2020 Jan 1.

PMID: 31894525

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  1. 4D flow vs. 2D cardiac MRI for the evaluation of pulmonary regurgitation and ventricular volume in repaired tetralogy of Fallot: a retrospective case control study.

Jacobs KG, Chan FP, Cheng JY, Vasanawala SS, Maskatia SA.

Int J Cardiovasc Imaging. 2020 Apr;36(4):657-669. doi: 10.1007/s10554-019-01751-1. Epub 2020 Jan 1.

PMID: 31894524

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  1. Performance of risk models predicting cardiac complications in pregnant women with congenital heart disease: a meta-analysis.

Wang TKM, Lowe B, Hlohovsky S, O’Donnell C.

Intern Med J. 2020 Apr;50(4):481-484. doi: 10.1111/imj.14810.

PMID: 32270622

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  1. Long-term results of aortic arch reconstruction with branch pulmonary artery homograft patches.

Lewis MJ, Johansson Ramgren J, Hallbergson A, Liuba P, Sjöberg G, Malm T.

J Card Surg. 2020 Apr;35(4):868-874. doi: 10.1111/jocs.14494. Epub 2020 Mar 11.

PMID: 32160354

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  1. Impact of race on survival in pulmonary arterial hypertension: Results from the REVEAL registry.

Medrek S, Sahay S, Zhao C, Selej M, Frost A.

J Heart Lung Transplant. 2020 Apr;39(4):321-330. doi: 10.1016/j.healun.2019.11.024. Epub 2020 Jan 24.

PMID: 32067864

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  1. Circulating NEDD9 is increased in pulmonary arterial hypertension: A multicenter, retrospective analysis.

Samokhin AO, Hsu S, Yu PB, Waxman AB, Alba GA, Wertheim BM, Hopkins CD, Bowman F, Channick RN, Nikolic I, Faria-Urbina M, Hassoun PM, Leopold JA, Tedford RJ, Ventetuolo CE, Leary PJ, Maron BA.

J Heart Lung Transplant. 2020 Apr;39(4):289-299. doi: 10.1016/j.healun.2019.12.002. Epub 2019 Dec 31.

PMID: 31952977

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  1. Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort.

Téllez L, Rodríguez de Santiago E, Minguez B, Payance A, Clemente A, Baiges A, Morales-Arraez D, La Mura V, Llop E, Garrido E, Garrido-Lestache E, Tasayco S, Bruno O, Prieto R, Montserrat S, Pons M, Olavarría A, Dos L, Legendre A, Jesús Del Cerro M, Bañares R, García-Pagán JC, Rautou PE, Albillos A; VALDIG an EASL consortium.

J Hepatol. 2020 Apr;72(4):702-710. doi: 10.1016/j.jhep.2019.10.027. Epub 2019 Nov 11.

PMID: 31726116

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  1. Dissecting visceral fibromuscular dysplasia reveals a new vascular phenotype of the disease: a report from the ARCADIA-POL study.

Warchoł-Celińska E, Pieluszczak K, Pappaccogli M, Soplińska A, Prejbisz A, Dobrowolski P, Klisiewicz A, Kądziela J, Falkowski A, Śmigielski W, Florczak E, Jóźwik-Plebanek K, Michałowska I, Kabat M, Zgorzelski C, Madej K, Nazarewski S, Smólski M, Olewnik Ł, Litwin M, Szczerbo-Trojanowska M, Zieniewicz K, Drygas W, Rowiński O, Witkowski A, Adlam D, Van der Niepen P, Persu A, Januszewicz A, Januszewicz M.

J Hypertens. 2020 Apr;38(4):737-744. doi: 10.1097/HJH.0000000000002327.

PMID: 31913220

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  1. Altered Ascending Aorta Hemodynamics in Patients After Arterial Switch Operation for Transposition of the Great Arteries.

van der Palen RLF, Deurvorst QS, Kroft LJM, van den Boogaard PJ, Hazekamp MG, Blom NA, Lamb HJ, Westenberg JJM, Roest AAW.

J Magn Reson Imaging. 2020 Apr;51(4):1105-1116. doi: 10.1002/jmri.26934. Epub 2019 Oct 7.

PMID: 31591799

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  1. Influence of pregnancy on long-term durability of allografts in right ventricular outflow tract.

Romeo JLR, Papageorgiou G, Takkenberg JJM, Roos-Hesselink JW, van Leeuwen WJ, Cornette JMJ, Rizopoulos D, Bogers AJJC, Mokhles MM.

J Thorac Cardiovasc Surg. 2020 Apr;159(4):1508-1516.e1. doi: 10.1016/j.jtcvs.2019.08.083. Epub 2019 Oct 4.

PMID: 31706555

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  1. Trans-Right-Ventricle and Transpulmonary MicroRNA Gradients in Human Pulmonary Arterial Hypertension.

Chouvarine P, Geldner J, Giagnorio R, Legchenko E, Bertram H, Hansmann G.

Pediatr Crit Care Med. 2020 Apr;21(4):340-349. doi: 10.1097/PCC.0000000000002207.

PMID: 31876555

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  1. Commentary: Shunting Between the Left Ventricle and Right Atrium Can Be Produced by Straddling Tricuspid Valve With Dual Orifices.

Lopes BS, Coats L, Anderson RH.

Semin Thorac Cardiovasc Surg. 2020 Spring;32(1):143-144. doi: 10.1053/j.semtcvs.2019.09.013. Epub 2019 Oct 2. No abstract available.

PMID: 31586467

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  1. COVID-19 in a Young Girl with Restrictive Cardiomyopathy and Chronic Lung Disease.

Inciyildirim A, Karaagac A.

Indian Pediatr. 2020 Apr 30. pii: S097475591600170. [Epub ahead of print] No abstract available.

PMID: 32358229 Free Article

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  1. Right Ventricular Systolic Function After the Cone Procedure for Ebstein’s Anomaly: Comparison Between Echocardiography and Cardiac Magnetic Resonance.

Lianza AC, Rodrigues ACT, Mercer-Rosa L, Vieira MLC, de Oliveira WAA, Afonso TR, Nomura CH, da Silva JP, da Silva LDF, Szarf G, Tavares GMP, Fischer CH, Morhy SS.

Pediatr Cardiol. 2020 Apr 25. doi: 10.1007/s00246-020-02347-6. [Epub ahead of print]

PMID: 32335735

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  1. Right Atrial Conduit Phase Emptying Predicts Risk of Adverse Events in Pediatric Pulmonary Arterial Hypertension.

Frank BS, Schafer M, Thomas TM, Haxel C, Ivy DD, Jone PN.

J Am Soc Echocardiogr. 2020 Apr 23. pii: S0894-7317(20)30131-0. doi: 10.1016/j.echo.2020.02.015. [Epub ahead of print]

PMID: 32336608

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  1. Cardiac Magnetic Resonance Imaging Features in Hypertrophic Cardiomyopathy Diagnosed at <21 Years of Age.

Bonura ED, Bos JM, Abdelsalam MA, Araoz PA, Ommen SR, Ackerman MJ, Geske JB.

Am J Cardiol. 2020 Apr 15;125(8):1249-1255. doi: 10.1016/j.amjcard.2020.01.027. Epub 2020 Jan 28.

PMID: 32088002

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  1. Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension: extended study results.

Aypar E, Alehan D, Karagöz T, Aykan H, Ertugrul İ.

Cardiol Young. 2020 Apr 15:1-5. doi: 10.1017/S1047951120000773. [Epub ahead of print]

PMID: 32290885

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  1. Cardiovascular complications in patients with total cavopulmonary connection: A nationwide cohort study.

Schmiegelow MD, Idorn L, Gislason G, Hlatky M, Køber L, Torp-Pedersen C, Søndergaard L.

Int J Cardiol. 2020 Apr 15;305:120-126. doi: 10.1016/j.ijcard.2019.12.062. Epub 2019 Dec 30.

PMID: 32001036

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  1. Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications.

Toprak B, Szöcs K, Zengin-Sahm E, Sinning C, Hot A, Bannas P, Hecher K, Hüneke B, Mir TS, Rybczynski M, Girdauskas E, Blankenberg S, von Kodolitsch Y.

J Clin Med. 2020 Apr 15;9(4). pii: E1124. doi: 10.3390/jcm9041124.

PMID: 32326432 Free Article

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  1. Pulmonary vasodilators can lead to various complications in pulmonary “arterial” hypertension associated with congenital heart disease.

Chida-Nagai A, Sagawa K, Tsujioka T, Fujimoto T, Taniguchi K, Sasaki O, Izumi G, Yamazawa H, Masaki N, Manabe A, Takeda A.

Heart Vessels. 2020 Apr 13. doi: 10.1007/s00380-020-01604-1. [Epub ahead of print]

PMID: 32285188 Free PMC Article

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  1. Myocardial localization of coronavirus in COVID-19 cardiogenic shock.

Tavazzi G, Pellegrini C, Maurelli M, Belliato M, Sciutti F, Bottazzi A, Sepe PA, Resasco T, Camporotondo R, Bruno R, Baldanti F, Paolucci S, Pelenghi S, Iotti GA, Mojoli F, Arbustini E.

Eur J Heart Fail. 2020 Apr 10. doi: 10.1002/ejhf.1828. [Epub ahead of print]

PMID: 32275347

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Congenital Heart and Pediatric Cardiac Electrophysiology Featured Articles

Congenital and Pediatric Cardiac EP Review of April 2020 Manuscripts

 

Congenital and Pediatric Cardiac EP Review of April 2020 Manuscripts

 

 

  1. Impact of Cardiac Resynchronization Therapy on Heart Transplant-Free Survival in Pediatric and Congenital Heart Disease Patients.

Chubb H, Rosenthal DN, Almond CS, Ceresnak SR, Motonaga KS, Arunamata AA, Long J, Trela AV, Hanisch D, McElhinney DB, Dubin AM.

Circ Arrhythm Electrophysiol. 2020 Apr;13(4):e007925. doi: 10.1161/CIRCEP.119.007925. Epub 2020 Mar 22.

PMID: 32202126

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Take Home Points:

 

  1. Cardiac Resynchronization Therapy (CRT) has become a mainstay in adult heart failure management and a potential option in children with and without congenital heart disease.
  2. CRT is associated with improved transplant-free survival in those patients with symptomatic heart failure (AHA Stage C or D), ventricular dysfunction (systemic ventricular EF <45%), and electrical dyssynchrony (QRS duration Z-score ≥ 3 or ventricular paced ≥ 40%).
  3. Heart transplantation or death was significantly lower in the CRT group compared to controls, 19% vs 58% (hazard ratio, 0.24, p<0.001).
  4. CRT was effective in pediatric patients with congenital heart disease, cardiomyopathy, and systemic RV failure.
  5. CRT was associated with sustained improvements in ejection fraction at 5 years

 

Comment from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch.  Heart failure among pediatric patients with and without congenital heart disease continues to provide challenges due to varying efficacy of anti-congestive therapy and limitations on advanced therapies such as heart transplantation. Cardiac resynchronization therapy (CRT) for the treatment of heart failure in adults without congenital heart disease (ACHD) is well established and has shown improvements in ventricular function, functional status, and mortality. The role of CRT in pediatric patients with and without congenital heart disease remains limited due to the small numbers of patients. In particular, the pediatric cohort is impacted by the large heterogeneity of congenital heart disease lesions, morphology of the systemic failing ventricle (right vs. left), variations in circulation (univentricular vs. biventricular), variations in patient size during childhood, and limited follow-up. In addition, the primary outcomes of CRT studies in pediatrics has focused on improvements in ventricular function and not on long term survival. This study aimed to assess the impact of CRT in pediatric patients with and without congenital heart disease on transplant-free survival.

 

This was a retrospective case-control single center study of pediatric patients  (≤ 21 years) with and without congenital heart disease from 2004-2017 who had reduced systemic ventricular function (ejection fraction < 45%),  symptomatic heart failure (AHA Stage C or D), and electrical dyssynchrony (unpaced QRS duration z-score ≥ 3 or ventricular pacing ≥ 40%).  Cases were enrolled at time of implant and defined as patients who underwent CRT implantation during this time frame with ≥ 1 lead on the systemic ventricle.  Controls were enrolled at first outpatient visit meeting inclusion criteria and were subsequently matched using propensity scoring.  Exclusion criteria included Eisenmenger syndrome, current VAD, previous heart transplant, and weight <4 kg. See Figure Below

 

The clinical practice for determining who underwent CRT placement during this time period was made on an individual basis through a multidisciplinary approach involving the heart failure, electrophysiology, and surgical teams.  After implantation, optimization of device programming was based on routine clinical practice which changed in 2016. Pre-2016, optimization was focused primarily on echocardiographic assessment for mechanical dyssynchrony and cardiac output. Post-2016, optimization was focused primarily on electrocardiographic assessment of  electrical dyssynchrony. Baseline clinical, device, electrocardiographic, and echocardiographic data were obtained. Follow-up data was obtained at 6 (±3) months, 1 (±0.5) years, 2 (±0.5) years and 5 (±1) year when available. Outcome measures included death or heart transplantation, overall survival, time to first transplant listing, and time to first heart failure hospitalization.

The study group included 86 with CRT, 133 controls,  and 63 propensity score matched (PSM) pairs. Of note, 6 cases (10%) had CRT turned off in > 1month for reason other than death or transplant. These patients were included in the CRT group for analysis. See Figure Below.

 

The median age of the CRT-PSM cohort was 11 years with 62% male and 81% with congenital heart disease. The systemic ventricle was left in 81% with a mean systemic EF of 32%. The  mean QRS duration z-score was 8.1. The median NHYA Class was 2. Only 5% were listed for transplant at baseline. There was no significant difference with controls across a total of 21 indices used match cases and controls (only 10 shown in table below).

 

There was no difference in type of circulation (biventricular vs univentricular) and systemic ventricle (left vs right) between the PSM-CRT and control groups.

 

The median follow-up was 2.7 (0.8 – 6.1) years overall and 2.4 (0.6 – 5.1) years for non-transplanted survivors.

 

The CRT approach was affected by size and anatomy with the majority of devices implanted being epicardial CRT-P. See Figure Below.

 

Device implantation
Approach
   Transvenous 17 (27%)
   Epicardial 43 (68%)
   Hybrid 3 (5%)
Device Type in CRT Group
    CRT-P at baseline 44 (70%)
    CRT-D at baseline 19 (30%)
    CRT-P to D upgrade during follow-up  1 (2%)
Device Type in Control Group
    ICD at baseline 12 (19%)
    ICD during follow-up 2 (3%)

 

Overall, heart transplantation or death was significantly lower in the CRT group compared to controls, 12 (19%) vs. 37 (58%), [HR of 0.24, (95% CI, 0.12–0.46), p <0.001]. In addition, the CRT group had a higher rate of overall survival and survival without heart failure hospitalization or transplant listing.  See Figures Below.

 

Risk factors associated with death or transplantation after multivariate analysis included increased risk with higher NHYA/Ross Class and decreased risk with use of CRT and presence of congenital heart disease.

 

Subgroup analysis showed CRT was effective in improving transplant-free survival in those with and without congenital heart disease, in those without and without the need for bradycardia pacing, and those with systemic right ventricles.  In this cohort, CRT in univentricular anatomy did not demonstrate significant improvement upon transplant-free survival.

Longitudinal follow-up showed the median QRS duration decreased 23 msec (95% CI, −38 to −6 msec) in the PSM-CRT group and increased 1 msec  (95% CI, −4 to +8 msec) in the PSM-control group at 6 months (P<0.001).  Of note, there was a disproportionate decline in PSM controls over time, but a significant increase in absolute QRS duration from baseline over time. There was no mention of Z-score change over time. See Figure Below.

 

Longitudinal follow-up showed a significant increase in median systemic ventricular EF  at 6 months,  11% (95% CI, +0.5% to +21%) in the PSM-CRT group compared to 0.1% (95% CI, −9.8% to +3.2%) in the PSM-control group  (P<0.001).   Of note, there was a disproportionate decline in PSM controls over time, but a significant increase in absolute and change from baseline EF over time.

Device complications were noted in 5% (acute) and 31% (chronic) with lead issues as the most common complication. There was no mortality with CRT procedures. See Table below.

 

Device therapy was noted in both groups. Appropriate shocks for VF/VT was seen in 4 patients in the CRT-D group and 2 in the control group with ICDs. Inappropriate shocks were seen in 2 patients in CRT-D group  and none in the control group with ICDs.

This study demonstrates the improved transplant-free survival for pediatric patients with and without congenital heart disease who received CRT for symptomatic heart failure (AHA Stage C or D and systemic EF < 45%) with electrical dyssynchrony  (QRS duration z-score ≥ 3 or ventricular pacing ≥ 40%). The design of this study allowed for a more robust retrospective analysis of CRT therapy in pediatric patients than prior studies using propensity score matching and providing a longer term of follow-up with a median of 2.7 years

Overall, there was improvement in ventricular function seen at 6 months and throughout follow-up to 5 years. However, a limitation of this study was the assessment of ventricular function with LV EF extrapolated from shortening fraction when EF by Simpson method was not available. In addition, the RV EF was estimated from fractional area change for the systemic RV. In addition, there was attrition during follow-up limiting data over time. Of further interest would be the assessment of CRT on longitudinal  function (EF)  in the univentricular heart which was not associated with survival benefit and in the systemic right ventricle which was associated with survival benefit in this cohort.

Heart failure functional status also plays a key role due to its impact on management decisions and survival outcomes. A lower function status at baseline was associated with a reduced risk for death or transplantation in this study. In addition, the presence of CRT was associated with improved survival to first heart failure hospitalization or transplant listing.  Due to sample size and objectives, subgroup analysis was not performed to assess risk factors for heart failure hospitalization or transplant listing.

QRS duration has been an important predictor of response to CRT in adults without congenital heart disease. All patients in this study had significant conduction disease and CRT resulted in a significant decrease in QRS duration. However, it appears that the paced QRS duration remained >120 msec in the majority potentially impacting the degree of response.  The varied response to CRT is multifactorial based on variations in bundle morphology (i.e. left vs right ventricular dyssynchrony), anatomy, lead locations, and optimization protocols. In this study, the location of multisite ventricular pacing positions was not standardized or analyzed.  In addition, the optimization prior to 2016 was based on echocardiographic assessments thus resulting in limited follow-up using electrocardiographic optimization. Despite these variations, CRT showed improvement in overall transplant-free survival.

Overall, this study showed that  81% of those who had CRT were alive without heart transplantation compared to the 42% in the control group.  This association between CRT and transplant-free survival remained significant  when controlling for confounders. However, due to the heterogeneity of patients and approaches in this study,  not all groups may demonstrate similar findings in a generalizable population.

This study provides additional data regarding the potential benefits of CRT in pediatric heart failure with and without congenital heart disease.  However, more data is needed with homogenous congenital populations, standardized approaches,  and longer term follow-up to determine those who will respond to CRT that can  be used to ultimately refine existing guidelines.

Management of heart failure in pediatric patients is important to reduce mortality, transplantations, hospitalizations, and comorbidities. As mentioned in this study, a multidisciplinary  approach is needed when determining who and how one should receive CRT therapy. In addition, medical and device optimization management will continue to require an individualized approach.

 

 

Congenital Heart and Pediatric Cardiac EP Abstracts of April 2020

 

  1. The standardized 12-lead fetal electrocardiogram of the healthy fetus in mid-pregnancy: A cross-sectional study.

Lempersz C, van Laar JO, Clur SB, Verdurmen KM, Warmerdam GJ, van der Post J, Blom NA, Delhaas T, Oei SG, Vullings R.

PLoS One. 2020 Apr 30;15(4):e0232606. doi: 10.1371/journal.pone.0232606. eCollection 2020.

PMID: 32353083 Free Article

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  1. Cardiac arrhythmias in pregnant women: need for mother and offspring protection.

Manolis TA, Manolis AA, Apostolopoulos EJ, Papatheou D, Melita H, Manolis AS.

Curr Med Res Opin. 2020 Apr 29:1. doi: 10.1080/03007995.2020.1762555. [Epub ahead of print]

PMID: 32347120

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  1. Permanent left bundle branch area pacing in a child with a third-degree atrioventricular block: A case report.

Huang J, Zhou R, Pan Y, Yang B.

J Cardiovasc Electrophysiol. 2020 Apr 26. doi: 10.1111/jce.14520. [Epub ahead of print]

PMID: 32337777

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  1. Value of provocative electrophysiology testing in the management of pediatric patients after congenital heart surgery.

Dionne A, Kheir JN, Gauvreau K, Triedman JK, Abrams DJ, Alexander ME, DeWitt ES, Mah DY, Walsh EP, Bezzerides VJ.

Pacing Clin Electrophysiol. 2020 Apr 24. doi: 10.1111/pace.13925. [Epub ahead of print]

PMID: 32329521

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  1. We Have Plenty of Reasons to Propose New, Updated Policies for Preventing Sudden Cardiac Death in Young Athletes.

Angelini P, Muthupillai R, Cheong B, Paisley R.

J Am Heart Assoc. 2020 Apr 21;9(8):e014368. doi: 10.1161/JAHA.119.014368. Epub 2020 Apr 20. No abstract available.

PMID: 32306825 Free Article

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  1. Early detection of ventricular arrhythmias in adults with congenital heart disease using an insertable cardiac monitor (EDVA-CHD study).

Sakhi R, Kauling RM, Theuns DA, Szili-Torok T, Bhagwandien RE, van den Bosch AE, Cuypers JAAE, Roos-Hesselink JW, Yap SC.

Int J Cardiol. 2020 Apr 15;305:63-69. doi: 10.1016/j.ijcard.2020.02.009. Epub 2020 Feb 4.

PMID: 32057477 Free Article

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  1. Implantable cardiac electronic device therapy for patients with a systemic right ventricle.

Barracano R, Brida M, Guarguagli S, Palmieri R, Diller GP, Gatzoulis MA, Wong T.

Heart. 2020 Apr 8. pii: heartjnl-2019-316202. doi: 10.1136/heartjnl-2019-316202. [Epub ahead of print] Review.

PMID: 32269130

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  1. Frontal QRS-T angle and ventricular mechanics in congenital heart disease.

Lau LY, So EK, Chow PC, Cheung YF.

Heart Vessels. 2020 Apr 3. doi: 10.1007/s00380-020-01601-4. [Epub ahead of print]

PMID: 32246195

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  1. Autoimmune-mediated congenital heart block.

Wainwright B, Bhan R, Trad C, Cohen R, Saxena A, Buyon J, Izmirly P.

Best Pract Res Clin Obstet Gynaecol. 2020 Apr;64:41-51. doi: 10.1016/j.bpobgyn.2019.09.001. Epub 2019 Oct 8. Review.

PMID: 31685414

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  1. Impact of Cardiac Resynchronization Therapy on Heart Transplant-Free Survival in Pediatric and Congenital Heart Disease Patients.

Chubb H, Rosenthal DN, Almond CS, Ceresnak SR, Motonaga KS, Arunamata AA, Long J, Trela AV, Hanisch D, McElhinney DB, Dubin AM.

Circ Arrhythm Electrophysiol. 2020 Apr;13(4):e007925. doi: 10.1161/CIRCEP.119.007925. Epub 2020 Mar 22.

PMID: 32202126

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  1. Heart rate variability is depressed in the early transitional period for newborns with complex congenital heart disease.

Mulkey SB, Govindan R, Metzler M, Swisher CB, Hitchings L, Wang Y, Baker R, Larry Maxwell G, Krishnan A, du Plessis AJ.

Clin Auton Res. 2020 Apr;30(2):165-172. doi: 10.1007/s10286-019-00616-w. Epub 2019 Jun 25.

PMID: 31240423

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  1. The Brain-Heart Connection in Sympathetically Triggered Inherited Arrhythmia Syndromes.

Winbo A, Paterson DJ.

Heart Lung Circ. 2020 Apr;29(4):529-537. doi: 10.1016/j.hlc.2019.11.002. Epub 2019 Dec 16. Review.

PMID: 31959550

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  1. Reply to the Letter to the Editor: “Exploring the role of atrial arrhythmias in adults with congenital heart disease”.

Khairy P.

Int J Cardiol. 2020 Apr 1;304:38. doi: 10.1016/j.ijcard.2020.01.004. No abstract available.

PMID: 32178797

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  1. Exploring the role of atrial arrhythmias in adults with congenital heart disease.

Pasqualin G.

Int J Cardiol. 2020 Apr 1;304:37. doi: 10.1016/j.ijcard.2019.11.124. No abstract available.

PMID: 32178796

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  1. Evolution of ventricular function in children with permanent right ventricular pacing after tetralogy of Fallot repair: A midterm follow-up.

El-Shabrawi M, Lotfy W, Hegazy R, Abdelaziz O, Sobhy R, Abdelmohsen G, Ibrahim H, Dohain AM.

J Card Surg. 2020 Apr;35(4):831-839. doi: 10.1111/jocs.14477. Epub 2020 Feb 24.

PMID: 32092198

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  1. Intravenous Sotalol in the Young: Safe and Effective Treatment With Standardized Protocols.

Borquez AA, Aljohani OA, Williams MR, Perry JC.

JACC Clin Electrophysiol. 2020 Apr;6(4):425-432. doi: 10.1016/j.jacep.2019.11.019. Epub 2020 Feb 26.

PMID: 32327076

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  1. Prevalence and Outcomes of Pediatric In-Hospital Cardiac Arrest Associated With Pulmonary Hypertension.

Morgan RW, Topjian AA, Wang Y, Atkin NJ, Kilbaugh TJ, McGowan FX, Berg RA, Mercer-Rosa L, Sutton RM, Himebauch AS.

Pediatr Crit Care Med. 2020 Apr;21(4):305-313. doi: 10.1097/PCC.0000000000002187.

PMID: 31688674

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  1. Association between severity of cardiac dysfunction caused by ventricular pre-excitation-led dyssynchrony and cardiac function recovery after ablation in children.

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

J Cardiovasc Electrophysiol. 2020 Apr 28. doi: 10.1111/jce.14521. [Epub ahead of print]

PMID: 32343451

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  1. A distinct molecular mechanism by which phenytoin rescues a novel long QT 3 variant.

Gando I, Campana C, Tan RB, Cecchin F, Sobie EA, Coetzee WA.

J Mol Cell Cardiol. 2020 Apr 24;144:1-11. doi: 10.1016/j.yjmcc.2020.04.027. [Epub ahead of print]

PMID: 32339567

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  1. Genetic variants of uncertain significance: How to match scientific rigour and standard of proof in sudden cardiac death?

Grassi S, Campuzano O, Coll M, Brión M, Arena V, Iglesias A, Carracedo Á, Brugada R, Oliva A.

Leg Med (Tokyo). 2020 Apr 23;45:101712. doi: 10.1016/j.legalmed.2020.101712. [Epub ahead of print]

PMID: 32361481 Free Article

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  1. Time to Redefine the Natural History and Clinical Management of Type 1 Andersen-Tawil Syndrome?

Ackerman MJ, Giudicessi JR.

J Am Coll Cardiol. 2020 Apr 21;75(15):1785-1787. doi: 10.1016/j.jacc.2020.03.005. No abstract available.

PMID: 32299590

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  1. Generation of three iPSC lines (XACHi007-A, XACHi008-A, XACHi009-A) from a Chinese family with long QT syndrome type 5 with heterozygous c.226G>A (p.D76N) mutation in KCNE1gene.

Zhang Y, Li H, Wang J, Wang G, Tan X, Lei M.

Stem Cell Res. 2020 Apr 20;45:101798. doi: 10.1016/j.scr.2020.101798. [Epub ahead of print]

PMID: 32344329 Free Article

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  1. Recurrent single echo beats after cryoablation of atrioventricular nodal reentrant tachycardia: The pediatric population.

Dasgupta S, Kelleman M, Whitehill R, Fischbach P.

Indian Pacing Electrophysiol J. 2020 Apr 18. pii: S0972-6292(20)30044-9. doi: 10.1016/j.ipej.2020.04.007. [Epub ahead of print]

PMID: 32311435 Free Article

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  1. Extremely late recurrences (≥3 years) of atrioventricular nodal reentrant tachycardia: Electrophysiological characteristics of the index and repeat ablation procedures.

Chin CG, Chung FP, Lin YJ, Chang SL, Lo LW, Hu YF, Tuan TC, Chao TF, Liao JN, Lin CY, Chang TY, Vicera JJB, Chen CC, Chuang CM, Cheng WH, Liu SH, Hsieh MH, Chen SA.

Int J Cardiol. 2020 Apr 15;305:70-75. doi: 10.1016/j.ijcard.2020.02.007. Epub 2020 Feb 4.

PMID: 32059994

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  1. Prevalence, predictors and complications with defibrillation threshold testing in pediatric patients: Results from the NCDR.

Prutkin JM, Wang Y, Escudero CA, Stephenson EA, Minges KE, Curtis JP, Hsu JC.

Int J Cardiol. 2020 Apr 15;305:44-49. doi: 10.1016/j.ijcard.2020.01.027. Epub 2020 Jan 15.

PMID: 31980272

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  1. Validity of administrative claims-based algorithms for ventricular arrhythmia and cardiac arrest in the pediatric population.

Czaja AS, Collins K, Valuck RJ, Anderson HD, Ghosh D, Davidson JA.

Pharmacoepidemiol Drug Saf. 2020 Apr 13. doi: 10.1002/pds.5001. [Epub ahead of print]

PMID: 32283564

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  1. Assessing the cost-effectiveness of home automated external defibrillators for pediatric patients: Getting us closer to the truth.

Kaltman JR.

Heart Rhythm. 2020 Apr 8. pii: S1547-5271(20)30291-5. doi: 10.1016/j.hrthm.2020.03.029. [Epub ahead of print] No abstract available.

PMID: 32278073

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  1. Left cardiac sympathetic denervation reduces skin sympathetic nerve activity in patients with long QT syndrome.

Han J, Ackerman MJ, Moir C, Cai C, Xiao PL, Zhang P, Briske KA, Zheng LR, Chen PS, Cha YM.

Heart Rhythm. 2020 Apr 8. pii: S1547-5271(20)30284-8. doi: 10.1016/j.hrthm.2020.03.023. [Epub ahead of print]

PMID: 32276050

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  1. Urgent Guidance for Navigating and Circumventing the QTc-Prolonging and Torsadogenic Potential of Possible Pharmacotherapies for Coronavirus Disease 19 (COVID-19).

Giudicessi JR, Noseworthy PA, Friedman PA, Ackerman MJ.

Mayo Clin Proc. 2020 Apr 7. pii: S0025-6196(20)30313-X. doi: 10.1016/j.mayocp.2020.03.024. [Epub ahead of print]

PMID: 32359771 Free PMC Article

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  1. Ventricular arrhythmia suppression with ivabradine in a patient with catecholaminergic polymorphic ventricular tachycardia refractory to nadolol, flecainide, and sympathectomy.

Kohli U, Aziz Z, Beaser AD, Nayak HM.

Pacing Clin Electrophysiol. 2020 Apr 7. doi: 10.1111/pace.13913. [Epub ahead of print]

PMID: 32259298

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  1. Possible Propofol-Induced Priapism Following Cardiac Catheter Ablation in a Teenager.

Young ML, Lam S.

Am J Case Rep. 2020 Apr 5;21:e920692. doi: 10.12659/AJCR.920692.

PMID: 32248201 Free PMC Article

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  1. Study of the time-relationship of the mechano-electrical interaction in an animal model of tetralogy of Fallot: implications for the risk assessment of ventricular arrhythmias.

Bove T, Alipour Symakani R, Verbeke J, Vral A, El Haddad M, De Wilde H, Stroobandt R, De Pooter J.

Interact Cardiovasc Thorac Surg. 2020 Apr 3. pii: ivaa047. doi: 10.1093/icvts/ivaa047. [Epub ahead of print]

PMID: 32243531

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  1. Intravenous Sotalol in the Young: Safe and Effective Treatment With Standardized Protocols.

Borquez AA, Aljohani OA, Williams MR, Perry JC.

JACC Clin Electrophysiol. 2020 Apr;6(4):425-432. doi: 10.1016/j.jacep.2019.11.019. Epub 2020 Feb 26.

PMID: 32327076

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  1. Lidocaine versus amiodarone for pediatric in-hospital cardiac arrest: An observational study.

Holmberg MJ, Ross CE, Atkins DL, Valdes SO, Donnino MW, Andersen LW; American Heart Association’s for the AHA’s Get With The Guidelines®-Resuscitation Pediatric Research Task Force.

Resuscitation. 2020 Apr;149:191-201. doi: 10.1016/j.resuscitation.2019.12.033. Epub 2020 Jan 16.

PMID: 31954741

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  1. Prevalence and electrophysiological phenotype of rare SCN5A genetic variants identified in unexplained sudden cardiac arrest survivors.

Giudicessi JR, Ye D, Stutzman MJ, Zhou W, Tester DJ, Ackerman MJ.

Europace. 2020 Apr 1;22(4):622-631. doi: 10.1093/europace/euz337.

PMID: 32091595

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  1. Percutaneous continuous left stellate ganglion block as an effective bridge to bilateral cardiac sympathetic denervation.

Savastano S, Pugliese L, Baldi E, Dusi V, Tavazzi G, De Ferrari GM.

Europace. 2020 Apr 1;22(4):606. doi: 10.1093/europace/euaa007. No abstract available.

PMID: 32034906

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  1. A Review of Long QT Syndrome: Everything a Hospitalist Should Know.

Sharma N, Cortez D, Disori K, Imundo JR, Beck M.

Hosp Pediatr. 2020 Apr;10(4):369-375. doi: 10.1542/hpeds.2019-0139. Epub 2020 Mar 6. Review.

PMID: 32144177

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  1. Management of Arrhythmias in Pediatric Emergency.

Rohit M, Kasinadhuni G.

Indian J Pediatr. 2020 Apr;87(4):295-304. doi: 10.1007/s12098-020-03267-2. Epub 2020 Mar 13. Review.

PMID: 32166608

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  1. Effects of maternal sleep position on fetal and maternal heart rate patterns using overnight home fetal ECG recordings.

Lucchini M, Wapner RJ, Chia-Ling NC, Torres C, Yang J, Williams IA, Fifer WP.

Int J Gynaecol Obstet. 2020 Apr;149(1):82-87. doi: 10.1002/ijgo.13096. Epub 2020 Jan 30.

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  1. Left sinus of Valsalva-Electroanatomic basis and outcomes with ablation for outflow tract arrhythmias.

Kapa S, Mehra N, Deshmukh AJ, Friedman PA, Asirvatham SJ.

J Cardiovasc Electrophysiol. 2020 Apr;31(4):952-959. doi: 10.1111/jce.14388. Epub 2020 Feb 18.

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  1. A novel percutaneous stabilizing sheath for minimal invasive epicardial echocardiography and ablation.

Sugrue A, Vaidya VR, Padmanabhan D, Yasin O, Abudan A, Isath A, Killu AM, Naksuk N, Bolon B, Friedman PA, Asirvatham SJ.

J Interv Card Electrophysiol. 2020 Apr;57(3):453-464. doi: 10.1007/s10840-019-00553-8. Epub 2019 Jun 6.

PMID: 31172420

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  1. An Exciting New Tool in the Electrophysiologist’s Toolbox, Intravenous Sotalol: Faster, Safer, Better?

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JACC Clin Electrophysiol. 2020 Apr;6(4):433-435. doi: 10.1016/j.jacep.2019.12.016. No abstract available.

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Congenital Heart Interventions Featured Articles

Interventional Cardiology Reviews of April 2020 Manuscripts

 

Congenital Heart Interventions Review of April 2020 Manuscripts

 

Here today, gone tomorrow: Outcomes of residual leak following secundum atrial septal defect closure with the GORE CARDIOFORM Septal Occluder.

Gordon BM, Abudayyeh I, Goble J, Collado NA, Paolillo J.Catheter Cardiovasc Interv. 2020 Apr 1;95(5):932-936. doi: 10.1002/ccd.28666. Epub 2019 Dec 26.PMID: 31876383

 

Take Home Points:

 

  • Residual leaks following device closure of secundum atrial septal defects, particularly when using the GORE CARDIOFORM Septal Occluder, are poorly understood.
  • Patients with larger defects, smaller aortic rims, and with the presence of multiple fenestrations are more likely to have residual leaks following ASD device closure with the GSO.
  • Residual leaks following ASD device closure with the GSO frequently disappear within the first year following implantation.

 

Commentary from Dr. Arash  Salavitabar (Ann Arbor, MI), section editor of Congenital Heart Disease Interventions Journal Watch:  In this retrospective, multicenter study, the authors aimed to review experiences with outcomes of residual leaks following device closure of secundum atrial septal defects (ASD) with the GORE CARDIOFORM Septal Occluder (GSO) during the pivotal and continued access study. This is a well-known potential issue post-device closure of ASDs and the expectant course is not well-described, particularly with this device. One important characteristic of the GSO device is that it is not “self-centering”, and so it has the ability to shift as it conforms to the septal anatomy. This can lead to potential small leaks around the edge of the device. The authors sought to characterize the medium-term outcomes of residual leaks noted with the GSO and to report on potential risk factors associated with eventual closure rates.

This study included 69 patients who had a residual leak following device implantation, out of 374 total patients who underwent ASD closure with the GSO for the pivotal and continued access U.S. trials. Sixty-five (17.5%) patients met inclusion criteria following retrospective review of their echocardiograms. When comparing those with and without residual leak, those with residual leaks had larger defects (10.33 ± 3.05 mm vs. 9.13 ± 2.89 mm, p = .006), larger stop flow balloon sizes (12.91 ± 3.02 mm vs. 11.43 ± 2.89 mm, p < .001), smaller aortic rims (4.87 ± 3.33 mm vs. 6.17 ± 3.78 mm, p = .019), the presence of multiple fenestrations (43.08% vs. 18.69%, p < .001), and increased fluoroscopic time (16.02 ± 9.65 min vs. 13.17 ± 9.03 min, p = .004). Larger devices tended to be implanted and more devices per case were utilized in the residual leak cohort as compared to those without leak. There was no significant differences between procedural time, device- to-defect ratio, or type of anesthesia among groups.

Importantly, there was a significant decrease in the leak size over 1 year in those patients with residual leak, from 1.55 ± 0.75mm to 0.25 ± 0.74mm (p < 0.001), with the majority disappearing by that 1 year follow-up (Figure 1).

 

The authors postulate that residual shunts adjacent to ASD closure devices disappear over time due to remodeling of the right atrium with subsequent normalization of right atrial size following removal of the volume load caused by the ASD. This is thought to augment the endothelialization that occurs from adjacent tissue.

While this study is limited by its retrospective nature, it provides important information regarding risk factors for residual leak following ASD device closure, particularly with the GSO. It is also valuable to understand that these residual leaks frequently completely disappear over the first year following implantation, which can affect patient counseling, frequency of follow-up, and potential need for future interventions.

 

  1. Incidence and fate of device-related left pulmonary artery stenosis and aortic coarctation in small infants undergoing transcatheter patent ductus arteriosus closure.

Tomasulo CE, Gillespie MJ, Munson D, Demkin T, O’Byrne ML, Dori Y, Smith CL, Rome JJ, Glatz AC.

Catheter Cardiovasc Interv. 2020 Apr 27. doi: 10.1002/ccd.28942. [Epub ahead of print]

PMID: 32339400

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Select item 32338402

 

Take Home Points: 

  • Transcatheter PDA closure is the preferred treatment option for nearly all patients with PDAs, even those near 1 kg, at most centers
  • LPA stenosis and aortic coarctation are known risk factors in infants undergoing PDA closure
  • This study supports that the majority of LPA and aortic obstruction tend to improve over time

Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Congenital Heart Disease Interventions Journal Watch:   Transcatheter PDA closure (TC-PDA) is at least as safe and as efficacious as surgical ligation and offers a lower morbidity option. It has long been the standard for larger infants, children, and adults. The number of available devices that are able to be delivered through small sheaths has led to improved outcomes in TC-PDA and allowed for closure in smaller infants who were previously routinely referred for surgical PDA ligation. Left pulmonary artery (LPA) stenosis and aortic coarctation are known concerns in this patient population and are well described. However, longer term outcomes of these complications are not known.

Tomasulo et al. report on a retrospective analysis of all infants ≤ 4 kg who underwent TC-PDA from 2007-2018. A total of 44 infants met inclusion criteria and had successful TC-PDA. The median weight was 2.8 kg (range 1.2-4 kg), with a trend towards lower weights later in the study time frame (6/10 from 2018 weighed <2 kg). The majority of patients (30/44) underwent PDA closure with an Amplatzer Vascular Plug II (AVP-II), 10 with an Amplatzer Duct Occluder II-Additional sizes (ADOII-AS, now known as the Piccolo device), 3 with an Amplatzer Duct Occluder I (ADO1), and 1 with an Amplatzer Vascular Plug I filled with 3 Cook embolization coils. The devices were placed via an antegrade approach from the femoral vein in all patients. Arterial access was obtained in 59% of patients, though in only 3/17 patients since 2017 (and none who had an ADOII-AS device placed). Type F (61%) and Type C (23%) PDAs made up the large majority of cases.

Of the 44 patients, 39 had post procedure echocardiograms performed, all of which showed complete closure of the PDA. Angiography showed mild or less residual shunt in the other 5. Median follow up was 0.7 years (range 2 days-7 years) with 38 patients having assessment for LPA stenosis and aortic coarctation. Of these 38 patients, 21 patients (55%) had obstruction in at least 1 vessel. A total of 17 patients had mild flow acceleration (defined as an echocardiographic flow velocity of 1.5-2 m/sec) or stenosis (flow velocity >2 m/sec) in the LPA (though 3 of these already had flow acceleration/stenosis on their pre procedure echocardiogram). The majority of these improved as shown below. Those who developed LPA obstruction were younger, had larger PDAs, and were less likely to have an AVP II device placed.

A total of 4 patients developed flow acceleration in the aorta (flow velocity 1.5-2 m/sec) and 3 developed mild aortic coarctation (flow velocity >2 m/sec). Of these, 4/7 had improved at the time of last follow up as detailed below. Lower birth weight was the only statistically significant factor associated with aortic obstruction, though there was a trend towards later gestational age and shorter PDAs.

 

 

This study confirms that LPA and aortic obstruction are frequently seen in TC-PDA in small infants. However, the amount of obstruction seen is typically quite mild and likely clinically irrelevant. Additionally, most of the obstruction improves over time and none of the patients in this cohort required additional intervention (though other studies on TC-PDA closure in small infants have shown a small number requiring intervention). While this study adds reassuring information to the literature, the types of devices being used in this population is changing. The Piccolo device now has FDA approval for TC-PDA in this patient population and is now most interventionalists go to device. Additionally, the MVP microvascular plug (Medtronic) has frequently been used for TC-PDA closure in small infants and is not reported on in this study. Future studies are warranted to evaluate these known problems with the devices that are more frequently used today.

 

CHD Interventions Abstracts of April 2020

 

  1. Transcatheter biventricular conversion in an adult patient with a 1.5 ventricle Glenn palliation and superior vena cava syndrome.

Tannous P, Popescu A, Forbess L, Nugent A.

Catheter Cardiovasc Interv. 2020 Apr 28. doi: 10.1002/ccd.28920. [Epub ahead of print]

PMID: 32343465

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  1. Incidence and fate of device-related left pulmonary artery stenosis and aortic coarctation in small infants undergoing transcatheter patent ductus arteriosus closure.

Tomasulo CE, Gillespie MJ, Munson D, Demkin T, O’Byrne ML, Dori Y, Smith CL, Rome JJ, Glatz AC.

Catheter Cardiovasc Interv. 2020 Apr 27. doi: 10.1002/ccd.28942. [Epub ahead of print]

PMID: 32339400

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  1. Echocardiography-fluoroscopy fusion imaging: The essential features used in congenital and structural heart disease interventional guidance.

Jone PN, Sandoval JP, Haak A, Hammers J, Rodriguez-Zanella H, Quaife RA, Salcedo EE, Carroll JD, Gill E.

Echocardiography. 2020 Apr 27. doi: 10.1111/echo.14670. [Epub ahead of print] Review.

PMID: 32338402

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  1. A Case Series Describing Percutaneous Management of Aortic Isthmic Atresia.

Menon PJ, Walsh K.

Vasc Endovascular Surg. 2020 Apr 26:1538574420921280. doi: 10.1177/1538574420921280. [Epub ahead of print]

PMID: 32338186

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  1. Malignant right coronary artery origin from the left sinus of Valsalva: Complementary role for transesophageal echocardiography upon the cath-lab diagnosis.

de Gregorio C, Ceresa F, Ferrazzo G, Patanè F.

J Clin Ultrasound. 2020 Apr 25. doi: 10.1002/jcu.22845. [Epub ahead of print]

PMID: 32333791

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  1. Partnership Between Japan and the United States for Early Development of Pediatric Medical Devices - Harmonization By Doing for Children.

Takahashi S, Ibrahim N, Yasukochi S, Ringel R, Ing F, Tomita H, Sugiyama H, Yamagishi M, Forbes TJ, Kim SH, Ho M, Gillette N, Nakamura Y, Mineta K, Fearnot N, Dineen D, Vang E, Haskin R, Becker LAM, Sekiguchi K, Sakamoto K, Ruiz CE; Harmonization by Doing for Children Working Group.

Circ J. 2020 Apr 24;84(5):786-791. doi: 10.1253/circj.CJ-19-1092. Epub 2020 Apr 1.

PMID: 32238666 Free Article

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  1. Three-Dimensional Rotational Angiography during Catheterization of Congenital Heart Disease – A ten Years’ experience at a single center.

Söder S, Wällisch W, Dittrich S, Cesnjevar R, Pfammatter JP, Glöckler M.

Sci Rep. 2020 Apr 24;10(1):6973. doi: 10.1038/s41598-020-63903-x.

PMID: 32332807 Free PMC Article

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  1. Short-term results of interventional therapy for infants (7-36 months old) with patent ductus arteriosus and moderate-to-severe pulmonary hypertension: a retrospective study.

Shu Y, Guo Y, Wang X, Zhou D.

J Cardiothorac Surg. 2020 Apr 22;15(1):68. doi: 10.1186/s13019-020-01110-5.

PMID: 32321548 Free PMC Article

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  1. Feasibility and early outcomes of aortic coarctation treatments with BeGraft Aortic stent.

Promphan W, Han Siang K, Prachasilchai P, Jarutach J, Makonkawkeyoon K, Siwaprapakorn W, Chutimapongrat N, Sueachim P, Butchan Y.

Catheter Cardiovasc Interv. 2020 Apr 16. doi: 10.1002/ccd.28892. [Epub ahead of print]

PMID: 32299151

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  1. Pilot phase experience of the International Quality Improvement Collaborative catheterization registry.

Barry OM, Ali F, Ronderos M, Sudhaker A, Kumar RK, Mood MC, Corona-Villalobos C, Nguyen DT, Doherty-Schmeck K, Bergersen L, Gauvreau K, Jenkins KJ, Hasan BS.

Catheter Cardiovasc Interv. 2020 Apr 15. doi: 10.1002/ccd.28908. [Epub ahead of print]

PMID: 32294315

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  1. Preclinical comparative assessment of a dedicated pediatric poly-L-lactic-acid-based bioresorbable scaffold with a low-profile bare metal stent.

Shibbani K, De Lima E Silva Bagno L, Poulin MF, Matella T, Diab K, Kavinsky C, Ramesh N, Bhat V, Hijazi ZM, Kenny D.

Catheter Cardiovasc Interv. 2020 Apr 15. doi: 10.1002/ccd.28893. [Epub ahead of print]

PMID: 32294303

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  1. Velocity Vector Imaging Assessment of Functional Change in the Right Ventricle during Transcatheter Closure of Atrial Septal Defect by Intracardiac Echocardiography.

Jung SY, Shin JI, Choi JY, Park SJ, Kim NK.

J Clin Med. 2020 Apr 15;9(4). pii: E1132. doi: 10.3390/jcm9041132.

PMID: 32326588 Free Article

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  1. SCAI position statement on adult congenital cardiac interventional training, competencies and organizational recommendations.

Aboulhosn JA, Hijazi ZM, Kavinsky CJ, McElhinney DB, Asgar AW, Benson LN, Daniels CJ, Ghobrial J, Horlick E, Ing FF, Inglessis I, Kay J, Levi DS.

Catheter Cardiovasc Interv. 2020 Apr 9. doi: 10.1002/ccd.28885. [Epub ahead of print] No abstract available.

PMID: 32272495

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Select item 32271829

 

  1. Surgically placed radiopaque markers: Proof-of-concept of a novel technique to facilitate percutaneous interventions in neonates and infants.

Hummel J, Kubicki R, Pingpoh C, Stiller B, Sigler M, Siepe M, Grohmann J.

Catheter Cardiovasc Interv. 2020 Apr 8. doi: 10.1002/ccd.28891. [Epub ahead of print]

PMID: 32267611

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  1. Percutaneous coronary intervention following Fontan procedure.

Subahi A, Forbes T, Ali OE.

Int J Cardiol Heart Vasc. 2020 Apr 8;28:100511. doi: 10.1016/j.ijcha.2020.100511. eCollection 2020 Jun.

PMID: 32300638 Free PMC Article

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  1. Coronary artery fistulas detected with coronary CT angiography: a pictorial review of 73 cases.

Li N, Zhao P, Wu D, Liang C.

Br J Radiol. 2020 Apr;93(1108):20190523. doi: 10.1259/bjr.20190523. Epub 2019 Oct 31.

PMID: 31638419

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  1. Hybrid trans-apical atrioventricular valve-in-valve implantation in Fontan circulation.

Butera G, Lucchese G, Austin C, Frigiola A.

Catheter Cardiovasc Interv. 2020 Apr 1;95(5):950-953. doi: 10.1002/ccd.28714. Epub 2020 Jan 8.

PMID: 31912986

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Select item 31876383

 

  1. Here today, gone tomorrow: Outcomes of residual leak following secundum atrial septal defect closure with the GORE CARDIOFORM Septal Occluder.

Gordon BM, Abudayyeh I, Goble J, Collado NA, Paolillo J.

Catheter Cardiovasc Interv. 2020 Apr 1;95(5):932-936. doi: 10.1002/ccd.28666. Epub 2019 Dec 26.

PMID: 31876383

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  1. Light anti-thrombotic regimen for prevention of device thrombosis and/or thrombotic complications after interatrial shunts device-based closure.

Rigatelli G, Zuin M, Dell’Avvocata F, Roncon L, Vassilev D, Nghia N.

Eur J Intern Med. 2020 Apr;74:42-48. doi: 10.1016/j.ejim.2019.12.010. Epub 2020 Jan 3.

PMID: 31902564

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  1. Percutaneous Pulmonary Valve Implantation.

Giugno L, Faccini A, Carminati M.

Korean Circ J. 2020 Apr;50(4):302-316. doi: 10.4070/kcj.2019.0291. Review.

PMID: 32157831 Free PMC Article

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  1. Cardiac catheterization procedures in children with congenital heart disease: Increased chromosomal aberrations in peripheral lymphocytes.

Gao Y, Wang P, Su Y, Wang Z, Han L, Li J, Fu Y, Zhao F, Sun Q, Lyu Y.

Mutat Res. 2020 Apr;852:503163. doi: 10.1016/j.mrgentox.2020.503163. Epub 2020 Feb 25.

PMID: 32265037

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  1. Combined hybrid pulmonary valve placement and atrial septal defect closure: case report and literature review.

Abu-Anza O, Carr K, Aldoss O.

Cardiol Young. 2020 Apr 27:1-3. doi: 10.1017/S1047951120000724. [Epub ahead of print]

PMID: 32336315

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Select item 32339400

 

  1. Incidence and fate of device-related left pulmonary artery stenosis and aortic coarctation in small infants undergoing transcatheter patent ductus arteriosus closure.

Tomasulo CE, Gillespie MJ, Munson D, Demkin T, O’Byrne ML, Dori Y, Smith CL, Rome JJ, Glatz AC.

Catheter Cardiovasc Interv. 2020 Apr 27. doi: 10.1002/ccd.28942. [Epub ahead of print]

PMID: 32339400

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Select item 32338402

 

  1. A low threshold for neonatal intervention yields a high rate of biventricular outcomes in pulmonary atresia with intact ventricular septum.

Morgan GJ, Narayan SA, Goreczny S, Chubb H, Krasemann T, Rosenthal E, Qureshi SA.

Cardiol Young. 2020 Apr 23:1-7. doi: 10.1017/S1047951120000700. [Epub ahead of print]

PMID: 32321616

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Select item 32323311

 

  1. Percutaneous closure of a left ventricular pseudoaneurysm after transcatheter ventricular septal defect closure.

Gokalp S, Ugan Atik S, Saltik IL.

Cardiol Young. 2020 Apr 20:1-3. doi: 10.1017/S1047951120000815. [Epub ahead of print]

PMID: 32308169

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Select item 32325371

 

  1. Treatment of severe pulmonary insufficiency with bilateral branch pulmonary artery Melody valve implantation.

Abu-Anza O, Nakamura Y, Aldoss O.

Cardiol Young. 2020 Apr 17:1-3. doi: 10.1017/S1047951120000827. [Epub ahead of print]

PMID: 32301405

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Select item 32202295

 

  1. 3D advanced imaging overlay with rapid registration in CHD to reduce radiation and assist cardiac catheterisation interventions.

Arar Y, Reddy SRV, Kim H, Dimas VV, Zellers TM, Abou Zahr R, Vamsee R, Greer JS, Tandon A, Pontiki A, Dillenbeck J, Zabala L, Greil G, Nugent AW, Hussain T.

Cardiol Young. 2020 Apr 15:1-7. doi: 10.1017/S1047951120000712. [Epub ahead of print]

PMID: 32290877

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Select item 32294315

 

  1. Velocity Vector Imaging Assessment of Functional Change in the Right Ventricle during Transcatheter Closure of Atrial Septal Defect by Intracardiac Echocardiography.

Jung SY, Shin JI, Choi JY, Park SJ, Kim NK.

J Clin Med. 2020 Apr 15;9(4). pii: E1132. doi: 10.3390/jcm9041132.

PMID: 32326588 Free Article

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Select item 32326432

 

  1. Interventional atrial septal defect closure: histology of long-term performance.

Sigler M, Hofbeck M.

Eur Heart J. 2020 Apr 14;41(15):1478. doi: 10.1093/eurheartj/ehz338. No abstract available.

PMID: 31132082

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Select item 32291873

 

  1. Considerations for Triaging Elective Cases in Children with Cardiac Disease in a Time of Crisis.

Gal DB, Char DS.

Circulation. 2020 Apr 13. doi: 10.1161/CIRCULATIONAHA.120.047087. [Epub ahead of print] No abstract available.

PMID: 32282242

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Select item 32285188

 

  1. Device Closure of Iatrogenic Left Ventricular Perforation Through the Chest Wall.

McGill M, Aggarwal V, Hiremath G.

JACC Cardiovasc Interv. 2020 Apr 13;13(7):897-898. doi: 10.1016/j.jcin.2019.10.011. Epub 2019 Dec 25. No abstract available.

PMID: 31883718

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Select item 32282016

 

  1. Late cardiac erosion after percutaneous ventricular septal defect closure: a complication after ventricular septal defect device implantation.

Schmiady M, Kretschmar O, Hübler M, Sigler M.

Eur Heart J. 2020 Apr 7;41(14):1410. doi: 10.1093/eurheartj/ehz617. No abstract available.

PMID: 32259267

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Select item 32281525

 

  1. Interventional cardiac catheterization in neonatal age: results in a multicentre Italian experience.

Giordano M, Santoro G, Agnoletti G, Carminati M, Donti A, Guccione P, Marasini M, Milanesi O, Russo MG, Castaldi B, Cheli M, Formigari R, Gaio G, Giugno L, Lunardini A, Pepino C, Spadoni I.

Int J Cardiol. 2020 Apr 5. pii: S0167-5273(20)30384-3. doi: 10.1016/j.ijcard.2020.04.013. [Epub ahead of print]

PMID: 32303417

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Select item 32309207

 

  1. Perforating the GORE® CARDIOFORM septal occluder and atrial septal defect occluder to gain access to the left atrium.

Anderson JH, Adamson T, Migliati E, Herlihy J, Danieu P, Daly J, Allen C, Hua K, Inglessis I.

Catheter Cardiovasc Interv. 2020 Apr 2. doi: 10.1002/ccd.28884. [Epub ahead of print]

PMID: 32239801

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Select item 32249125

 

  1. The Natural and Unnatural History of Ventricular Septal Defects Presenting in Infancy: An Echocardiography-Based Review.

Cox K, Algaze-Yojay C, Punn R, Silverman N.

J Am Soc Echocardiogr. 2020 Apr 2. pii: S0894-7317(20)30035-3. doi: 10.1016/j.echo.2020.01.013. [Epub ahead of print]

PMID: 32249125 Free Article

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Select item 32234207

 

  1. Short- and intermediate-term results of balloon aortic valvuloplasty and surgical aortic valvotomy in neonates.

Zaban NB, Herrmann JL, Hoyer MH, Brown JW, Alexy RD.

Cardiol Young. 2020 Apr;30(4):489-492. doi: 10.1017/S1047951120000372. Epub 2020 Feb 24.

PMID: 32090726

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Select item 32063255

 

  1. Transcatheter mitral valve-in-valve implantation in a pediatric patient.

Momenah TS, Alsahari A, Ahmed E, Al Khalaf K.

Catheter Cardiovasc Interv. 2020 Apr 1;95(5):1062-1065. doi: 10.1002/ccd.28390. Epub 2019 Jul 11.

PMID: 31293050

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Select item 32202126

 

  1. Decisions, Decisions, Decisions…: A Novel Approach Helps Evaluate Potential Benefit of Fetal Aortic Valvuloplasty.

O’Byrne ML, Peyvandi S.

Circ Cardiovasc Qual Outcomes. 2020 Apr;13(4):e006636. doi: 10.1161/CIRCOUTCOMES.120.006636. Epub 2020 Apr 7. No abstract available.

PMID: 32252550

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Select item 32252549

 

  1. Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart Syndrome: A Decision Analysis.

Pickard SS, Wong JB, Bucholz EM, Newburger JW, Tworetzky W, Lafranchi T, Benson CB, Wilkins-Haug LE, Porras D, Callahan R, Friedman KG.

Circ Cardiovasc Qual Outcomes. 2020 Apr;13(4):e006127. doi: 10.1161/CIRCOUTCOMES.119.006127. Epub 2020 Apr 7.

PMID: 32252549

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Select item 32167373

 

  1. 33. Takayasu Arteritis With Coronary Artery Involvement: Differences Between Pediatric and Adult Patients.

Lei C, Huang Y, Yuan S, Chen W, Liu H, Yang M, Shen Z, Fang L, Fang Q, Song H, Tian X, Zeng X, Guo X, Zhang S.

Can J Cardiol. 2020 Apr;36(4):535-542. doi: 10.1016/j.cjca.2019.08.039. Epub 2019 Sep 6.

PMID: 31924450

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Select item 32256914

 

  1. Infective endocarditis developing early after percutaneous closure of a patent ductus arteriosus in a child using the Amplatzer Duct Occluder II.

Yilmazer MM, Meşe T.

Cardiol Young. 2020 Apr;30(4):591-593. doi: 10.1017/S1047951120000542. Epub 2020 Mar 19.

PMID: 32188522

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Congenital Heart Surgery

Congenital Heart Surgery Reviews of MApril 2020 Manuscripts

 

  1. CHD Surgery April 2020

     

    1. Minimally invasive periareolar approach to repair sinus venosus atrial septal defect with partial anomalous pulmonary venous connection.

    Bozso SJ, Evans B, Chu MWA.

    J Card Surg. 2020 Apr 30. doi: 10.1111/jocs.14591. [Epub ahead of print]

    PMID: 32353904

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    Select item 32354361

     

    1. Effects of sugammadex on postoperative respiratory management in children with congenital heart disease: a randomized controlled study.

    Xiaobing L, Yan J, Wangping Z, Rufang Z, Jia L, Rong W.

    Biomed Pharmacother. 2020 Apr 27;127:110180. doi: 10.1016/j.biopha.2020.110180. [Epub ahead of print]

    PMID: 32353822 Free Article

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    Select item 32339400

     

    1. Lung protective ventilation in infants undergoing cardiopulmonary bypass surgery for congenital heart disease: a prospective randomized controlled trial.

    Sun Y, Shen SE, Deng XM, Cai Y, Du Y.

    Paediatr Anaesth. 2020 Apr 27. doi: 10.1111/pan.13894. [Epub ahead of print]

    PMID: 32338441

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    Select item 32342149

     

    1. Continuous postoperative pericardial flushing method versus standard care for wound drainage after adult cardiac surgery: A randomized controlled trial.

    Diephuis E, de Borgie C, Tomšič A, Winkelman J, van Boven WJ, Bouma B, Eberl S, Juffermans N, Schultz M, Henriques JP, Koolbergen D.

    EBioMedicine. 2020 Apr 25;55:102744. doi: 10.1016/j.ebiom.2020.102744. [Epub ahead of print]

    PMID: 32344201 Free PMC Article

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    Select item 32335820

     

    1. A successful heart and liver transplantation requiring intraoperative extracorporeal membrane oxygenation for primary cardiac allograft dysfunction in a patient with Fontan failure.

    Siddiqi UA, Hibino N, Combs PS, Baker T, Song T, Kim G, Jeevanandam V.

    J Card Surg. 2020 Apr 25. doi: 10.1111/jocs.14561. [Epub ahead of print]

    PMID: 32333435

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    Select item 32333429

     

    1. A hybrid heart for heart failure – how much remains to be done? An interview with Jolanda Kluin.

    Kluin J.

    Future Cardiol. 2020 Apr 23. doi: 10.2217/fca-2020-0041. [Epub ahead of print]

    PMID: 32323578 Free Article

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    Select item 32328807

     

    1. COVID-19 FAQ’s in Pediatric Cardiac Surgery.

    Levy E, Blumenthal J, Chiotos K, Dearani JA.

    World J Pediatr Congenit Heart Surg. 2020 Apr 21:2150135120924653. doi: 10.1177/2150135120924653. [Epub ahead of print] No abstract available.

    PMID: 32316830

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    Select item 32382545

     

    1. Computational Evaluation of Surgical Design for Multisegmental Complex Congenital Tracheal Stenosis.

    Zhu L, Gong X, Liu J, Li Y, Zhong Y, Shen J, Xu Z.

    Biomed Res Int. 2020 Apr 20;2020:3509814. doi: 10.1155/2020/3509814. eCollection 2020.

    PMID: 32382545 Free Article

     

    Select item 32314508

     

    1. Incidental diagnosis of a large aortopulmonary window with reversible pulmonary arterial hypertension in adult age and its surgical management.

    Khanna S, Mahajan S, Halder V, Gowda N.

    J Card Surg. 2020 Apr 19. doi: 10.1111/jocs.14569. [Epub ahead of print]

    PMID: 32306468

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    Select item 32315647

     

    1. Persistent Fifth Aortic Arch with Left Ventricular Dysfunction and Left Bronchial Obstruction.

    Kato N, Tachibana T, Asai H, Ebuoka N, Shingu Y, Ooka T, Kato H, Wakasa S.

    Ann Thorac Surg. 2020 Apr 18. pii: S0003-4975(20)30562-2. doi: 10.1016/j.athoracsur.2020.03.039. [Epub ahead of print]

    PMID: 32315647

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    Select item 32315641

     

    1. Surgical management of congenital bilateral multiple atrial aneurysms.

    Widenka H, Stoller F, Glöckler M, Erdös G, Carrel T, Kadner A.

    Ann Thorac Surg. 2020 Apr 18. pii: S0003-4975(20)30571-3. doi: 10.1016/j.athoracsur.2020.03.045. [Epub ahead of print]

    PMID: 32315641

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    Select item 32305952

     

    1. Pig-to-human heart transplantation: who goes first?

    Pierson RN 3rd, Burdorf L, Madsen JC, Lewis GD, D’Alessandro DA.

    Am J Transplant. 2020 Apr 17. doi: 10.1111/ajt.15916. [Epub ahead of print]

    PMID: 32301262

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    Select item 32305978

     

    1. Imaging features of complete congenital atresia of left coronary artery.

    Raimondi F, Secinaro A, Boddaert N, Bonnet D.

    Diagn Interv Imaging. 2020 Apr 17. pii: S2211-5684(20)30056-5. doi: 10.1016/j.diii.2020.02.009. [Epub ahead of print] No abstract available.

    PMID: 32312564

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    Select item 32303878

     

    1. “Dual pentalogy in a child”: A rare anatomy deciphered on CT.

    S H C, Verma M, Ojha V, Sharma A, Kumar S.

    J Card Surg. 2020 Apr 17. doi: 10.1111/jocs.14540. [Epub ahead of print] No abstract available.

    PMID: 32302029

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    Select item 32299151

     

    1. Corrective surgery alters plasma protein profiling in congenital heart diseases and clinical perspectives.

    He GW, Hou HT, Xuan C, Wang J, Liu LX, Zhang JF, Liu XC, Yang Q.

    Am J Transl Res. 2020 Apr 15;12(4):1319-1337. eCollection 2020.

    PMID: 32355544 Free PMC Article

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    Select item 32293321

     

    1. Ventriculoatrial malalignment in atrioventricular septal defect resulting in uniatrial biventricular connection: surgical options.

    Hofbeck M, Wiegand G, Michel J, Schlensak C.

    J Cardiothorac Surg. 2020 Apr 15;15(1):59. doi: 10.1186/s13019-020-01099-x.

    PMID: 32295626 Free PMC Article

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    Select item 32326588

     

    1. Fontan completion during winter season is not associated with higher mortality or morbidity in the early post-operative period.

    Nordmeyer S, Krettek S, Nordmeyer J, Schafstedde M, Rehm K, Photiadis J, Berger F, Ovroutski S.

    Cardiol Young. 2020 Apr 13:1-4. doi: 10.1017/S1047951120000670. [Epub ahead of print]

    PMID: 32279698

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    Select item 32279695

     

    1. Cold-inducible RNA binding protein agonist enhances the cardioprotective effect of UW solution during extended heart preservation.

    Pan HZ, Zhang LJ, Liu YW, Li YN, Su ZH, Meng J, Zhang H.

    Artif Organs. 2020 Apr 12. doi: 10.1111/aor.13695. [Epub ahead of print]

    PMID: 32279354

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    Select item 32290605

     

    1. Primary carnitine deficiency – diagnosis after heart transplantation: better late than never!

    Grünert SC, Tucci S, Schumann A, Schwendt M, Gramer G, Hoffmann GF, Erbel M, Stiller B, Spiekerkoetter U.

    Orphanet J Rare Dis. 2020 Apr 10;15(1):87. doi: 10.1186/s13023-020-01371-2.

    PMID: 32276632 Free PMC Article

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    Select item 32272495

     

    1. Proceedings From the 2019 Stanford Single Ventricle Scientific Summit: Advancing Science for Single Ventricle Patients: From Discovery to Clinical Applications.

    Reddy S, Handler SS, Wu S, Rabinovitch M, Wright G.

    J Am Heart Assoc. 2020 Apr 7;9(7):e015871. doi: 10.1161/JAHA.119.015871. Epub 2020 Mar 19.

    PMID: 32188306 Free Article

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    Select item 32266476

     

    1. Partial anomalous pulmonary venous drainage in patients presenting with suspected pulmonary hypertension: A series of 90 patients from the ASPIRE registry.

    Lewis RA, Billings CG, Bolger A, Bowater S, Charalampopoulos A, Clift P, Elliot CA, English K, Hamilton N, Hill C, Hurdman J, Jenkins PJ, Johns C, MacDonald S, Oliver J, Papaioannou V, Rajaram S, Sabroe I, Swift AJ, Thompson AAR, Kiely DG, Condliffe R.

    Respirology. 2020 Apr 6. doi: 10.1111/resp.13815. [Epub ahead of print]

    PMID: 32249494 Free Article

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    Select item 32249445

     

    1. Chylothorax after Surgery for Congenital Cardiac Disease: A Prevention and Management Protocol.

    Shin YR, Lee H, Park YH, Park HK.

    Korean J Thorac Cardiovasc Surg. 2020 Apr 5;53(2):41-48. doi: 10.5090/kjtcs.2020.53.2.41.

    PMID: 32309201 Free PMC Article

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    Select item 32275884

     

    1. A nurse-driven analgesia and sedation protocol reduces length of PICU stay and cumulative dose of benzodiazepines after corrective surgery for tetralogy of Fallot.

    Hanser A, Neunhoeffer F, Hayer T, Hofbeck M, Schlensak C, Mustafi M, Kumpf M, Michel J.

    J Spec Pediatr Nurs. 2020 Apr 3:e12291. doi: 10.1111/jspn.12291. [Epub ahead of print]

    PMID: 32243076

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    Select item 32348933

     

    1. An intriguing case of post-operative respiratory failure from an occult diaphragmatic hernia- be aware of the masquerader.

    Borthakur B, Hanjoora VM, Mahajan V, Srivastava AR, Jyoti A, Raut M, Nawal A.

    Ann Card Anaesth. 2020 Apr-Jun;23(2):237-240. doi: 10.4103/aca.ACA_227_18.

    PMID: 32275047 Free Article

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    Select item 32246937

     

    1. ST2 predicts risk of unplanned readmission within one year after pediatric congenital heart surgery.

    Parker DM, Everett AD, Stabler ME, Jacobs ML, Jacobs JP, Vricella L, Thiessen-Philbrook H, Parikh CR, Manlhiot C, Brown JR.

    Ann Thorac Surg. 2020 Apr 1. pii: S0003-4975(20)30431-8. doi: 10.1016/j.athoracsur.2020.02.056. [Epub ahead of print]

    PMID: 32246937

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    1. Anti-Pig Antibody in Infants: Can a Genetically Engineered Pig Heart Bridge to Allotransplantation?

    Li Q, Hara H, Banks CA, Yamamoto T, Ayares D, Mauchley DC, Dabal RJ, Padilla L, Carlo WF, Rhodes LA, Cooper DKC, Cleveland DC.

    Ann Thorac Surg. 2020 Apr;109(4):1268-1273. doi: 10.1016/j.athoracsur.2019.08.061. Epub 2019 Sep 30.

    PMID: 31580857

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    Select item 32199055

     

    1. Impact of Vacuum-Assisted Venous Drainage on Forward Flow in Simulated Pediatric Cardiopulmonary Bypass Circuits Utilizing a Centrifugal Arterial Pump Head.

    Guimarães DP, Caneo LF, Matte G, Carletto LP, Policarpo VC, Castro AVCX, Miranda MHC, Costa PS, Jatene MB, Cestari I, Jatene FB.

    Braz J Cardiovasc Surg. 2020 Apr 1;35(2):134-140. doi: 10.21470/1678-9741-2019-0311.

    PMID: 32369291 Free PMC Article

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    Select item 32144038

     

    1. Pre-operative neutrophil-lymphocyte ratio predicts low cardiac output in children after cardiac surgery.

    Iliopoulos I, Alder MN, Cooper DS, Villarreal EG, Loomba R, Sahay RD, Fei L, Steele PE, Flores S.

    Cardiol Young. 2020 Apr;30(4):521-525. doi: 10.1017/S1047951120000487. Epub 2020 Mar 5.

    PMID: 32131918

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    Select item 31912986

     

    1. Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience.

    Liu X, Yuan H, Chen J, Cen J, Nie Z, Xu G, Wen S, Zhuang J.

    Eur J Cardiothorac Surg. 2020 Apr 1;57(4):628-634. doi: 10.1093/ejcts/ezz310.

    PMID: 31740945

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    Select item 32114792

     

    1. Serum vitamin D status following pediatric cardiac surgery and association with clinical outcome.

    Dohain AM, Almogati J, Al-Radi OO, Elassal AA, Zaher ZF, Fatani TH, Abdulgawad A, Abdelmohsen G.

    Eur J Pediatr. 2020 Apr;179(4):635-643. doi: 10.1007/s00431-019-03538-x. Epub 2019 Dec 21.

    PMID: 31865429

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    Select item 32236547

     

    1. Neck cannulation for bypass in redo sternotomy in children and adults with congenital heart disease.

    Mustafa MR, Neijenhuis RML, Furci B, Tsang VT.

    Interact Cardiovasc Thorac Surg. 2020 Apr 1. pii: ivaa045. doi: 10.1093/icvts/ivaa045. [Epub ahead of print]

    PMID: 32236557

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    Select item 31821450

     

    1. Choice of shunt type for the Norwood I procedure: does it make a difference?

    Vitanova K, Georgiev S, Lange R, Cleuziou J.

    Interact Cardiovasc Thorac Surg. 2020 Apr 1;30(4):630-635. doi: 10.1093/icvts/ivz294.

    PMID: 31821450

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    Select item 32270622

     

    1. Ross operation 23 years after surgery: It should not be a “forgotten” option.

    Nappi F, Avtaar Singh SS, Spadaccio C, Acar C.

    J Card Surg. 2020 Apr;35(4):952-956. doi: 10.1111/jocs.14489. Epub 2020 Mar 1.

    PMID: 32115768

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    Select item 32115762

     

    1. Is two-staged repair for truncus arteriosus type A3 unpractical?

    Yokoyama S, Fukuba R, Mitani K, Tonomura R, Uemura H.

    J Card Surg. 2020 Apr;35(4):957-960. doi: 10.1111/jocs.14488. Epub 2020 Mar 1.

    PMID: 32115762

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    Select item 32092198

     

    1. Risk Factors for Prolonged Mechanical Ventilation After Total Cavopulmonary Connection Surgery: 8 Years of Experience at Fuwai Hospital.

    Luo Q, Su Z, Jia Y, Liu Y, Wang H, Zhang L, Li Y, Wu X, Liu Q, Yan F.

    J Cardiothorac Vasc Anesth. 2020 Apr;34(4):940-948. doi: 10.1053/j.jvca.2019.10.043. Epub 2019 Nov 2.

    PMID: 31983510

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    Select item 31899144

     

    1. Retrosternal Clots After Fontan Surgery by Systematic Evaluation With Transthoracic Ultrasound.

    Cantinotti M, Giordano R, Marchese P, Franchi E, Viacava C, Pak V, Murzi B, Arcieri L, Poli V, Federici D, Koestenberger M, Assanta N.

    J Cardiothorac Vasc Anesth. 2020 Apr;34(4):951-955. doi: 10.1053/j.jvca.2019.11.009. Epub 2019 Nov 18.

    PMID: 31812566

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    Select item 31543295

     

    1. Single-Shot Cold Histidine-Tryptophan-Ketoglutarate Cardioplegia for Long Aortic Cross-Clamping Durations in Neonates.

    Dolcino A, Gaudin R, Pontailler M, Raisky O, Vouhé P, Bojan M.

    J Cardiothorac Vasc Anesth. 2020 Apr;34(4):959-965. doi: 10.1053/j.jvca.2019.08.039. Epub 2019 Aug 27.

    PMID: 31543295

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    Select item 31515190

     

    1. Efficacy of Bilateral Erector Spinae Plane Block in Management of Acute Postoperative Surgical Pain After Pediatric Cardiac Surgeries Through a Midline Sternotomy.

    Kaushal B, Chauhan S, Magoon R, Krishna NS, Saini K, Bhoi D, Bisoi AK.

    J Cardiothorac Vasc Anesth. 2020 Apr;34(4):981-986. doi: 10.1053/j.jvca.2019.08.009. Epub 2019 Aug 12.

    PMID: 31515190

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    Select item 31732917

     

    1. Preoperative miRNA-208a as a Predictor of Postoperative Complications in Children with Congenital Heart Disease Undergoing Heart Surgery.

    Zloto K, Tirosh-Wagner T, Bolkier Y, Bar-Yosef O, Vardi A, Mishali D, Paret G, Nevo-Caspi Y.

    J Cardiovasc Transl Res. 2020 Apr;13(2):245-252. doi: 10.1007/s12265-019-09921-1. Epub 2019 Nov 15.

    PMID: 31732917

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    Select item 31867804

     

    1. ISHLT consensus statement on donor organ acceptability and management in pediatric heart transplantation.

    Kirk R, Dipchand AI, Davies RR, Miera O, Chapman G, Conway J, Denfield S, Gossett JG, Johnson J, McCulloch M, Schweiger M, Zimpfer D, Ablonczy L, Adachi I, Albert D, Alexander P, Amdani S, Amodeo A, Azeka E, Ballweg J, Beasley G, Böhmer J, Butler A, Camino M, Castro J, Chen S, Chrisant M, Christen U, Danziger-Isakov L, Das B, Everitt M, Feingold B, Fenton M, Garcia-Guereta L, Godown J, Gupta D, Irving C, Joong A, Kemna M, Khulbey SK, Kindel S, Knecht K, Lal AK, Lin K, Lord K, Möller T, Nandi D, Niesse O, Peng DM, Pérez-Blanco A, Punnoose A, Reinhardt Z, Rosenthal D, Scales A, Scheel J, Shih R, Smith J, Smits J, Thul J, Weintraub R, Zangwill S, Zuckerman WA.

    J Heart Lung Transplant. 2020 Apr;39(4):331-341. doi: 10.1016/j.healun.2020.01.1345. Epub 2020 Jan 31.

    PMID: 32088108

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    1. Safety, Efficacy, and Timeliness of Intravenous Potassium Chloride Replacement Protocols in a Pediatric Cardiothoracic Intensive Care Unit.

    Amirnovin R, Lieu P, Imperial-Perez F, Taketomo C, Markovitz BP, Moromisato DY.

    J Intensive Care Med. 2020 Apr;35(4):371-377. doi: 10.1177/0885066617752659. Epub 2018 Jan 22.

    PMID: 29357785

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    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. 2020 Apr;159(4):1532-1540.e7. doi: 10.1016/j.jtcvs.2019.03.062. Epub 2019 Apr 4.

    PMID: 31043318

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    1. Extracorporeal Membrane Oxygenation Support After Heart Transplantation in Children-Outcomes of a Single Center Cohort.

    Nair AG, Sleeper LA, Smoot LB, Wigmore D, Mecklosky J, Andren K, Bastardi HJ, Blume ED, Fynn-Thompson F, Thiagarajan RR, Alexander PMA.

    Pediatr Crit Care Med. 2020 Apr;21(4):332-339. doi: 10.1097/PCC.0000000000002192.

    PMID: 31658187

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    1. Thyroid Hormone (Triiodothyronine) Therapy in Children After Congenital Heart Surgery: A Meta-Analysis.

    Flores S, Loomba RS, Checchia PA, Graham EM, Bronicki RA.

    Semin Thorac Cardiovasc Surg. 2020 Spring;32(1):87-95. doi: 10.1053/j.semtcvs.2019.05.020. Epub 2019 May 22.

    PMID: 31128253

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    1. Programmatic Responses to the Coronavirus Pandemic: A Survey of 502 Cardiac Surgeons.

    Seese L, Aranda-Michel E, Sultan I, Morell VO, Mathier MA, Mulukutla SR, Saba S, Dueweke EJ, Levenson JE, Kilic A.

    Ann Thorac Surg. 2020 Apr 28. pii: S0003-4975(20)30617-2. doi: 10.1016/j.athoracsur.2020.04.014. [Epub ahead of print] No abstract available.

    PMID: 32360389 Free PMC Article

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    1. Outcome of arterial switch operation for transposition of the great arteries. A 35-year follow-up study.

    Santens B, Van De Bruaene A, De Meester P, Gewillig M, Troost E, Claus P, Bogaert J, Budts W.

    Int J Cardiol. 2020 Apr 26. pii: S0167-5273(19)36246-1. doi: 10.1016/j.ijcard.2020.04.072. [Epub ahead of print]

    PMID: 32348813

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    1. Rebuttal to Letter to Editor: Determining Novel Urinary Biomarkers for Acute Kidney Injury and Prediction of Clinical Outcomes After Pediatric Cardiac Surgery.

    Yoneyama F, Maruo K.

    Pediatr Cardiol. 2020 Apr 26. doi: 10.1007/s00246-020-02350-x. [Epub ahead of print] No abstract available.

    PMID: 32337624

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    1. Impact of Confounding on Cost, Survival, and Length-of-Stay Outcomes for Neonates with Hypoplastic Left Heart Syndrome Undergoing Stage 1 Palliation Surgery.

    Gong CL, Song AY, Horak R, Friedlich PS, Lakshmanan A, Pruetz JD, Yieh L, Ram Kumar S, Williams RG.

    Pediatr Cardiol. 2020 Apr 26. doi: 10.1007/s00246-020-02348-5. [Epub ahead of print]

    PMID: 32337623

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    1. Early Outcomes for In-situ Pericardial Roll Repair for Distant Anomalous Pulmonary Venous Return.

    Najm HK, Ahmad M, Salam Y, Klein J, Hasan SM, Majdalany D, Stewart RD, Pettersson G, Karamlou T.

    Ann Thorac Surg. 2020 Apr 24. pii: S0003-4975(20)30596-8. doi: 10.1016/j.athoracsur.2020.03.063. [Epub ahead of print]

    PMID: 32339505

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    1. Determining Novel Urinary Biomarkers for Acute Kidney Injury and Prediction of Clinical Outcomes After Pediatric Cardiac Surgery.

    Liu FK, Xue FS.

    Pediatr Cardiol. 2020 Apr 24. doi: 10.1007/s00246-020-02349-4. [Epub ahead of print] No abstract available.

    PMID: 32333036

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    1. Comparison of cardiac output and cardiac index values measured by critical care echocardiography with the values measured by pulse index continuous cardiac output (PiCCO) in the pediatric intensive care unit:a preliminary study.

    Aslan N, Yildizdas D, Horoz OO, Coban Y, Demir F, Erdem S, Sertdemir Y.

    Ital J Pediatr. 2020 Apr 16;46(1):47. doi: 10.1186/s13052-020-0803-y.

    PMID: 32299455 Free PMC Article

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    1. Anesthesia for bilateral pulmonary banding as part of hybrid stage I approach palliating neonates with hypoplastic left heart syndrome.

    Zajonz T, Cupka P, Koerner C, Mann V, Menges T, Akintuerk H, Valeske K, Thul J, Schranz D, Mueller M.

    Paediatr Anaesth. 2020 Apr 14. doi: 10.1111/pan.13876. [Epub ahead of print]

    PMID: 32291873

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    1. Evolution of Pediatric Ventricular Assist Devices and their Neurologic and Renal Complications – a 24-year single center experience.

    Mantell B, Addonizio L, Jain N, LaPar D, Chai P, Bacha E, Kalfa D, McAllister J, Zuckerman W, Lee T, Richmond M, Law S.

    Artif Organs. 2020 Apr 12. doi: 10.1111/aor.13696. [Epub ahead of print]

    PMID: 32279327

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    1. The First Pediatric Heart Transplantation Bridged by a Durable Left Ventricular Assist Device in Korea.

    Shin JH, Park HK, Jung SY, Kim AY, Jung JW, Shin YR.

    Korean J Thorac Cardiovasc Surg. 2020 Apr 5;53(2):79-81. doi: 10.5090/kjtcs.2020.53.2.79.

    PMID: 32309207 Free PMC Article

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    1. Significance of Aortopulmonary Collaterals in a Single-Ventricle Patient Supported With a HeartMate 3.

    Smith M, El-Said H, Pretorius V, Mendenhall M, Thomas T, Reeves RR, Silva Enciso J, Alshawabkeh L, Nigro J, Adler ED, Urey MA.

    Circ Heart Fail. 2020 Apr;13(4):e006473. doi: 10.1161/CIRCHEARTFAILURE.119.006473. Epub 2020 Apr 6. No abstract available.

    PMID: 32248696

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    1. Long-term follow-up after transatrial-transpulmonary repair of tetralogy of Fallot: influence of timing on outcome.

    van den Bosch E, Bogers AJJC, Roos-Hesselink JW, van Dijk APJ, van Wijngaarden MHEJ, Boersma E, Nijveld A, Luijten LWG, Tanke R, Koopman LP, Helbing WA.

    Eur J Cardiothorac Surg. 2020 Apr 1;57(4):635-643. doi: 10.1093/ejcts/ezz331.

    PMID: 31872208 Free PMC Article

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    1. Follow-up after biventricular repair of the hypoplastic left heart complex.

    IJsselhof RJ, Duchateau SDR, Schouten RM, Freund MW, Heuser J, Fejzic Z, Haas F, Schoof PH, Slieker MG.

    Eur J Cardiothorac Surg. 2020 Apr 1;57(4):644-651. doi: 10.1093/ejcts/ezz293.

    PMID: 31651943

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    1. Long-term results of aortic arch reconstruction with branch pulmonary artery homograft patches.

    Lewis MJ, Johansson Ramgren J, Hallbergson A, Liuba P, Sjöberg G, Malm T.

    J Card Surg. 2020 Apr;35(4):868-874. doi: 10.1111/jocs.14494. Epub 2020 Mar 11.

    PMID: 32160354

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    1. Three-dimensional printing for surgical planning of a double aortic arch case.

    Arcieri L, Provost B, Charbonneau P, Fournier E, Hascoet S, Le Bret E.

    J Card Surg. 2020 Apr;35(4):912-915. doi: 10.1111/jocs.14479. Epub 2020 Feb 24.

    PMID: 32092176

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    1. Fontan procedure: Early outcomes of 87 consecutive patients in a tertiary care center.

    Kilcoyne MF, Stevens RM, Mahan V, Gray P, Moulick AN.

    J Card Surg. 2020 Apr;35(4):738-739. doi: 10.1111/jocs.14462. Epub 2020 Feb 19.

    PMID: 32073683

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    1. Cardiac Collapse Secondary to Phenytoin Toxicity in a Neonate Treated with Extracorporeal Membrane Oxygenation Support (ECMO).

    Knecht M, LaRochelle J, Barkemeyer B, Gupta R, Brumund M, Mumphrey C.

    J Med Toxicol. 2020 Apr;16(2):230-235. doi: 10.1007/s13181-019-00742-x. Epub 2019 Nov 26.

    PMID: 31773636

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    1. Pulmonary artery banding in complete atrioventricular septal defect.

    Devlin PJ, Jegatheeswaran A, McCrindle BW, Karamlou T, Blackstone EH, Williams WG, DeCampli WM, Mertens L, Fackoury CT, Eghtesady P, Jacobs JP, Baffa JM, Fleishman CE, Dodge-Khatami A, Pizarro C, Pourmoghadam K, Cohen MS, Meyer DB, Overman DM.

    J Thorac Cardiovasc Surg. 2020 Apr;159(4):1493-1503.e3. doi: 10.1016/j.jtcvs.2019.09.019. Epub 2019 Sep 24.

    PMID: 31669019

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    1. Extracorporeal Membrane Oxygenation Support After Heart Transplantation in Children-Outcomes of a Single Center Cohort.

    Nair AG, Sleeper LA, Smoot LB, Wigmore D, Mecklosky J, Andren K, Bastardi HJ, Blume ED, Fynn-Thompson F, Thiagarajan RR, Alexander PMA.

    Pediatr Crit Care Med. 2020 Apr;21(4):332-339. doi: 10.1097/PCC.0000000000002192.

    PMID: 31658187

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    1. Regionalization of Congenital Heart Surgery in the United States.

    Welke KF, Pasquali SK, Lin P, Backer CL, Overman DM, Romano JC, Karamlou T.

    Semin Thorac Cardiovasc Surg. 2020 Spring;32(1):128-137. doi: 10.1053/j.semtcvs.2019.09.005. Epub 2019 Sep 10.

    PMID: 31518703

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    1. Hemitruncus Arteriosus in a 10-Day-Old Neonate with Patent Ductus Arteriosus and Thrombocytopenia.

    Zolfi-Gol A, Radvar M, Sedokani A.

    Vasc Health Risk Manag. 2020 Apr 1;16:99-101. doi: 10.2147/VHRM.S245033. eCollection 2020.

    PMID: 32280232 Free PMC Article

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Fetal Cardiology Featured Articles

Fetal Cardiology Reviews of April 2020 Manuscripts

 

Telediagnosis system for congenital heart disease in a Japanese prefecture.

Mabuchi A, Waratani M, Tanaka Y, Mori T, Kitawaki J.J Med Ultrason (2001). 2020 Apr 23. doi: 10.1007/s10396-020-01020-y. Online ahead of print.PMID: 32328807

 

Take Home Points 

  • Fetal diagnosis via telehealth using 3D images transferred via an optical fiber network system is feasible and reliable.
  • In 161 cases screened, cardiac defects were noted in 24 fetal scans. Though there were some discrepancies in prenatal cardiac diagnosis versus post-natal diagnosis, no significant cardiac abnormalities were missed.
  • Appropriate post-natal planning was performed using the telehealth fetal images with significant cardiac defects birthed at a tertiary pediatric hospital whereas minor cardiac defects were birthed at the home hospital.

 

Commentary from Dr. Clifford Cua (Columbus, OH), Section Editor of Pediatric Cardiology Journal Watch:   In this retrospective study from a single institution, Kyoto Prefectural University of Medicine, a spatio-temporal image correlation (STIC) based tele-diagnosis system was created for remote fetal diagnostic purposes.  STIC is a 3D technique of acquiring structural and temporal data using a single scan.  Data are digitized and cross-sectional images can be reconstructed to be reviewed at a later date.  This system was created to help screen fetal cardiac exams from distant locations from the main pediatric cardiac hospital to maximize care and minimize unnecessary travel for the families.

A total of 182 fetal scans over a nine-year period (2009 – 2018) were referred for evaluation from six hospitals within the Kyoto Prefecture.  21 cases were excluded (10 – error in transmission, 11 – no postnatal diagnosis available), therefore 161 cases were studied.  Images were obtained via a Voluson E7, E8, or E10 (GE Medical Systems) and transferred images were reviewed by a pediatric cardiologist within 24 to 72 hours from transmission.  Images were evaluated using a standard protocol to assess the cardiac anatomy.

Cardiac disease was noted 14.9% of cases (24/161) and accuracy of diagnosis was 95.0% (153/161).  In four cases, the cardiac diagnosis changed from prenatal to post-natal: (1) double outlet right ventricle (DORV) to ventricular septal defect (VSD); (2) DORV and pulmonary artery stenosis (PS) to single ventricular and tricuspid atresia; (3) transposition of the great arteries (TGA) to DORV and PS; and (4) total anomalous pulmonary venous connection to normal heart after the visit.  In another four cases, the prenatal diagnosis was thought to be normal, but postnatally, two patients had a VSD, one had an ASD, and one patient an aneurysm of the PDA.  Seven cases suspected of having severe cardiac issues were delivered at the tertiary pediatric hospital whereas the other patients birthed at their respective home hospitals experienced no adverse outcomes.

This study is limited by its retrospective nature, single center evaluation, and relatively small cases evaluated.  That being said, this paper adds more evidence that tele-health for fetal cardiac screening purposes is feasible and allows for efficiency of medical care for both the family and health professional.

 

 

 

 

  1. Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

Ronai C, Freud LR, Brown DW, Tworetzky W.

Prenat Diagn. 2020 Apr 20. doi: 10.1002/pd.5715. [Epub ahead of print]

PMID: 32314369

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Select item 32354651]

 

Take Home Points

 

  • Despite routine prenatal cardiac screening ultrasounds, congenital heart disease lesions with normal four chamber view has low sensitivity in prenatal detection.
  • Prenatal diagnosis of pulmonary valve stenosis maybe improved with the use of cine clips to define the valve mobility and color flow Doppler to detect valvar flow acceleration.
  • Unlike pulmonary atresia and intact ventricular septum, fetuses with pulmonary stenosis has near normal appearing tricuspid valve and pulmonary valve size making the diagnosis of pulmonary stenosis without cine clips and Doppler imaging challenging.

 

Comment from Dr. Jennifer Johnson (Pittsburgh, PA), Section Editor of Pediatric Cardiology Journal Watch:  This is a single center retrospective chart review of all neonates who underwent cardiac catherization for critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum.  The cohort was evaluated for prenatal diagnosis of congenital heart disease and those patient’s fetal echocardiograms were reviewed.

Methods:  Data was collected on all neonates who underwent cardiac catheterization from 2000-2014 at Boston Children’s Hospital with the diagnosis of critical pulmonary valve stenosis or pulmonary atresia intact ventricular septum.

Results:  A total of 178 patients met inclusion criteria with 91 patients having critical pulmonary valve stenosis and 87 patients have pulmonary atresia intact ventricular septum.   Prenatal diagnosis was significant lower in those neonates with critical pulmonary valve stenosis at 37% compared to those with pulmonary atresia intact ventricular septum 60% (p=0.003).

Tricuspid and pulmonary valve measurements were evaluated for those patients with fetal echocardiograms and all patients with postnatal transthoracic echocardiograms.  The tricuspid and pulmonary valve z score was significantly higher in those patients with critical pulmonary stenosis compared to those with pulmonary atresia intact ventricular septum (p = <0.001 and p =0.004).  Comparison of the transthoracic tricuspid and pulmonary valve z score of those patients prenatally and postnatally diagnosed showed no statically significance.

 

Discussion: In this cohort, as the authors proposed the prenatal detection of critical pulmonary valve stenosis would be significantly lower than those patients with pulmonary atresia intact ventricular septum.   The decreased detection for those patients with critical pulmonary valve stenosis was thought to be linked to a normal appearing tricuspid valve and right ventricular on the obstetric screening four chamber view, obstetric imaging of the right ventricular outflow tract was not routinely recommended until 2013 and the decreased obstetric use of cine/color flow mapping imaging.  Lastly, critical pulmonary valve stenosis may progress in severity over pregnancy therefore being undetected in routine obstetric ultrasound.

Limitations:  Single center, retrospective study.

Next Steps:  It would be of interest to see how many of the critical pulmonary valve stenosis patients had progressive pulmonary valve disease with a normal screening obstetric ultrasound at 18-20 weeks of gestation.

 

Figure 1 Kawasaki disease emergency management of suspected myocardial ischaemia in children with previous Kawasaki disease and possible coronary artery aneurysms. CAA, coronary artery aneurysms; nSTEMI, non-STEMI; PSP, person-specific protocol; STEMI, ST-elevation myocardial infarction.

 

 

Figure 2 Kawasaki disease emergency management of suspected myocardial ischaemia in adults with previous Kawasaki disease and possible coronary artery aneurysms. nSTEMI, non-STEMI; STEMI, ST-elevation myocardial infarction.

 

 

 

Fetal Cardiology Abstracts of April 2020

 

  1. Identification of epigenetic factor KAT2B gene variants for possible roles in congenital heart diseases.

Hou YS, Wang JZ, Shi S, Han Y, Zhang Y, Zhi JX, Xu C, Li FF, Wang GY, Liu SL.

Biosci Rep. 2020 Apr 30;40(4). pii: BSR20191779. doi: 10.1042/BSR20191779.

PMID: 32239175 Free PMC Article

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  1. Preterm Birth of Infants Prenatally Diagnosed with Congenital Heart Disease, Characteristics, Associations, and Outcomes.

Mustafa HJ, Cross SN, Jacobs KM, Tessier KM, Tofte AN, McCarter AR, Narasimhan SL.

Pediatr Cardiol. 2020 Apr 30. doi: 10.1007/s00246-020-02345-8. [Epub ahead of print]

PMID: 32356015

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  1. Prenatal diagnosis of fetal aortopulmonary window by two- and four-dimensional echocardiography with spatiotemporal image correlation.

Tang H, Wang Y, Sun X, Zhang Y.

Echocardiography. 2020 Apr 29. doi: 10.1111/echo.14666. [Epub ahead of print]

PMID: 32347569

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  1. EM-mosaic detects mosaic point mutations that contribute to congenital heart disease.

Hsieh A, Morton SU, Willcox JAL, Gorham JM, Tai AC, Qi H, DePalma S, McKean D, Griffin E, Manheimer KB, Bernstein D, Kim RW, Newburger JW, Porter GA Jr, Srivastava D, Tristani-Firouzi M, Brueckner M, Lifton RP, Goldmuntz E, Gelb BD, Chung WK, Seidman CE, Seidman JG, Shen Y.

Genome Med. 2020 Apr 29;12(1):42. doi: 10.1186/s13073-020-00738-1.

PMID: 32349777 Free PMC Article

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  1. Good at heart: Developing a tertiary perinatal cardiac service; the first eight years of experience.

Heland S, de Chellis A, Rieder W, Sleeman M, Johns J, Lancefield T, Robinson A, Fung A, Walker S.

Aust N Z J Obstet Gynaecol. 2020 Apr 23. doi: 10.1111/ajo.13160. [Epub ahead of print]

PMID: 32323865

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  1. The Evaluation of Fetal Cardiac Remote Screening in the Second Trimester of Pregnancy Using the Spatio-Temporal Image Correlation Method.

Inamura N, Taniguchi T, Yamada T, Tanaka T, Watanabe K, Kumagaya K, Ishii Y, Ishii T, Hayashi G, Shiono N, Ikeda T; Osaka Fetal Cardiology Group.

Pediatr Cardiol. 2020 Apr 23. doi: 10.1007/s00246-020-02346-7. [Epub ahead of print]

PMID: 32328669

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  1. Systems Analysis Implicates WAVE2 Complex in the Pathogenesis of Developmental Left-Sided Obstructive Heart Defects.

Edwards JJ, Rouillard AD, Fernandez NF, Wang Z, Lachmann A, Shankaran SS, Bisgrove BW, Demarest B, Turan N, Srivastava D, Bernstein D, Deanfield J, Giardini A, Porter G, Kim R, Roberts AE, Newburger JW, Goldmuntz E, Brueckner M, Lifton RP, Seidman CE, Chung WK, Tristani-Firouzi M, Yost HJ, Ma’ayan A, Gelb BD.

JACC Basic Transl Sci. 2020 Apr 8;5(4):376-386. doi: 10.1016/j.jacbts.2020.01.012. eCollection 2020 Apr.

PMID: 32368696 Free PMC Article

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  1. First trimester fetal echocardiography limitations and its expected clinical values.

Kamel H, Yehia A.

Egypt Heart J. 2020 Apr 7;72(1):16. doi: 10.1186/s43044-020-00049-1.

PMID: 32266496 Free PMC Article

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  1. Novel foetal echocardiographic image processing software (5D Heart) improves the display of key diagnostic elements in foetal echocardiography.

Hu WY, Zhou JH, Tao XY, Li SY, Wang B, Zhao BW.

BMC Med Imaging. 2020 Apr 3;20(1):33. doi: 10.1186/s12880-020-00429-8.

PMID: 32245426 Free PMC Article

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  1. Congenital heart disease, prenatal diagnosis and management.

Meller CH, Grinenco S, Aiello H, Córdoba A, Sáenz-Tejeira MM, Marantz P, Otaño L.

Arch Argent Pediatr. 2020 Apr;118(2):e149-e161. doi: 10.5546/aap.2020.eng.e149. English, Spanish.

PMID: 32199055 Free Article

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  1. Fetal Congenital Heart Disease Echocardiogram Screening Based on DGACNN: Adversarial One-Class Classification Combined with Video Transfer Learning.

Gong Y, Zhang Y, Zhu H, Lv J, Cheng Q, Zhang H, He Y, Wang S.

IEEE Trans Med Imaging. 2020 Apr;39(4):1206-1222. doi: 10.1109/TMI.2019.2946059. Epub 2019 Oct 7.

PMID: 31603775

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  1. MR imaging of the fetal heart.

Marini D, van Amerom J, Saini BS, Sun L, Seed M.

J Magn Reson Imaging. 2020 Apr;51(4):1030-1044. doi: 10.1002/jmri.26815. Epub 2019 Jun 13. Review.

PMID: 31190452

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  1. Prenatal diagnosis of chromosomal aberrations in fetuses with conotruncal heart defects by genome-wide high-resolution SNP array.

Lin M, Zheng J, Peng R, Du L, Zheng Q, Lei T, Xie H.

J Matern Fetal Neonatal Med. 2020 Apr;33(7):1211-1217. doi: 10.1080/14767058.2018.1517316. Epub 2018 Sep 20.

PMID: 30149741

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  1. Impact of maternal pulmonary insufficiency on fetal growth in pregnancy.

Brun S, L’Ecuyer E, Dore A, Mongeon FP, Guedon AC, Leduc L.

J Matern Fetal Neonatal Med. 2020 Apr;33(7):1100-1106. doi: 10.1080/14767058.2018.1514492. Epub 2018 Sep 19.

PMID: 30130989

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  1. The accuracy of prenatal diagnosis of major congenital heart disease is increasing.

Lytzen R, Vejlstrup N, Bjerre J, Bjørn Petersen O, Leenskjold S, Keith Dodd J, Stener Jørgensen F, Søndergaard L.

J Obstet Gynaecol. 2020 Apr;40(3):308-315. doi: 10.1080/01443615.2019.1621814. Epub 2019 Aug 28.

PMID: 31455124

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  1. Cardiac progenitors and paracrine mediators in cardiogenesis and heart regeneration.

Witman N, Zhou C, Grote Beverborg N, Sahara M, Chien KR.

Semin Cell Dev Biol. 2020 Apr;100:29-51. doi: 10.1016/j.semcdb.2019.10.011. Epub 2019 Dec 18. Review.

PMID: 31862220 Free Article

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  1. The Evaluation of Fetal Cardiac Remote Screening in the Second Trimester of Pregnancy Using the Spatio-Temporal Image Correlation Method.

Inamura N, Taniguchi T, Yamada T, Tanaka T, Watanabe K, Kumagaya K, Ishii Y, Ishii T, Hayashi G, Shiono N, Ikeda T; Osaka Fetal Cardiology Group.

Pediatr Cardiol. 2020 Apr 23. doi: 10.1007/s00246-020-02346-7. [Epub ahead of print]

PMID: 32328669

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  1. Fetal CHD and perinatal outcomes.

Ge CJ, Mahle AC, Burd I, Jelin EB, Sekar P, Jelin AC.

Cardiol Young. 2020 Apr 20:1-6. doi: 10.1017/S1047951120000785. [Epub ahead of print]

PMID: 32308170

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  1. Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

Ronai C, Freud LR, Brown DW, Tworetzky W.

Prenat Diagn. 2020 Apr 20. doi: 10.1002/pd.5715. [Epub ahead of print]

PMID: 32314369

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  1. A review of fetal and neonatal consequences of maternal systemic lupus erythematosus.

Limaye MA, Buyon JP, Cuneo BF, Mehta-Lee SS.

Prenat Diagn. 2020 Apr 13. doi: 10.1002/pd.5709. [Epub ahead of print]

PMID: 32282083

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  1. Neonatal management of prenatally suspected coarctation of the aorta.

Hede SV, DeVore G, Satou G, Sklansky M.

Prenat Diagn. 2020 Apr 11. doi: 10.1002/pd.5696. [Epub ahead of print]

PMID: 32277716

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Congenital Heart Anesthesia and Intensive Care

CHD Anesthesia April 2020

 

  1. Congenital Heart and Pediatric Cardiac Anesthesia and Intensive Care Review of Early 2020 Manuscripts
  2. Section Editors Section
    Viviane Nasr – Boston

     

     

     

     

Rania Abbasi – Indianapolis

  1. Conngenital Heart and Pediatric Cardiac Anesthesia and Intensive Care Review of Early 2020 Manuscripts 

     

    Bilateral Erector Spinae Plane Block for Acute Post-Surgical Pain in Adult Cardiac Surgical Patients: A Randomized Controlled Trial.

    Krishna SN, Chauhan S, Bhoi D, Kaushal B, Hasija S, Sangdup T, Bisoi AK. Krishna SN, et al. J Cardiothorac Vasc Anesth. 2019 Feb;33(2):368-375. doi: 10.1053/j.jvca.2018.05.050. Epub 2018 Jun 4.J Cardiothorac Vasc Anesth. 2019. PMID: 30055991 Clinical Trial.

     

    Take Home Points:

    • Postoperative pain management following pediatric cardiac surgery is largely dependent on parenteral opioid medications. Novel regional anesthetic techniques offer the promise of improved postoperative pain with reduced opioid consumption.
    • The authors conducted a single-center, single-blind, randomized controlled trial of preoperative erector spinae plane blocks compared to standard medical management.  They found improved pain scores in the first 10 hours post-extubation, lower overall fentanyl consumption in the first 12 hours, longer time to first rescue analgesia, and lower sedation scores.
    • The study is limited by the fact that it includes only otherwise healthy patients undergoing relatively simple cardiac surgeries, and who were extubated in the immediate post-operative period. Though the results demonstrated statistically significant differences in the outcomes measured, their clinical relevance and generalizability remains unclear.

     

    Commentary by Richard Hubbard, MD and Nischal Gautam, MD, pediatric cardiac anesthesiologists at the McGovern Medical School/Children’s Memorial Hermann Hospital in Houston, Texas.

     

    For anesthesiologists caring for children with congenital heart disease (CHD), the challenge of balancing adequate analgesia with over sedation in the acute postoperative period is well-known.  Neuraxial anesthesia can significantly improve pain control, lower opioid requirements, and improve respiratory function; however, the specter of epidural hematoma in patients requiring full anticoagulation for surgery is a major concern.1  Paravertebral blocks are a valid alternative, but they carry the risk of pneumothorax.2 Fascial plane blocks offer the benefit of an improved safety profile while still providing significant analgesia for major thoracic, abdominal, pelvic, and lower extremity surgery.

    The erector spinae block (ESB) is one such technique.  Utilizing ultrasound guidance, local anesthetic is deposited deep to the erector spinae muscle and superficial to the transverse process.3  The resulting multi-dermatomal sensory block is believed to be caused by migration of local anesthetic to the paravertebral space, blocking the dorsal and ventral rami of the spinal nerves.3,4  Both single-shot and catheter-based techniques have been described.3-5  The ESB has been used with success in pediatric patients undergoing major thoracic, abdominal, and even lower extremity procedures.3,5,6  It has also shown promise in reducing postoperative pain following sternotomy for cardiac surgery in adults.2,7

    Kaushal and colleagues attempted to determine if presurgical single-shot, bilateral ESBs were effective in reducing postoperative pain and sedation in pediatric patients undergoing cardiac surgery via midline sternotomy.  This was a single-center, randomized, observer-blinded study. Exclusion criteria included the following: need for redo or emergency surgery, intubation for three or more hours postoperatively, ASA class III and above, heart failure, inotropic support requirement, history of ventricular arrhythmias, and allergy to amide anesthetics. Patients were randomized into a control group (scheduled intravenous (IV) acetaminophen and rescue fentanyl) and a block intervention group (bilateral ESBs plus scheduled IV acetaminophen and rescue fentanyl).  The blocks were conducted with ultrasound guidance at the T3 level, with 1.5 mg/kg of 0.2% ropivacaine deposited on each side.  Outcomes included pain scores using the Modified Objective Pain Scale (MOPS) for the first 12 hours postoperatively, intraoperative and postoperative fentanyl requirements, time to first rescue analgesic medication, extubation time, Ramsay sedation score, intensive care unit (ICU) length of stay, and adverse events. The MOPS grades crying, movement, agitation, posture, and verbal for a maximum of 10 points (0-2 points per category). To provide a perspective on the pain assessment using this scale, the following patients could be graded as such:

    • An asleep, calm, comfortable child is graded 0
    • A consolable, restless, mildly agitated child is graded 3
    • A consolable, restless, mildly agitated child complaining of non-localizing pain is graded 4
    • A consolable, restless, mildly agitated child complaining and localizing pain is graded 6
    • A non-consolable, thrashing, hysterical child, localizing pain with breath-holding is graded 10.

     

    Out of 100 patients recruited, 80 met inclusion criteria, and 40 patients were included in each control and block group.  Groups were similar in terms of patient age (just over two years), surgery type (mix of atrial septal defect (ASDs) and ventricular septal defect (VSDs)), and short bypass and cross clamp times (approximately 40 and 20 minutes, respectively). Statistically, the block group had lower pain scores up to (but not including) 12 hour after extubation, longer time to rescue analgesia, lower postoperative fentanyl requirement, lower sedation scores, and shorter ICU length of stay.

    Clinically, the most impressive differences between the two groups was that while all the patients in the control group received fentanyl within 12 hours after extubation, 30% of patients in the block group did not receive any fentanyl during this period. The block group also had a longer period free from the first analgesic rescue (1.8 hrs. vs 4.5 hrs.) and had a lower fentanyl requirement (1.1 versus 5.5μg/kg). Within the first 12 hours after extubation, none of the patients in either group were noted to have a pain score of more than 5, indicating good pain control with either therapy. There were no differences seen in major postoperative complications.

    What This Means for Our Practice

    The authors showed that single-shot, bilateral ESBs reduced opioid requirements with slightly lower pain scores in the initial postoperative period. Although pain scores were lower in the block group, the efficacy of the block lasted for only 10 hours.  This may be explained by the limited duration of action of single-shot fascial plane blocks when compared to indwelling catheters.  A major critique of this work is its generalizability to the majority of pediatric patients presenting for congenital heart surgery.  The exclusion criteria essentially eliminated all but the healthiest patients, and the surgeries performed were the simplest and fastest cardiopulmonary bypass cases performed in children.  While the authors in this study show that single-shot ESBs appear to be helpful in patients who will be fast-tracked for extubation  their validity for post-sternotomy pain in a diverse group of patients, particularly those with borderline cardiopulmonary reserve (cyanotic patients for Glenn, Fontan procedure), requires significantly more investigation before it can be adopted broadly.  This block may have limited utility in more complex, longer duration procedures in which children are likely to require continued intubation in the immediate postoperative period.  Many of these less complex cardiac lesions can be corrected without the need for a sternotomy, either via device closure in the catheterization laboratory or via a minimally invasive approach, which may also limit the utility of the ESB.  Additionally, outcomes 12 hours after the procedure and other metrics until hospital discharge were not presented in the manuscript.

    The ESB remains a promising modality to prevent post-sternotomy pain.  The attractiveness of the block is the ease of its performance under ultrasound guidance. The target is easy to visualize, fast to perform, and the needle trajectory is straightforward. In addition, the risk of injury to nerves, dura, pleura, lungs, or vascular structures is minimal, as none of these structures are in the needle path or adjacent to the plane of injection. The idea of bilateral erector spinae continuous block with dual catheters for longer analgesia in post-sternotomy pain is promising, and provides an avenue for future research in this area.

     

    References:

     

    1. Rosen D, Hawkinberry D, Rosen K, et al. An epidural hematoma in an adolescent patient after cardiac surgery. Anesth Analg. 2004; 98(4): 966-969.
    2. Muñoz-Leyva F, Chin J, Mendiola W, et al. Bilateral Continuous Erector Spinae Plane (ESP) Blockade for Perioperative Opioid-Sparing in Median Sternotomy. J Cardiothorac Vasc An. 2019; 33(6): 1698-1703.

     

    1. Muñoz, F., Cubillos, J., Bonilla, A.J. et al. Erector spinae plane block for postoperative analgesia in pediatric oncological thoracic surgery. Can J Anesth/J Can Anesth 2017; 64: 880–882.

     

    1. Kaushal B, Chauhan S, Magoon R, Krishna NS, et al. Efficacy of bilateral erector spinae plane block in management of acute postoperative surgical pain after pediatric cardiac surgeries through a midline sternotomy. J Cardiothorac Vasc An. 2020; 34: 981-986.

     

    1. De la Cuadra-Fontaine JC, Concha M, Vuletin F, Arancibia H. Continuous Erector Spinae Plane block for thoracic surgery in a pediatric patient. Paediatr Anaesth. 2018; 28(1): 74-75.

     

    6.Munshey F, Caruso T, Wang E, Tsui B. Programmed Intermittent Bolus Regimen for Erector Spinae Plane Blocks in Children: A Retrospective Review of a Single-Institution Experience. Anesth Analg. 2020; 130(3): e63-e66.

     

    1. Krishna NS, Chauhan S, Bhoi D, et al. Bilateral Erector Spinae Plane Block for Acute Post-Surgical Pain in Adult Cardiac Surgical Patients: A Randomized Controlled Trial. J Cardiothorac Vasc An. 2019; 33: 368-375

     

     

    Comparison of intranasal midazolam, intranasal ketamine, and oral chloral hydrate for conscious sedation during paediatric echocardiography: results of a prospective randomised study.

    Alp H, Elmacı AM, Alp EK, Say B.Cardiol Young. 2019 Sep;29(9):1189-1195. doi: 10.1017/S1047951119001835.PMID: 31451130

     

    Take Home Points:

    • Intranasal midazolam, intranasal ketamine, and oral chloral hydrate all provide adequate sedation for transthoracic echocardiography in acyanotic children ages 9 – 38 months with a failed sedation rate of 4.1%, 4.1% and 5.5%, respectively.
    • Midazolam had the shortest onset of action at 14 (range 7-65) minutes.
    • Ketamine had the shortest duration of sedation at 55 (range 25-75) minutes.
    • Each of the sedatives had modest side effects, including agitation (midazolam), respiratory (midazolam and chloral hydrate) and gastrointestinal (ketamine and chloral hydrate).

     

    Commentary by Lori Q. Riegger MD, Associate Professor, Mott Children’s Hospital, Michigan Medicine in Ann Arbor, Michigan.

     

    Young children often require sedation to obtain high quality diagnostic images during transthoracic echocardiography. Midazolam, ketamine, and chloral hydrate are used for sedation in varying doses and with various routes of administration. Onset, duration, side effects and success of the sedatives are key elements in choice of appropriate medication for sedation and were the focus of this study.

    This prospective randomized study compared the efficacy of three sedatives: intranasal midazolam, intranasal ketamine and oral chloral hydrate in acyanotic children aged 9 – 38 months, undergoing a transthoracic echocardiogram to assess a cardiac murmur. The goal was to determine the ideal sedative, defined as providing adequate sedation (as evidenced by high quality images), rapid onset, optimal duration and low incidence of side effects.  Two-hundred seventeen children, with median age 22 months (range 9 – 38 months), were included. There were no statistically significant differences in age, weight or gender between the groups. All subjects were awake for at least 6 hours prior to sedation. They were randomized to one of the three groups; 73 received midazolam (0.2 mg/kg, max 5 mg), 72 received ketamine, (4 mg/kg, max 100 mg), 72 received chloral hydrate (50 mg/kg, max 1 gram).  After the medication was given, depth of sedation was assessed using the Richmond Agitation Sedation Scale (RASS) at 15, 30, 45 and 60 minutes by two blinded pediatricians, using 1) calm, 2) drowsy or 3) sedated, to assess the level of sedation. Sedation was defined as complete unconsciousness and lack of movement. Rescue medications were not used during the study. Success was defined by RASS scores and ability to obtain high quality echocardiographic images.

    Vitals, including temperature, blood pressure, heart rate, respiratory rate and oxygen saturation, showed no statistically significant differences between the groups during the sedated and non-sedated phases. Echocardiography could not be performed due to failed sedation in three children each in the midazolam and ketamine groups and four in the chloral hydrate group. Successful sedation thus occurred in 95.9% of the midazolam, 95.9% of the ketamine, and 94.5% of the chloral hydrate cohorts.

    Fifteen minutes after administration midazolam demonstrated the shortest onset of action with 37.1% drowsy and 35.8% sedated, while ketamine had 34.7% drowsy and none sedated, and chloral hydrate had 23.6% drowsy and none sedated. The median onset of sedation was 14 (range 7-65) minutes (p < 0.001) for midazolam, 34 (range 12-56) minutes for ketamine (p < 0.001), and 40 (range 25-57) minutes for chloral hydrate (p < 0.001). At 60 minutes all of the midazolam and ketamine patients and 92.7% of the chloral hydrate patients were sedated. Median duration of sedation was 68 (range 20-75) minutes for patients receiving midazolam (p = 0.023), 55 (range 25-75) minutes for ketamine (p = 0.712), and 61 (range of 34-78) minutes for chloral hydrate (p= 0.045).

    One patient in the midazolam group and one in the chloral hydrate group required oxygen to maintain saturation > 95%, and in both patients, the oxygen was weaned off during the first 60 minutes of sedation. Other side effects included agitation in 3 midazolam patients, and nausea and vomiting in two ketamine and eight chloral hydrate patients.

     

    What does this mean for us?

    In this study, intranasal midazolam, intranasal ketamine and oral chloral hydrate were all demonstrated to be efficacious in providing adequate sedation with a low side effect profile in acyanotic young children undergoing transthoracic echocardiography. A limitation of this study is that it did not include children with cyanotic cardiac disease and therefore, these findings cannot be extrapolated to that population.

    Although midazolam provided the shortest onset of sedation at 14 (7-65) minutes, ketamine patients spent less time sedated at 55 (25-75) minutes, which the authors concluded to be optimal. This should be enough time for all but the most difficult echocardiograms. Each of the sedatives had side effects including agitation (midazolam), respiratory (midazolam and chloral hydrate), and gastrointestinal (ketamine and chloral hydrate), all of which have been reported previously with these medications. The side effects involved few patients and were minor.

    The authors do not comment on the overall length of time from administration to complete recovery, but if one assesses the median time of onset of sedation and the median duration of sedation, it appears that patients who received chloral hydrate required monitoring for longer periods of time compared with the patients who had received midazolam or ketamine. In fact, it is somewhat surprising that the study did not demonstrate any prolonged sedative effects of chloral hydrate, including lack of muscle tone and coordination, which have been previously reported. Also, chloral hydrate has become scarce in the United States, so the chloral hydrate arm of the study is likely to be more pertinent for those outside that country. A similar study involving the more commonly used intranasal dexmedetomidine compared with midazolam and ketamine may be more relevant for those who practice in the United States.

     

     

     

    Intraoperative methadone is associated with decreased perioperative opioid use without adverse events: a case-matched cohort study.

    Robinson JD, Caruso TJ, Wu M, Kleiman ZI, Kwiatkowski DM.

    Journal of Cardiothoracic and Vascular Anesthesia 2020; 34: 335-341.

    DOI:  10.1053/j.jvca.2019.09.033

    PMID:  31699597

     

    Take Home Points:

     

    • Intraoperative methadone was associated with a decrease of total opioid exposure during the early postoperative period with comparable analgesic efficacy and no increase in adverse events compared to the control group.

     

    • Corrected QTc prolongation was not increased in the methadone group when compared to the control group.

     

    Commentary by Destiny F. Chau MD, pediatric cardiac anesthesiologist at Arkansas Children’s Hospital in Little Rock, Arkansas.

     

    Opioids have a critical role in the pain management of pediatric patients undergoing congenital heart surgery. The advantages of perioperative opioids are eclipsed by their associated risks of short-term and long-term adverse events, including concerns for persistent opioid use and undue exposure in the setting of the current opioid crisis. Efforts continue in the search for effective opioid-sparing strategies for perioperative pain management, and also for maximizing the benefits of the chosen opioids while minimizing their side effects.  The available literature, mostly from adult studies, suggest that the intraoperative use of long-acting opioids such as methadone, may decrease total perioperative opioid exposure without an increase in complications.

    The authors of this single-center retrospective, case-matched cohort study primarily aimed to evaluate the association of intraoperative methadone use on total perioperative opioid exposure in children undergoing congenital heart surgery. Secondary outcomes included the association of intraoperative methadone with adverse events, clinical outcomes and dose-dependent effect on total opioid use.  Pediatric patients receiving intraoperative methadone during congenital heart surgery from November 2017 to July 2018 were retrospectively identified via a pharmaceutical electronic database. Each of them was matched according to age and surgical procedure with a control who did not receive methadone. Patients were excluded if they received opioids the day before the surgery, had postoperative nerve blocks, had delayed sternal closure, required additional procedures during the first postoperative 24 h or had not found a matched control. A total of 37 patients were matched 1:1 with a control patient. The intraoperative use and dosing of methadone was at the discretion of the attending anesthesiologist.  Anesthetic managements were not standardized, including use of other opioids and sedatives. Postoperatively, analgesic control followed the cardiac intensive care unit’s (ICU) multimodal pain management protocols. For analysis purposes, all intravenous and enteral opioids were converted to morphine equivalents per kilogram and were compared in 12 h blocks until 36 h postoperatively.  Also, patients in the methadone group were divided into low-dose (N = 22; 0.2 mg/kg or less) and high-dose (N = 15; over 0.2 mg/kg) to study dose-dependent effects.

    Both cohorts were similar in demographics and surgical bypass times. Median age and weight were 8.9 years and 22.9 kg for the methadone and 7.4 years and 26.3 kg for the control groups. Surgical procedures had a median complexity category of 3 and interquartile range of 2 to 4 per the Society of Thoracic Surgeons-European Association of Cardio-Thoracic Surgery (STAT) system. Cardiopulmonary bypass (CPB) was used in 33 patients (89%). Methadone dosages ranged from 0.1 to 0.4 mg/kg with a maximum of 30 mg, with the majority of the overall dose given during the earlier parts of the case and administered prior to commencement of CPB. Use of nonopioid sedatives and anesthetics, including inhaled agents were comparable between cohorts. Two patients in each group were extubated in the operating room. More patients in the methadone group received intraoperative morphine, while none received benzodiazepines in the ICU period as compared to the control group.

    Primary outcome results showed decreased total opioid dose in the methadone group versus the control group intraoperatively (2.51 v 4.39 mg morphine equivalent/kg), during the initial 12 h postoperatively (0.43 v 1.28 mg morphine equivalent/kg), and cumulatively during the entire postoperative 36 h (0.83 v 1.91 morphine equivalents/kg). No difference was found during the second and third 12 h time blocks.  For secondary outcomes, intraoperative methadone was not associated with time to extubation, postoperative pain scores, postoperative nausea and vomiting, ICU length of stay, rate of extubation failure or in-hospital mortality. Corrected QTc prolongation was not increased in the methadone group when compared to the control group. No difference was observed in total opioid exposure based on high or low methadone dosing.

    What does this mean for us?

    Retrospective study limitations aside, the authors are commended for studying the use of intraoperative methadone in this patient population in the quest of achieving adequate analgesia with lower opioid exposure and minimal adverse effects.

    Although the results report a total opioid exposure reduction during the first postoperative 12 h period that was not sustained through the second and third 12 h blocks, the total cumulative dose (intraoperatively through the postoperative 36 h) were reduced with comparable analgesic effectiveness and without an observed increase in complications. This is promising data for methadone’s role as part of the armamentarium to improve safe effective care for all patients while combating the opioid epidemic and navigating through different periodic opioid shortages.

    Methadone is a synthetic μ-opioid agonist with N -methyl-D-aspartate receptor antagonism properties, whose pharmacokinetics and pharmacodynamics in children are not well studied. Methadone is described to have a rapid onset and long elimination half-life, which would clinically suggest a relatively fast onset of action with prolonged effect and less frequent redosing.  Sharma and his group found that the pharmacokinetics of methadone in adolescents undergoing non-cardiac surgery were comparable to those in adults. The Society for Pediatric Anesthesia, providing guidance for perioperative opioid use in children, reports that “methadone pharmacodynamics effects are prolonged compared to morphine and the pharmacokinetic profile appears to be consistent across pediatric age ranges”. In this study, an important factor to highlight is that most of the methadone dosing occurred prior to starting CPB; CPB would alter methadone’s pharmacokinetics due to the added volume of distribution from the prime and exposure to the bypass circuit surfaces. As a speculation, dosing methadone after CPB separation has the potential to confer a longer postoperative opioid-sparing time period.

    In conclusion, this single-center retrospective cohort-matched study explored the opioid-sparing effect of intraoperative methadone use on pediatric patients undergoing congenital cardiac surgery. The results showed that intraoperative methadone was associated with a decrease of total opioid exposure during the early postoperative period with comparable analgesic efficacy and no increase in adverse events compared to the control group. This promising concept should be further investigated with prospective trials including larger number of patients and expanded age range to better characterize methadone’s role during congenital cardiac surgery.

     

     

    References:

     

    1. Robinson JD, Caruso TJ, Wu M, Kleiman ZI, Kwiatkowski DM. Intraoperative Methadone Is Associated with Decreased Perioperative Opioid Use Without Adverse Events: A Case-Matched Cohort Study. J Cardiothorac Vasc Anesth. 2020; 34(2): 335–341.

     

    1. Sharma A, Tallchief D, Blood J, Kim T, London A, Kharasch ED. Perioperative Pharmacokinetics of Methadone in Adolescents.  Anesthesiology2011; 115(6): 1153-1161.

     

    1. Cravero JP, Agarwal R, Breed C, et al. The Society for Pediatric Anesthesia recommendations for the use of opioids in children during the perioperative period. Paediatr Anaesth. 2019; 29(6): 547–571.

     

    1. Valencia E, Nasr VG. Is Methadone an Opioid Sparing Strategy? J Cardiothorac Vasc Anesth. 2020 Feb; 34(2):342-343. Nov 16.

     

     

     

     

    Congenital Heart and Pediatric Cardiac Anesthesia and Intensive Care Abstracts of April 2020

     

    1. The Year in Review: Anesthesia for Congenital Heart Disease 2019.

    Houska NM, Schwartz LI.

    Semin Cardiothorac Vasc Anesth. 2020 Apr 29:1089253220920476. doi: 10.1177/1089253220920476. [Epub ahead of print]

    PMID: 32347188

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    1. Anesthesia timing for children undergoing therapeutic cardiac catheterization after upper respiratory infection: a prospective observational study.

    Kan Z, Siyuan W, Mengqi L, Chi W, Liping S, Sen Z, Jie B, Mazhong Z, Jijian Z.

    Minerva Anestesiol. 2020 Apr 6. doi: 10.23736/S0375-9393.20.14293-7. [Epub ahead of print]

    PMID: 32251574

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    1. Effect of xenon and dexmedetomidine as adjuncts for general anesthesia on postoperative emergence delirium after elective cardiac catheterization in children: study protocol for a randomized, controlled, pilot trial.

    Devroe S, Devriese L, Debuck F, Fieuws S, Cools B, Gewillig M, Van de Velde M, Rex S.

    Trials. 2020 Apr 3;21(1):310. doi: 10.1186/s13063-020-4231-5.

    PMID: 32245513 Free PMC Article

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    1. Anesthetic Management of a Pediatric Patient With Cardiofaciocutaneous Syndrome.

    Shionoya Y, Yamamoto M, Sunada K, Nakamura K.

    Anesth Prog. 2020 Spring;67(1):45-47. doi: 10.2344/anpr-67-01-07.

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    1. Fifty Percent Effective Dose of Intranasal Dexmedetomidine Sedation for Transthoracic Echocardiography in Children With Cyanotic and Acyanotic Congenital Heart Disease.

    Yang F, Li S, Shi Y, Liu L, Ye M, Zhang J, Liu H, Liu F, Yu Q, Sun M, Tian Q, Tu S.

    J Cardiothorac Vasc Anesth. 2020 Apr;34(4):966-971. doi: 10.1053/j.jvca.2019.11.037. Epub 2019 Dec 6.

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    1. Anesthetic management of catheter-based patent ductus arteriosus closure in neonates weighing <3 kg: A Retrospective Observational Study.

    Hubbard R, Edmonds K, Rydalch E, Pawelek O, Griffin E, Gautam N.

    Paediatr Anaesth. 2020 Apr;30(4):506-510. doi: 10.1111/pan.13838. Epub 2020 Feb 24.

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    1. Assessment of an Unplanned Extubation Bundle to Reduce Unplanned Extubations in Critically Ill Neonates, Infants, and Children.

    Klugman D, Melton K, Maynord PO, Dawson A, Madhavan G, Montgomery VL, Nock M, Lee A, Lyren A.

    JAMA Pediatr. 2020 Apr 13:e200268. doi: 10.1001/jamapediatrics.2020.0268. [Epub ahead of print]

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    1. AANA JournalCourse: Update for Nurse Anesthetists-Optimizing Mechanical Ventilation During General Anesthesia.

    Wright GT, Ashworth L, Pettey S.

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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.

We encourage your continued support as well as your valuable comments and feedback.

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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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