CHiP Network Congenital Heart Journal Watch – August 2019
Pediatric Cardiology Featured Articles
Pediatric Cardiology Featured Manuscripts of June 2019
Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium).
Oster ME, McCracken C, Kiener A, Aylward B, Cory M, Hunting J, Kochilas LK.
Am J Cardiol. 2019 Jun 6. pii: S0002-9149(19)30626-5. doi: 10.1016/j.amjcard.2019.05.047. [Epub ahead of print]
PMID: 31272703
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Take Home Points:
• The long-term survival in patients with aortic coarctation who underwent surgery before the age of 12 months is excellent.
• Weight <2.5 kg at the time of CoA repair, presence of a genetic syndrome and surgery before 1990 seems to be associated with increased late mortality.
Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch: It has been shown that patients with aortic coarctation (CoA) have an increased cardiovascular morbidity and mortality despite successful surgical repair. This retrospective study evaluated a large cohort of CoA patients who underwent surgical repair before 12 months of age between 1982 and 2003 using data from the Pediatric Cardiac Care Consortium (US-based registry for interventions for pediatric heart diseases) as well as the National Death Index and the Organ Sharing Procurement Network. Long-term survival and risk factors associated with late mortality were assessed.
Patients who had lesions other than atrial and ventricular septal defect, bicuspid aortic valve, aortic stenosis and hypoplastic aortic arch were excluded. The CoA type was categorized as follows: 1) CoA with genetic syndrome, 2) CoA with VSD, 3) CoA with left-sided obstruction including hypoplastic aortic arch, bicuspid aortic valve, aortic stenosis and 4) simple CoA with no accompanying defects other than a patent ductus arteriosus or atrial septal defect.
2424 CoA patients from 43 centers met the inclusion criteria. Of them 57 died postoperatively during the admission for CoA repair. 2367 patients were discharged alive and of them 1913 patients had adequate identifiers to be submitted to National death Index and Organ Procurement and Transplant Network. The 1-year and 20-year survival of these patients was 97.5% and 95.6%, respectively. Those patients who were discharged alive were likely to be older at the time of surgery, to have a greater weight at the time of surgical repair, to have undergone surgical repair outside the neonatal period and to have a simple CoA. Those patients who died during the postoperative course after CoA repair were likely to have a genetic syndrome and CoA with VSD. The overall long-term survival of all patients included in this study was 94.5% (Figure 2A) and 95.8% of those who were discharged alive (Figure 2B).
CoA repair as a neonate was associated with worse survival (Figure 3A) and patients with a genetic syndrome had worse survival compared to simple CoA (Figure 3B). Furthermore, patients who underwent patch angioplasty and subclavian flap repair showed decreased survival compared to patients who had surgical repair with end-to-end anastomosis (Figure 3C). Weight <2.5 kg at the time of repair, presence of a genetic syndrome and surgery in the 1980’s was associated with increased late mortality. More than half of the deaths were related to the cardiovascular system.
Overall, this interesting study increases our knowledge about the long-term survival in CoA patients who underwent surgical repair in the neonatal period and infancy.
Figure 2
Figure 3
Factors associated with exercise capacity in patients with a systemic right ventricle.
Gavotto A, Abassi H, Rola M, Serrand C, Picot MC, Iriart X, Thambo JB, Iserin L, Ladouceur M, Bredy C, Amedro P.
Int J Cardiol. 2019 Jun 13. pii: S0167-5273(19)31187-8. doi: 10.1016/j.ijcard.2019.06.030. [Epub ahead of print]
PMID: 31256996
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Take Home Points:
• NYHA functional class is a strong predictor of reduced exercise capacity in patients with a systemic right ventricle.
• Impaired right ventricular functional parameters are also associated with limited exercise capacity, while 2D global longitudinal strain correlated best with percent-predicted VO2max.
Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch: This is a French multicenter study assessing factors related to exercise capacity in a cohort of patients with a systemic right ventricle. The authors included 111 patients with transposition of the great arteries after atrial switch operation (n= 94) and patients with congenitally corrected transposition of the great arteries (n= 17). The mean age was 37.2 ± 8.2 years. Patients who were included in this study had at least a physical examination, a cardiopulmonary exercise test (CPET), an ECG and an echocardiogram.
68.5% of the patients were in NYHA I functional class. A right ventricular assistance or cardiac transplantation project was considered in 10 patients. Conduction disorders were common (70 patients) and included sinus node dysfunction, complete right bundle branch block and complete atrioventricular block. 34 patients had supraventricular tachycardias including atrial flutter and atrial fibrillation. Echocardiographic parameters (TAPSE, S wave, TEI index, RV surface shortening fraction, global longitudinal 2D Strain) suggested RV dysfunction; 17% of the patients had severe tricuspid regurgitation.
The mean VO2max, the ventilatory anaerobic threshold and the VE/VCO2 slope were impaired (mean VO2max 23.3 ± 6.9 ml/kg/min; VO2 13.7 ± 5 ml/kg/min; VE/VCO2 slope 34 ± 8). In the univariate analysis professional status, NYHA functional class, BNP level, the type of systemic right ventricle, decreased right ventricular function values, tricuspid regurgitation, the presence of a pacemaker or an implantable defibrillator, the ventilatory anaerobic threshold, the maximum load, and the maximal heart rate during exercise correlated with VO2max (Table 4). In the multivariate analysis, VO2max correlated with NYHA functional class (Table 4). All RV functional parameters as assessed by echocardiography were related to exercise capacity (Table 4), while 2D global longitudinal strain analysis showed the best correlation.
Cardiomegaly on chest radiographs as a predictor of heart disease in the pediatric population.
Dasgupta S, Kelleman M, Slesnick T, Oster ME.
Am J Emerg Med. 2019 Jun 25. pii: S0735-6757(19)30426-7. doi: 10.1016/j.ajem.2019.06.045. [Epub ahead of print]
PMID: 31272753
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Take Home Points:
• Cardiomegaly on chest x-ray (CXR) has a relatively poor positive predictive value (PPV) for subsequent diagnosis of true heart disease.
• The addition of an EKG and BNP > 100 pg/ml improves the PPV, especially in patients < 1 year of age but does not negate the need for a subsequent echocardiogram.
Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Cardiomegaly on chest x-ray is not an infrequent reason for pediatric cardiology referral and consultation. False positive diagnoses may be common, with enlargement of the cardiac silhouette often due to other factors such as thoracic wall abnormalities, mediastinal factors (thymus), or technical issues. A small study in 2001 showed that CXR had pretty high specificity and negative predictive value (NPV), but poor sensitivity and PPV. The goal of this study was to determine the PPV of cardiomegaly on CXR in predicting true heart disease, and whether the addition of an EKG and BNP would have utility. They also stratified their results based on patient age.
This was a retrospective chart review of all pediatric patients with cardiomegaly on initial CXR and who had a subsequent echocardiogram within 30 days. A study investigator confirmed the finding of cardiomegaly by recording the cardio-thoracic ratio (CTI) in a blinded fashion. A cut-off of CTI > 0.55 was used as the definition of cardiomegaly, with only those confirmed included in the study. If obtained, EKG findings and BNP levels were recorded, with a BNP > 100 pg/ml considered abnormal. PPV and NPV were calculated for all patients with cardiomegaly on CXR alone, + abnormal EKG, + BNP > 100, + both abnormal EKG and abnormal BNP, and those with normal EKG and normal BNP. A subgroup analysis excluding those with an abnormal cardiac exam was also performed.
1544 patients out of 282,618 had cardiomegaly on CXR. 1055 were excluded since they did not have a subsequent echocardiogram or they had a known diagnosis of congenital disease, and 37 of those did not meet CXR criteria for cardiomegaly when reanalyzed. 489 patients met the inclusion criteria, with an EKG done in 275 patients, BNP in 176, and both in 122. 31% of patients were diagnosed with structural or functional heart disease or pericardial effusion. The median age of those with heart disease was significantly lower as compared to those without, with patients < 1 year of age most likely to have heart disease diagnosed by echo (see figure 1). See Table 2 for types of heart disease diagnosed. 59% of patients with heart disease had an abnormal CV exam. PPV of CXR alone was 31%, + abnormal EKG was 67.1%, + BNP > 100 was 59%, and if both was 76.7%. In patients < 1 year of age, the PPV for all categories was higher whether or not the physical exam was included (see figure 2).
CXR alone as expected did not have a good PPV, and while the addition of an EKG and BNP improved the PPV, 19% of patients still had some type of heart disease and would thus not eliminate the need for an echocardiogram. This is of course clinically relevant, as an echo typically is not done easily in the ER setting, and cost-relevant, as the cost of an echocardiogram is quite expensive as compared to the other tests. Additionally, the authors report that 41% of patients with heart disease had a normal physical exam and did little to increase the PPV. This should probably be taken with some caution, as the expertise in physical exam has been known to have decreased in general, and the ER setting is sometimes not ideal to elicit subtle findings. Overall, while adding these tests to the patient with cardiomegaly on CXR may help, many will still likely need to be referred for a full cardiology evaluation and echocardiogram.
Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome.
Karaca-Altintas Y, Laux D, Gouton M, Bensemlali M, Roussin R, Hörer J, Raisky O, Bonnet D.
Eur J Cardiothorac Surg. 2019 Jun 10. pii: ezz167. doi: 10.1093/ejcts/ezz167. [Epub ahead of print]
PMID: 31180449
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Take Home Points:
• In absent pulmonary valve syndrome (APVS), a Nakata index (NI) of > 1500 mm2/m2 is a risk factor for death, even after pulmonary artery (PA) plasty.
• Whether repair at an earlier age (e.g. lower NI) would affect outcome remains to be seen, but this study does provide some prognostic and clinical information that could help providers counsel families and provide good post-operative care.
Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: APVS, a rare congenital abnormality often associated with an outlet VSD and overriding aorta (e.g. Tetralogy of Fallot with absent pulmonary valve), is typically characterized by massive dilation of the pulmonary arteries. Respiratory symptoms can develop due to tracheal and bronchial compression, with the clinical presentation often dependent on the severity of those symptoms. High morbidity and mortality is present in neonates with symptoms soon after birth, but others can have more mild symptoms, with surgery being delayed for a few months until elective repair is undertaken. The study aims were to report clinical and surgical outcomes of patients with APVS, investigate predictors of mortality and prolonged postoperative ventilation, to find a threshold PA size predictive of negative outcomes, and to evaluate if PA plasty or the LeCompte maneuver would influence airway relief.
There were 3 groups: neonates < 28 days at repair, infants 28 days to 1 year old, children > 1 year old. Preoperative airway compression was evaluated with chest x-rays or CT scan and bronchoscopy when available, and graded as mild, moderate or severe. PA size was evaluated by echo and the Nakata index (sum of cross-sectional area of the left and right PAs in mm2 divided by BSA). Figure 1 shows the flowchart for surgical technique used. Prolonged mechanical ventilation (PMV) was defined as > 7 days. There were 68 total patients with a median age at repair of 3.9 months and weight of 5 kg. Airway compression was more frequent at a younger age and more severe in the neonatal group as would be expected. Median NI was 938 mm2/m2, and those that had surgery at a younger age, had pre- or postoperative airway compression, or required PMV had a significantly higher NI (see figure 2).
The mortality rate at discharge was 12% and overall was 19%, with most early and late deaths due to respiratory failure. Table 2 lists the preoperative univariate and multivariable risk factors for mortality, with only the NI and basal SpO2 % being significant. Figure 3 shows the ROC curves of the ability of the NI to predict outcomes. The AUC was 0.92. A cut off of 1500 mm2/m2 had a sensitivity of 98% and a specificity of 82%. Figure 4 shows the Kaplan Meier survival curves according to NI.
The median duration of ventilation was 15 days and only lower weight at repair was a risk factor in multivariable analysis for PMV. The accuracy of the NI for PMV was not as good as for survival (AUC 0.76). Patients with a PA plasty +/- LeCompte had an increased risk of death and neither had an effect on duration of ventilation. The NI for this subgroup remained the only risk factor for mortality, but the sensitivity/specificity of a cut-off of 1637 mm2/m2 for those with a LeCompte was not as high (75% and 80% respectively).
As the authors note, PA dilation can not only cause external compression of major airways, but can also be associated with intrinsic abnormal lung function due to distal bronchial compression. Increased small airway resistance has been shown in this population, even higher than those with RSV. This may be a reason why a PA plasty and LeCompte did not show significant benefit, as it only helped the more proximal airway compression. A limitation of this study was the use of echo to calculate the NI in 40% of the cohort, as most centers use CT or MRI for this. This may affect the NI cut-off for prognosis. Additionally, given the small overall sample size, it may be difficult to fully extrapolate the results.
Pediatric cardiology June 2019
1. Influence of the aldehyde dehydrogenase 2 polymorphism on the vasodilatory effect of nitroglycerin in infants with congenital heart disease and pulmonary arterial hypertension.
Nagano T, Ushijima K, Taga N, Takeuchi M, Kawada MA, Aizawa K, Imai Y, Fujimura A.
Eur J Clin Pharmacol. 2019 Jun 27. doi: 10.1007/s00228-019-02709-x. [Epub ahead of print]
PMID: 31250045
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2. Genetic counselling and testing in congenital heart defects and hereditary thoracic aortic disease: Complex but essential.
Breckpot J.
Eur J Prev Cardiol. 2019 Jun 26:2047487319860296. doi: 10.1177/2047487319860296. [Epub ahead of print] No abstract available.
PMID: 31242054
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3. Diagnostic and prognostic significance of cardiovascular vortex formation.
Kheradvar A, Rickers C, Morisawa D, Kim M, Hong GR, Pedrizzetti G.
J Cardiol. 2019 Jun 26. pii: S0914-5087(19)30143-1. doi: 10.1016/j.jjcc.2019.05.005. [Epub ahead of print] Review.
PMID: 31255458
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4. Evolution and trends in a pediatric cardiac magnetic resonance imaging program in a tertiary hospital over a 14-year period.
Marín Rodríguez C, Álvarez Martín T, Lancharro Zapata Á, Ruiz Martín Y, Sánchez Alegre ML, Delgado Carrasco J.
Radiologia. 2019 Jun 26. pii: S0033-8338(19)30066-9. doi: 10.1016/j.rx.2019.05.003. [Epub ahead of print] English, Spanish.
PMID: 31255319
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5. A variety of unusually abnormal vessels in tetralogy of fallot with absent pulmonary valve.
Saito M, Ishii T, Hamamichi Y.
Ultrasound Obstet Gynecol. 2019 Jun 26. doi: 10.1002/uog.20384. [Epub ahead of print]
PMID: 31240812
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6. Cardiomegaly on chest radiographs as a predictor of heart disease in the pediatric population.
Dasgupta S, Kelleman M, Slesnick T, Oster ME.
Am J Emerg Med. 2019 Jun 25. pii: S0735-6757(19)30426-7. doi: 10.1016/j.ajem.2019.06.045. [Epub ahead of print]
PMID: 31272753
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7. A case report on endarteritis in a child with coarctation of aorta.
Gnanam D, Bartelds B, van Leeuwen WJ, Frohn-Mulder IM, Koopman LP.
Echocardiography. 2019 Jun 25. doi: 10.1111/echo.14418. [Epub ahead of print]
PMID: 31237036
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8. Regenerative Therapy for Patients with Congenital Heart Disease.
Kimura N.
Keio J Med. 2019 Jun 25;68(2):29-38. doi: 10.2302/kjm.2018-0002-IR. Epub 2018 Jun 19.
PMID: 29925723 Free Article
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9. Utility of 3D Printed Cardiac Models for Medical Student Education in Congenital Heart Disease: Across a Spectrum of Disease Severity.
Smerling J, Marboe CC, Lefkowitch JH, Pavlicova M, Bacha E, Einstein AJ, Naka Y, Glickstein J, Farooqi KM.
Pediatr Cardiol. 2019 Jun 25. doi: 10.1007/s00246-019-02146-8. [Epub ahead of print]
PMID: 31240370
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10. Echocardiographic assessment of single-ventricle diastolic function and its correlation to short-term outcomes after the Fontan operation.
Davis EK, Ginde S, Stelter J, Frommelt P, Hill GD.
Congenit Heart Dis. 2019 Jun 24. doi: 10.1111/chd.12814. [Epub ahead of print]
PMID: 31231979
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11. Developmental origins for semilunar valve stenosis identified in mice harboring congenital heart disease-associated GATA4 mutation.
LaHaye S, Majumdar U, Yasuhara J, Koenig SN, Matos-Nieves A, Kumar R, Garg V.
Dis Model Mech. 2019 Jun 24;12(6). pii: dmm036764. doi: 10.1242/dmm.036764.
PMID: 31138536 Free PMC Article
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12. The role of microRNAs in congenital heart disease.
Nagy O, Baráth S, Ujfalusi A.
EJIFCC. 2019 Jun 24;30(2):165-178. eCollection 2019 Jun. Review.
PMID: 31263391 Free PMC Article
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13. Congenital absence of coronary ostia in a single/common coronary system.
Wein CE, Weinberg PM, Guleserian KJ.
Cardiol Young. 2019 Jun 21:1-3. doi: 10.1017/S1047951119001100. [Epub ahead of print]
PMID: 31221235
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14. Three year experience of a clinical cardiovascular genetics program for infants with congenital heart disease.
Geddes GC, Syverson E, Earing MG.
Congenit Heart Dis. 2019 Jun 21. doi: 10.1111/chd.12817. [Epub ahead of print]
PMID: 31222963
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15. Genes and mechanisms of heterotaxy: patients drive the search.
Sempou E, Khokha MK.
Curr Opin Genet Dev. 2019 Jun 21;56:34-40. doi: 10.1016/j.gde.2019.05.003. [Epub ahead of print] Review.
PMID: 31234044
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16. Neonatal Congenital Central Hypoventilation Syndrome: Why We Should not Sleep on it. Literature Review of Fourty-two Neonatal Onset Cases.
Bardanzellu F, Pintus MC, Fanos V, Marcialis MA.
Curr Pediatr Rev. 2019 Jun 20. doi: 10.2174/1573396315666190621103954. [Epub ahead of print]
PMID: 31223092
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17. Ten-year trend in prevalence and outcome of Down syndrome with congenital heart disease in a middle-income country.
Zahari N, Mat Bah MN, A Razak H, Thong MK.
Eur J Pediatr. 2019 Jun 20. doi: 10.1007/s00431-019-03403-x. [Epub ahead of print]
PMID: 31222391
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18. Abnormal Left-Hemispheric Sulcal Patterns Correlate with Neurodevelopmental Outcomes in Subjects with Single Ventricular Congenital Heart Disease.
Morton SU, Maleyeff L, Wypij D, Yun HJ, Newburger JW, Bellinger DC, Roberts AE, Rivkin MJ, Seidman JG, Seidman CE, Grant PE, Im K.
Cereb Cortex. 2019 Jun 19. pii: bhz101. doi: 10.1093/cercor/bhz101. [Epub ahead of print]
PMID: 31216004
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19. Fistula between the right pulmonary artery and the left atrium coexisting with a secundum-type atrial septal defect: An unusual case of cyanosis in a girl.
Zhang WM, Zhu HX, Maimaitiaili A, Ayibieke N, Ermek T, Liu J, Zhang ZG, Li SG.
Echocardiography. 2019 Jun 19. doi: 10.1111/echo.14414. [Epub ahead of print]
PMID: 31215690
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20. Ostium primum atrial septal defect with persistent left superior vena cava opening into unroofed coronary sinus-A rare entity.
Garg A, Agrawal D, Mishra D, Sharma GL.
Echocardiography. 2019 Jun 19. doi: 10.1111/echo.14408. [Epub ahead of print]
PMID: 31215689
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21. [Psychomotor and Cognitive Development and Quality of Life in Children and Adolescents with Congenital Heart Defect].
Hövels-Gürich H.
Klin Padiatr. 2019 Jun 19. doi: 10.1055/a-0942-1756. [Epub ahead of print] German.
PMID: 31216577
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22. Echocardiographic predictors of elevated left ventricular end diastolic pressure in adolescent and adult patients with repaired tetralogy of Fallot.
Lubert AM, Cotts TB, Zampi JD, Yu S, Norris MD.
Cardiol Young. 2019 Jun 18:1-5. doi: 10.1017/S1047951119001331. [Epub ahead of print]
PMID: 31208473
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23. Challenges in a unique presentation of congenital dengue with congenital heart disease.
Swaminathan A, Kirupanandhan S, Rathnavelu E.
BMJ Case Rep. 2019 Jun 17;12(6). pii: e228855. doi: 10.1136/bcr-2018-228855.
PMID: 31213434
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24. Role of Doppler echocardiography for assessing right ventricular cardiac output in patients with atrial septal defect.
Yogeswaran V, Kanade R, Mejia C, Fatola A, Kothapalli S, Najam M, Sandhyavenu H, Angirekula M, Osma K, Jessey M, Hagler D, Egbe AC.
Congenit Heart Dis. 2019 Jun 17. doi: 10.1111/chd.12813. [Epub ahead of print]
PMID: 31207173
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25. Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease.
Abassi H, Gavotto A, Picot MC, Bertet H, Matecki S, Guillaumont S, Moniotte S, Auquier P, Moreau J, Amedro P.
Int J Cardiol. 2019 Jun 15;285:86-92. doi: 10.1016/j.ijcard.2019.02.069. Epub 2019 Mar 1.
PMID: 30857849
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26. Diffusion tensor cardiovascular magnetic resonance.
Khalique Z, Pennell D.
Postgrad Med J. 2019 Jun 15. pii: postgradmedj-2019-136429. doi: 10.1136/postgradmedj-2019-136429. [Epub ahead of print] Review.
PMID: 31203209
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27. Genetic aetiologies should be considered in paediatric cases of acute heart failure presumed to be myocarditis.
Brown EE, McMilllan KN, Halushka MK, Ravekes WJ, Knight M, Crosson JE, Judge DP, Murphy AM.
Cardiol Young. 2019 Jun 14:1-5. doi: 10.1017/S1047951119001124. [Epub ahead of print]
PMID: 31198128
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28. Neuropsychological Impairment in Children With Class 1 Congenital Heart Disease.
Venchiarutti M, Vergine M, Zilli T, Sommariva G, Gortan AJ, Crescentini C, Urgesi C, Fabbro F, Cogo P.
Percept Mot Skills. 2019 Jun 14:31512519856766. doi: 10.1177/0031512519856766. [Epub ahead of print]
PMID: 31200623
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29. Risk of congenital anomalies near municipal waste incinerators in England and Scotland: Retrospective population-based cohort study.
Parkes B, Hansell AL, Ghosh RE, Douglas P, Fecht D, Wellesley D, Kurinczuk JJ, Rankin J, de Hoogh K, Fuller GW, Elliott P, Toledano MB.
Environ Int. 2019 Jun 13:104845. doi: 10.1016/j.envint.2019.05.039. [Epub ahead of print]
PMID: 31230843 Free Article
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30. Factors associated with exercise capacity in patients with a systemic right ventricle.
Gavotto A, Abassi H, Rola M, Serrand C, Picot MC, Iriart X, Thambo JB, Iserin L, Ladouceur M, Bredy C, Amedro P.
Int J Cardiol. 2019 Jun 13. pii: S0167-5273(19)31187-8. doi: 10.1016/j.ijcard.2019.06.030. [Epub ahead of print]
PMID: 31256996
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31. Associations between ambient air pollution and mortality from all causes, pneumonia, and congenital heart diseases among children aged under 5 years in Beijing, China: A population-based time series study.
Wang J, Cao H, Sun D, Qi Z, Guo C, Peng W, Sun Y, Xie Y, Liu X, Li B, Luo Y, Pan Y, Li Y, Zhang L.
Environ Res. 2019 Jun 11;176:108531. doi: 10.1016/j.envres.2019.108531. [Epub ahead of print]
PMID: 31226628
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32. Psychometric testing of the developmental care scale for neonates with congenital heart disease.
Arter S, Miller E, Bakas T, Cooper DS.
Cardiol Young. 2019 Jun 10:1-7. doi: 10.1017/S1047951119000337. [Epub ahead of print]
PMID: 31179950
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33. Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome.
Karaca-Altintas Y, Laux D, Gouton M, Bensemlali M, Roussin R, Hörer J, Raisky O, Bonnet D.
Eur J Cardiothorac Surg. 2019 Jun 10. pii: ezz167. doi: 10.1093/ejcts/ezz167. [Epub ahead of print]
PMID: 31180449
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34. [Genetic analysis of a child with 13q deletion syndrome featuring congenital heart disease].
Shen N, Gou R, Yu H, Gao X, Pang H, Liu Y, Gai Z.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2019 Jun 10;36(6):620-623. doi: 10.3760/cma.j.issn.1003-9406.2019.06.023. Chinese.
PMID: 31055821
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35. Orodispersible minitablets of enalapril for use in children with heart failure (LENA): Rationale and protocol for a multicentre pharmacokinetic bridging study and follow-up safety study.
Bajcetic M, de Wildt SN, Dalinghaus M, Breitkreutz J, Klingmann I, Lagler FB, Keatley-Clarke A, Breur JM, Male C, Jovanovic I, Szatmári A, Ablonczy L, Burckhardt BB, Cawello W, Kleine K, Obarcanin E, Spatenkova L, Swoboda V, van der Meulen M, Wagner P, Walsh J, Läer S.
Contemp Clin Trials Commun. 2019 Jun 8;15:100393. doi: 10.1016/j.conctc.2019.100393. eCollection 2019 Sep.
PMID: 31249901 Free PMC Article
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36. Comparative Cardiorespiratory Fitness in Children: Racial Disparity May Begin Early in Childhood.
Bansal N, Mahadin DR, Smith R, French M, Karpawich PP, Aggarwal S.
Pediatr Cardiol. 2019 Jun 8. doi: 10.1007/s00246-019-02129-9. [Epub ahead of print]
PMID: 31177302
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37. Plastic bronchitis: A case report.
Grizales CL, González LM, Castrillon MA, Sua LF, Lores J, Aguirre M, Fernández-Trujillo L.
Respir Med Case Rep. 2019 Jun 8;28:100876. doi: 10.1016/j.rmcr.2019.100876. eCollection 2019.
PMID: 31245273 Free PMC Article
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38. Cross-sectional imaging of congenital pulmonary artery anomalies.
Zucker EJ.
Int J Cardiovasc Imaging. 2019 Jun 7. doi: 10.1007/s10554-019-01643-4. [Epub ahead of print]
PMID: 31175525
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39. Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium).
Oster ME, McCracken C, Kiener A, Aylward B, Cory M, Hunting J, Kochilas LK.
Am J Cardiol. 2019 Jun 6. pii: S0002-9149(19)30626-5. doi: 10.1016/j.amjcard.2019.05.047. [Epub ahead of print]
PMID: 31272703
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40. Germline-Activating RRAS2 Mutations Cause Noonan Syndrome.
Niihori T, Nagai K, Fujita A, Ohashi H, Okamoto N, Okada S, Harada A, Kihara H, Arbogast T, Funayama R, Shirota M, Nakayama K, Abe T, Inoue SI, Tsai IC, Matsumoto N, Davis EE, Katsanis N, Aoki Y.
Am J Hum Genet. 2019 Jun 6;104(6):1233-1240. doi: 10.1016/j.ajhg.2019.04.014. Epub 2019 May 23.
PMID: 31130285
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Select item 31169113
41. The effect of congenital diaphragmatic hernia on the development of left-sided heart structures.
Coffman ZJ, McGahren ED, Vergales BD, Saunders CH, Vergales JE.
Cardiol Young. 2019 Jun 6:1-6. doi: 10.1017/S1047951119000891. [Epub ahead of print]
PMID: 31169099
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Select item 31176777
42. Galectin-3 aggravates pulmonary arterial hypertension via immunomodulation in congenital heart disease.
Shen Q, Chen W, Liu J, Liang Q.
Life Sci. 2019 Jun 6:116546. doi: 10.1016/j.lfs.2019.116546. [Epub ahead of print]
PMID: 31176777
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Select item 31169342
43. The acute effects of 30 mg vs 60 mg of intravenous Fasudil on patients with congenital heart defects and severe pulmonary arterial hypertension.
Ruan H, Zhang Y, Liu R, Yang X.
Congenit Heart Dis. 2019 Jun 5. doi: 10.1111/chd.12764. [Epub ahead of print]
PMID: 31166081
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Select item 31166073
44. Long-term cardiovascular outcome of Williams syndrome.
Cha SG, Song MK, Lee SY, Kim GB, Kwak JG, Kim WH, Bae EJ.
Congenit Heart Dis. 2019 Jun 5. doi: 10.1111/chd.12810. [Epub ahead of print]
PMID: 31166070
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Select item 31168581
45. Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry.
Ong MS, Abman S, Austin ED, Feinstein JA, Hopper RK, Krishnan US, Mullen MP, Natter MD, Raj JU, Rosenzweig EB, Mandl KD; Pediatric Pulmonary Hypertension Network and National Heart, Lung, and Blood Institute Pediatric Pulmonary Vascular Disease Outcomes Bioinformatics Clinical Coordinating Center Investigators.
J Pediatr. 2019 Jun 5. pii: S0022-3476(19)30529-3. doi: 10.1016/j.jpeds.2019.04.046. [Epub ahead of print]
PMID: 31176455
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Select item 31251381
46. Characterisation of computed tomography angiography findings in paediatric patients with heterotaxy.
Ugas Charcape CF, Alpaca Rodriguez LR, Matos Rojas IA, Lazarte Rantes CI, Valdez Quintana M, Katekaru Tokeshi DA, Epelman M.
Pediatr Radiol. 2019 Jun 5. doi: 10.1007/s00247-019-04434-0. [Epub ahead of print]
PMID: 31165901
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Select item 31166962
47. Potential risk factors for Ebstein anomaly, National Birth Defects Prevention Study, 1997-2011.
Downing KF, Riehle-Colarusso T, Gilboa SM, Lin AE, Oster ME, Tinker SC, Farr SL; National Birth Defects Prevention Study.
Cardiol Young. 2019 Jun 4:1-9. doi: 10.1017/S1047951119000970. [Epub ahead of print]
PMID: 31159903
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Select item 31159896
48. A case of Coffin-Siris syndrome with severe congenital heart disease and a novel SMARCA4 variant.
Dsouza NR, Zimmermann MT, Geddes GC.
Cold Spring Harb Mol Case Stud. 2019 Jun 3;5(3). pii: a003962. doi: 10.1101/mcs.a003962. Print 2019 Jun.
PMID: 31160358 Free PMC Article
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Select item 31245011
49. Human Milk and Infants With Congenital Heart Disease: A Summary of Current Literature Supporting the Provision of Human Milk and Breastfeeding.
Davis JA, Spatz DL.
Adv Neonatal Care. 2019 Jun;19(3):212-218. doi: 10.1097/ANC.0000000000000582.
PMID: 30694819
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Select item 30954207
50. A novel method optimizing the normalization of cardiac parameters in small animal models: the importance of dimensional indexing.
Hagdorn QAJ, Bossers GPL, Koop AC, Piek A, Eijgenraam TR, van der Feen DE, Silljé HHW, de Boer RA, Berger RMF.
Am J Physiol Heart Circ Physiol. 2019 Jun 1;316(6):H1552-H1557. doi: 10.1152/ajpheart.00182.2019. Epub 2019 Apr 12.
PMID: 30978120
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Select item 30838867
51. Selected acculturation factors and birth defects in the National Birth Defects Prevention Study, 1997-2011.
Hoyt AT, Shumate CJ, Canfield MA, Le M, Ramadhani T, Scheuerle AE; National Birth Defects Prevention Study.
Birth Defects Res. 2019 Jun 1;111(10):598-612. doi: 10.1002/bdr2.1494. Epub 2019 Apr 25.
PMID: 31021057
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Select item 30576052
52. Prostaglandin E1 overdose in a term neonate with congenital heart disease.
Gorodetsky RM, Toole BM, Schult RF, Wiegand TJ.
Clin Toxicol (Phila). 2019 Jun;57(6):420-421. doi: 10.1080/15563650.2018.1533137. Epub 2018 Nov 17. No abstract available.
PMID: 30451018
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Select item 30891780
53. Loss of function of Kmt2d, a gene mutated in Kabuki syndrome, affects heart development in Xenopus laevis.
Schwenty-Lara J, Nürnberger A, Borchers A.
Dev Dyn. 2019 Jun;248(6):465-476. doi: 10.1002/dvdy.39. Epub 2019 May 1.
PMID: 30980591
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Select item 31013439
54. Intronic Polymorphisms in Gene of Second Heart Field as Risk Factors for Human Congenital Heart Disease in a Chinese Population.
Wang E, Nie Y, Fan X, Zheng Z, Hu S.
DNA Cell Biol. 2019 Jun;38(6):521-531. doi: 10.1089/dna.2018.4254. Epub 2019 Apr 23.
PMID: 31013439
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Select item 31116466
55. Quantification of pulmonary/systemic shunt ratio by single-acquisition phase-contrast cardiovascular magnetic resonance.
Dunn TS 2nd, Patel P, Abazid B, Nagaraj HM, Desai RV, Gupta H, Lloyd SG.
Echocardiography. 2019 Jun;36(6):1181-1190. doi: 10.1111/echo.14358. Epub 2019 May 13.
PMID: 31087463
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Select item 31025775
56. A rare case of anomalous origin of the left anterior descending artery from the pulmonary artery.
Zhang Y, Wang B, Li Y, Xie M.
Echocardiography. 2019 Jun;36(6):1208-1210. doi: 10.1111/echo.14355. Epub 2019 Apr 22.
PMID: 31012154
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Select item 30339206
57. TARP syndrome: Long-term survival, anatomic patterns of congenital heart defects, differential diagnosis and pathogenetic considerations.
Niceta M, Barresi S, Pantaleoni F, Capolino R, Dentici ML, Ciolfi A, Pizzi S, Bartuli A, Dallapiccola B, Tartaglia M, Digilio MC.
Eur J Med Genet. 2019 Jun;62(6):103534. doi: 10.1016/j.ejmg.2018.09.001. Epub 2018 Sep 3. Review.
PMID: 30189253 Free Article
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58. Analysis of a nanoparticle‑enriched fraction of plasma reveals miRNA candidates for Down syndrome pathogenesis.
Salvi A, Vezzoli M, Busatto S, Paolini L, Faranda T, Abeni E, Caracausi M, Antonaros F, Piovesan A, Locatelli C, Cocchi G, Alvisi G, De Petro G, Ricotta D, Bergese P, Radeghieri A.
Int J Mol Med. 2019 Jun;43(6):2303-2318. doi: 10.3892/ijmm.2019.4158. Epub 2019 Apr 9. Erratum in: Int J Mol Med. 2019 Aug;44(2):768.
PMID: 31017260 Free PMC Article
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Select item 30726938
59. The morphologically right and left ventricles cannot be distinguished by their coronary arterial pattern.
Hosseinpour AR, Juvin-Bouvier C, Adsuar-Gómez A, González-Calle A, Borrego-Domínguez JM, Ordoñez-Fernández A, McCarthy KP, Ho SY.
Interact Cardiovasc Thorac Surg. 2019 Jun 1;28(6):968-971. doi: 10.1093/icvts/ivy357.
PMID: 30668872
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Select item 30649430
60. Initial experience with cinematic rendering for the visualization of extracardiac anatomy in complex congenital heart defects†.
Röschl F, Purbojo A, Rüffer A, Cesnjevar R, Dittrich S, Glöckler M.
Interact Cardiovasc Thorac Surg. 2019 Jun 1;28(6):916-921. doi: 10.1093/icvts/ivy348.
PMID: 30649430
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Select item 31025765
61. The efficiency of systemic-to-pulmonary shunts in older children with hypoplastic pulmonary arteries.
Zhang H, Fan X, Su J, Liu Y, Zhao L, Li G.
J Card Surg. 2019 Jun;34(6):463-467. doi: 10.1111/jocs.14063. Epub 2019 Apr 26.
PMID: 31025765
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Select item 31012137
62. Hypoxia-induced ARHGAP26 deficiency inhibits the proliferation and migration of human ductus arteriosus smooth muscle cell through activating RhoA-ROCK-PTEN pathway.
Li M, Ye L, Ye X, Wang S, Zhang H, Liu J, Hong H.
J Cell Biochem. 2019 Jun;120(6):10106-10117. doi: 10.1002/jcb.28294. Epub 2018 Dec 28.
PMID: 30592323
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Select item 31204705
63. Minor alleles of genetic variants in second heart field increase the risk of hypoplastic right heart syndrome.
Wang E, Nie Y, Fan X, Zheng Z, Gu H, Zhang H, Hu S.
J Genet. 2019 Jun;98(2). pii: 11.
PMID: 31204705 Free Article
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Select item 31204702
64. Clinical presentation and genetic profiles of Chinese patients with velocardiofacial syndrome in a large referral centre.
Wu D, Chen Y, Chen Q, Wang G, Xu X, Peng A, Hao J, He J, Huang L, Dai J.
J Genet. 2019 Jun;98(2). pii: 8.
PMID: 31204702 Free Article
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Select item 30733156
65. Risk factors for in-hospital and follow-up mortality after childhood arterial ischemic stroke.
Lopez-Espejo M, Hernandez-Chavez M, Huete I.
J Neurol. 2019 Jun;266(6):1526-1532. doi: 10.1007/s00415-019-09293-1. Epub 2019 Mar 26.
PMID: 30915544
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Select item 30636080
66. Risk factors for in-hospital and follow-up mortality after childhood arterial ischemic stroke.
Lopez-Espejo M, Hernandez-Chavez M, Huete I.
J Neurol. 2019 Jun;266(6):1526-1532. doi: 10.1007/s00415-019-09293-1. Epub 2019 Mar 26.
PMID: 30915544
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Select item 30636080
67. Native aortic coarctation presenting as prolonged pyrexia in a teenager with 22q11.2 deletion.
Karatza AA, Gkentzi D, Kostopoulou E, Rammos S.
J Paediatr Child Health. 2019 Jun;55(6):711-714. doi: 10.1111/jpc.14341. Epub 2019 Jan 12. No abstract available.
PMID: 30636080
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Select item 31174733
68. Doppler-derived echocardiographic evidence of pulmonary hypertension in cats with left-sided congestive heart failure.
Vezzosi T, Schober KE.
J Vet Cardiol. 2019 Jun;23:58-68. doi: 10.1016/j.jvc.2019.01.007. Epub 2019 Feb 27.
PMID: 31174730
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Select item 31174723
69. Cor triatriatum dexter in 17 dogs.
Nadolny KE, Kellihan HB, Scansen BA, Tjostheim SS, Grint KA, Forrest LJ, Stepien RL.
J Vet Cardiol. 2019 Jun;23:129-141. doi: 10.1016/j.jvc.2019.03.002. Epub 2019 Apr 30.
PMID: 31174723
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Select item 31174721
70. The Future of Cardiovascular Computed Tomography: Advanced Analytics and Clinical Insights.
Nicol ED, Norgaard BL, Blanke P, Ahmadi A, Weir-McCall J, Horvat PM, Han K, Bax JJ, Leipsic J.
JACC Cardiovasc Imaging. 2019 Jun;12(6):1058-1072. doi: 10.1016/j.jcmg.2018.11.037. Review.
PMID: 31171259
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71. Accuracy of pulse oximeters at low oxygen saturations in children with congenital cyanotic heart disease: An observational study.
Kim EH, Lee JH, Song IK, Kim HS, Jang YE, Yoo S, Kim JT.
Paediatr Anaesth. 2019 Jun;29(6):597-603. doi: 10.1111/pan.13642. Epub 2019 Apr 29.
PMID: 30938906
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72. Concurrent Use of Calcium Chloride and Arginine Vasopressin Infusions in Pediatric Patients with Acute Cardiocirculatory Failure.
Karki KB, Towbin JA, Harrell C, Tansey J, Krebs J, Bigelow W, Saini A, Tadphale SD.
Pediatr Cardiol. 2019 Jun;40(5):1046-1056. doi: 10.1007/s00246-019-02114-2. Epub 2019 May 7.
PMID: 31065757
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Select item 31049646
73. Oxygen saturations and neurodevelopmental outcomes in single ventricle heart disease.
Wolfe KR, Brinton J, Di Maria MV, Meier M, Liptzin DR.
Pediatr Pulmonol. 2019 Jun;54(6):922-927. doi: 10.1002/ppul.24275. Epub 2019 Mar 28.
PMID: 30919581
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Select item 31026503
74. Echocardiographic screening of 4107 Nigerian school children for rheumatic heart disease.
Ekure EN, Amadi C, Sokunbi O, Kalu N, Olusegun-Joseph A, Kushimo O, Hassan O, Ikebudu D, Onyia S, Onwudiwe C, Nwankwo V, Akinwunmi R, Awusa F, Akere Z, Dele-Salawu O, Ajayi E, Ale O, Muoneke D, Muenke M, Kruszka P, Beaton A, Sable C, Adeyemo A.
Trop Med Int Health. 2019 Jun;24(6):757-765. doi: 10.1111/tmi.13235. Epub 2019 Apr 8.
PMID: 30938017
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75. [Asymmetric crying facies and vocal cord paralysis accompanied by congenital heart disease in an infant].
Wei HL, Piao MH, Zhang J, Liu L, Chang YM.
Zhongguo Dang Dai Er Ke Za Zhi. 2019 Jun;21(6):585-588. Chinese.
PMID: 31208514
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76. An unusual case of concordant ventriculoarterial connections, subpulmonary infundibulum, and parallel arterial trunks: a diagnostic challenge.
Nelson JA, Soriano BD, Buddhe S.
Cardiol Young. 2019 Jun 28:1-3. doi: 10.1017/S1047951119000684. [Epub ahead of print]
PMID: 31250773
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Select item 31250555
77. Can microsomal RNA be a biomarker in pulmonary hypertension secondary to bronchopulmonary dysplasia?
Guler E, Narin N, Pamukcu O, Taheri S, Tufan E, Guler Y, Tuncay A, Baykan A.
J Matern Fetal Neonatal Med. 2019 Jun 27:1-151. doi: 10.1080/14767058.2019.1638107. [Epub ahead of print]
PMID: 31248305
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Select item 31245936
78. Changes in left and right ventricular two-dimensional echocardiographic speckle-tracking indices in pediatric LVAD population: A retrospective clinical study.
Iacobelli R, Di Molfetta A, Cobianchi Bellisari F, Toscano A, Filippelli S, Di Chiara L, Pasquini L, Iorio FS, Amodeo A.
Int J Artif Organs. 2019 Jun 26:391398819857446. doi: 10.1177/0391398819857446. [Epub ahead of print]
PMID: 31238772
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Select item 31246738
79. An Update on Pediatric Cardiomyopathy.
Choudhry S, Puri K, Denfield SW.
Curr Treat Options Cardiovasc Med. 2019 Jun 25;21(8):36. doi: 10.1007/s11936-019-0739-y. Review.
PMID: 31236771
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Select item 31237036
80. A case report on endarteritis in a child with coarctation of aorta.
Gnanam D, Bartelds B, van Leeuwen WJ, Frohn-Mulder IM, Koopman LP.
Echocardiography. 2019 Jun 25. doi: 10.1111/echo.14418. [Epub ahead of print]
PMID: 31237036
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Select item 31250742
81. A case report on endarteritis in a child with coarctation of aorta.
Gnanam D, Bartelds B, van Leeuwen WJ, Frohn-Mulder IM, Koopman LP.
Echocardiography. 2019 Jun 25. doi: 10.1111/echo.14418. [Epub ahead of print]
PMID: 31237036
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Select item 31250742
82. The outcome of patients with functional single ventricular heart after pacemaker implantation-what makes it poor and what can we do?
Kodama Y, Kuraoka A, Ishikawa Y, Nakamura M, Ushinohama H, Sagawa K, Umemoto S, Hashimoto T, Sakamoto I, Ohtani K, Ide T, Tsutsui H, Ishikawa S.
Heart Rhythm. 2019 Jun 25. pii: S1547-5271(19)30576-4. doi: 10.1016/j.hrthm.2019.06.019. [Epub ahead of print]
PMID: 31252085
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Select item 31240821
83. Improved Left Ventricular Strain and Dyssynchrony After Pulmonary Artery Banding in an Infant with End-Stage Dilated Cardiomyopathy: Insights from Three-Dimensional Speckle Tracking.
Liu YH, Chen YS, Lin MT, Chen CA.
Pediatr Cardiol. 2019 Jun 25. doi: 10.1007/s00246-019-02138-8. [Epub ahead of print]
PMID: 31240369
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Select item 31238552
84. Isolated retroaortic innominate vein and right aortic arch: a case report and review of literature.
Kohli U.
Cardiol Young. 2019 Jun 21:1-3. doi: 10.1017/S1047951119001380. [Epub ahead of print]
PMID: 31221230
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Select item 31222948
85. Analysis of enriched rare variants in JPH2-encoded junctophilin-2 among Greater Middle Eastern individuals reveals a novel homozygous variant associated with neonatal dilated cardiomyopathy.
Jones EG, Mazaheri N, Maroofian R, Zamani M, Seifi T, Sedaghat A, Shariati G, Jamshidi Y, Allen HD, Wehrens XHT, Galehdari H, Landstrom AP.
Sci Rep. 2019 Jun 21;9(1):9038. doi: 10.1038/s41598-019-44987-6.
PMID: 31227780 Free PMC Article
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Select item 31233784
86. Vascular endotheEvaluation of long-term cardiac side effects of anthracycline chemotherapy by conventional and non-conventional echocardiographic methods in childhood cancer survivors.
Yoldaş T, Yeşil Ş, Karademir S, Şahin G, Arman Örün U, Doğan V, Özgür S.
Cardiol Young. 2019 Jun 20:1-6. doi: 10.1017/S1047951119001094. [Epub ahead of print]
PMID: 31218970
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Select item 31222473
lial growth factor polymorphism rs2010963 status does not affect patent ductus arteriosus incidence or cyclooxygenase inhibitor treatment success in preterm infants.
Sallmon H, Aydin T, Hort S, Kubinski A, Bode C, Klippstein T, Endesfelder S, Bührer C, Koehne P.
Cardiol Young. 2019 Jun 20:1-5. doi: 10.1017/S1047951119001033. [Epub ahead of print]
PMID: 31218973
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Select item 31218970
87. Evaluation of long-term cardiac side effects of anthracycline chemotherapy by conventional and non-conventional echocardiographic methods in childhood cancer survivors.
Yoldaş T, Yeşil Ş, Karademir S, Şahin G, Arman Örün U, Doğan V, Özgür S.
Cardiol Young. 2019 Jun 20:1-6. doi: 10.1017/S1047951119001094. [Epub ahead of print]
PMID: 31218970
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Select item 31222473
88. The effects of age at correction of aortic coarctation and recurrent obstruction on adolescent patients: MRI evaluation of wall shear stress and pulse wave velocity.
Juffermans JF, Nederend I, van den Boogaard PJ, Ten Harkel ADJ, Hazekamp MG, Lamb HJ, Roest AAW, Westenberg JJM.
Eur Radiol Exp. 2019 Jun 20;3(1):24. doi: 10.1186/s41747-019-0102-9.
PMID: 31222473 Free PMC Article
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Select item 31229680
89. Native valve endocarditis due to Candida albicans in two children: Two new case reports.
Guner G, Bal ZS, Dogan E, Umit Z, Levent E, Hilmioglu Polat S, Ozkinay F, Kurugol Z.
Echocardiography. 2019 Jun 19. doi: 10.1111/echo.14406. [Epub ahead of print]
PMID: 31215692
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90. Development and Evaluation of a New Chest Compression Technique for Cardiopulmonary Resuscitation in Infants.
Yang D, Kim KH, Oh JH, Son S, Cho J, Seo KM.
Pediatr Cardiol. 2019 Jun 19. doi: 10.1007/s00246-019-02135-x. [Epub ahead of print]
PMID: 31218374
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Select item 31218373
91. Saline Contrast Transesophageal Echocardiography in Fontan Patients: Assessment of the Presence, Type, and Size of Right to Left Shunts.
Zaidi SJ, Adhikari RR, Patel DR, Cui VW, Javois AJ, Roberson DA.
Pediatr Cardiol. 2019 Jun 19. doi: 10.1007/s00246-019-02132-0. [Epub ahead of print]
PMID: 31218373
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Select item 31218372
92. Echocardiographic predictors of elevated left ventricular end diastolic pressure in adolescent and adult patients with repaired tetralogy of Fallot.
Lubert AM, Cotts TB, Zampi JD, Yu S, Norris MD.
Cardiol Young. 2019 Jun 18:1-5. doi: 10.1017/S1047951119001331. [Epub ahead of print]
PMID: 31208473
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93. Catecholamine-Induced Myocarditis in a Child with Pheochromocytoma
Uçaktürk SA, Mengen E, Azak E, Çetin İİ, Kocaay P, Şenel E.
J Clin Res Pediatr Endocrinol. 2019 Jun 18. doi: 10.4274/jcrpe.galenos.2019.0045. [Epub ahead of print]
PMID: 31208160 Free Article
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Select item 31227283
94. Temporal changes in left ventricular strain with the development of rejection in paediatric heart transplant recipients.
Godown J, McEachern WA, Dodd DA, Stanley M, Havens C, Xu M, Slaughter JC, Bearl DW, Soslow JH.
Cardiol Young. 2019 Jun 17:1-6. doi: 10.1017/S1047951119001185. [Epub ahead of print]
PMID: 31204638
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Select item 31204631
95. Biatrial drainage of right superior caval vein with preferential streaming into the left atrium.
Abu-Anza O, Ashwath R.
Cardiol Young. 2019 Jun 17:1-3. doi: 10.1017/S1047951119001276. [Epub ahead of print]
PMID: 31204631
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Select item 31204627
96. Lessons learned in the use of clinical registry data in a multi-centre prospective study: the Pediatric Heart Network Residual Lesion Score Study.
Prospero CJ, Trachtenberg FL, Pemberton VL, Pasquali SK, Anderson BR, Ash KE, Bainton J, Dunbar-Masterson C, Graham EM, Hamstra MS, Hollenbeck-Pringle D, Jacobs JP, Jacobs ML, John R, Lambert LM, Oster ME, Swan E, Waldron A, Nathan M; Pediatric Heart Network Investigators.
Cardiol Young. 2019 Jun 17:1-9. doi: 10.1017/S1047951119001148. [Epub ahead of print]
PMID: 31204627
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Select item 31079504
97. Early Impairment Left Ventricular Mechanics in Children With Mitral Valve Prolapse.
Çelik SF.
Am J Cardiol. 2019 Jun 15;123(12):1992-1998. doi: 10.1016/j.amjcard.2019.03.009. Epub 2019 Mar 18.
PMID: 30961911
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Select item 30857846
98. Nomograms of pulsed Doppler velocities, times, and velocity time integrals for semilunar valves and great arteries in healthy Caucasian children.
Cantinotti M, Giordano R, Scalese M, Franchi E, Assanta N, Molinaro S, Marchese P, Paterni M, Iervasi G, Kutty S, Koestenberger M.
Int J Cardiol. 2019 Jun 15;285:133-139. doi: 10.1016/j.ijcard.2019.03.001. Epub 2019 Mar 3. No abstract available.
PMID: 30857846
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Select item 30857845
99. Creating three dimensional models of the right ventricular outflow tract: influence of contrast, sequence, operator, and threshold.
Burkhardt BEU, Brown NK, Carberry JE, Velasco Forte MN, Byrne N, Greil G, Hussain T, Tandon A.
Int J Cardiovasc Imaging. 2019 Jun 15. doi: 10.1007/s10554-019-01646-1. [Epub ahead of print]
PMID: 31203535
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Select item 31203534
100. Genetic aetiologies should be considered in paediatric cases of acute heart failure presumed to be myocarditis.
Brown EE, McMilllan KN, Halushka MK, Ravekes WJ, Knight M, Crosson JE, Judge DP, Murphy AM.
Cardiol Young. 2019 Jun 14:1-5. doi: 10.1017/S1047951119001124. [Epub ahead of print]
PMID: 31198128
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Select item 31198121
101. Living the heart in three dimensions: applications of 3D printing in CHD.
Forte MNV, Hussain T, Roest A, Gomez G, Jongbloed M, Simpson J, Pushparajah K, Byrne N, Valverde I.
Cardiol Young. 2019 Jun 14:1-11. doi: 10.1017/S1047951119000398. [Epub ahead of print]
PMID: 31198120
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Select item 31198119
102. Differential expression profiles and functional analysis of circular RNAs in children with fulminant myocarditis.
Zhang L, Han B, Wang J, Liu Q, Kong Y, Jiang D, Jia H.
Epigenomics. 2019 Jun 14. doi: 10.2217/epi-2019-0101. [Epub ahead of print]
PMID: 31198064 Free Article
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Select item 31199069
103. Variation in the management of Kawasaki disease.
Dionne A, Burgner D, De Ferranti S, Singh-Grewal D, Newburger J, Dahdah N.
Arch Dis Child. 2019 Jun 13. pii: archdischild-2019-317191. doi: 10.1136/archdischild-2019-317191. [Epub ahead of print]
PMID: 31196912
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Select item 31189430
104. Do Calcium and Potassium Levels Influence Ductal Patency in Preterm Infants?
Cakir U, Tayman C, Buyuktiryaki M, Unsal H, Ozer Bekmez B.
Am J Perinatol. 2019 Jun 5. doi: 10.1055/s-0039-1692389. [Epub ahead of print]
PMID: 31167236
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Select item 31167650
105. Racial and Ethnic Differences in Pediatric Pulmonary Hypertension: An Analysis of the Pediatric Pulmonary Hypertension Network Registry.
Ong MS, Abman S, Austin ED, Feinstein JA, Hopper RK, Krishnan US, Mullen MP, Natter MD, Raj JU, Rosenzweig EB, Mandl KD; Pediatric Pulmonary Hypertension Network and National Heart, Lung, and Blood Institute Pediatric Pulmonary Vascular Disease Outcomes Bioinformatics Clinical Coordinating Center Investigators.
J Pediatr. 2019 Jun 5. pii: S0022-3476(19)30529-3. doi: 10.1016/j.jpeds.2019.04.046. [Epub ahead of print]
PMID: 31176455
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Select item 31165902
106. Comparison Between Nomograms Used to Define Pediatric Aortic Arch Hypoplasia: Retrospective Evaluation Among Patients Less Than 1 Year Old with Coarctation of the Aorta.
Şişli E, Kalın S, Tuncer ON, Ayık MF, Alper H, Levent RE, Şahin H, Atay Y.
Pediatr Cardiol. 2019 Jun 5. doi: 10.1007/s00246-019-02130-2. [Epub ahead of print]
PMID: 31165902
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Select item 30020660
107. Comparison Between Nomograms Used to Define Pediatric Aortic Arch Hypoplasia: Retrospective Evaluation Among Patients Less Than 1 Year Old with Coarctation of the Aorta.
Şişli E, Kalın S, Tuncer ON, Ayık MF, Alper H, Levent RE, Şahin H, Atay Y.
Pediatr Cardiol. 2019 Jun 5. doi: 10.1007/s00246-019-02130-2. [Epub ahead of print]
PMID: 31165902
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Select item 30020660
108. A rare vascular ring of right aortic arch and aberrant left subclavian artery in association with bilateral ductus arteriosus.
Ma B, Wu L, Zhang W.
Ultrasound Obstet Gynecol. 2019 Jun 4. doi: 10.1002/uog.20365. [Epub ahead of print] No abstract available.
PMID: 31162848
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109. Interrelationship Between Hemodynamics, Brain Volumes, and Outcome in Hypoplastic Left Heart Syndrome.
Reich B, Heye KN, O’Gorman Tuura R, Beck I, Wetterling K, Hahn A, Aktintürk H, Schranz D, Jux C, Kretschmar O, Hübler M, Latal B, Knirsch W.
Ann Thorac Surg. 2019 Jun;107(6):1838-1844. doi: 10.1016/j.athoracsur.2018.12.012. Epub 2019 Jan 9.
PMID: 30639363
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110. Subcutaneous and Intravenous Treprostinil Pharmacokinetics in Children With Pulmonary Vascular Disease.
Hall K, Ogawa M, Sakarovitch C, Hopper RK, Adamson GT, Hanna B, Ivy DD, Miller-Reed K, Yung D, McCarthy E, Siehr-Handler SL, Feinstein JA.
J Cardiovasc Pharmacol. 2019 Jun;73(6):383-393. doi: 10.1097/FJC.0000000000000674.
PMID: 31162247
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111. Increased Arterial Stiffness Adversely Affects Left Ventricular Mechanics in Patients With Pediatric Takayasu Arteritis From a Toronto Cohort.
Grotenhuis HB, Aeschlimann FA, Hui W, Slorach C, Yeung RSM, Benseler SM, Bradley TJ, Grosse-Wortmann L.
J Clin Rheumatol. 2019 Jun;25(4):171-175. doi: 10.1097/RHU.0000000000000824.
PMID: 29782425
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112. Early-Onset Marfan Syndrome: A Case Series.
Ardhanari M, Barbouth D, Swaminathan S.
J Pediatr Genet. 2019 Jun;8(2):86-90. doi: 10.1055/s-0038-1675338. Epub 2018 Nov 2.
PMID: 31061752
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113. Hypotensive Response to IV Acetaminophen in Pediatric Cardiac Patients.
Achuff BJ, Moffett BS, Acosta S, Lasa JJ, Checchia PA, Rusin CG.
Pediatr Crit Care Med. 2019 Jun;20(6):527-533. doi: 10.1097/PCC.0000000000001880.
PMID: 30676493
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114. Human genotyping and an experimental model reveal NPR-C as a possible contributor to morbidity in coarctation of the aorta.
LaDisa JF Jr, Tomita-Mitchell A, Stamm K, Bazan K, Mahnke DK, Goetsch MA, Wegter BJ, Gerringer JW, Repp K, Palygin O, Zietara AP, Krolikowski MM, Eddinger TJ, Alli AA, Mitchell ME.
Physiol Genomics. 2019 Jun 1;51(6):177-185. doi: 10.1152/physiolgenomics.00049.2018. Epub 2019 Apr 19.
PMID: 31002586
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115. Retrospective study of the course, treatment and long-term follow-up of Kawasaki disease: a single-center experience from Poland.
Stasiak A, Smolewska E.
Rheumatol Int. 2019 Jun;39(6):1069-1076. doi: 10.1007/s00296-019-04286-9. Epub 2019 Mar 22.
PMID: 30903369
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ACHD Featured Articles in association with ISACHD
ACHD Featured Manuscripts of June 2019 (sponsored by ISACHD)
Mechanism and Risk Factors for Death in Adults With Tetralogy of Fallot.
Egbe AC, Kothapalli S, Borlaug BA, Ammash NM, Najam M, Bajwa N, Tarek K, Matthew J, Connolly HM.
Am J Cardiol. 2019 Jun 7. pii: S0002-9149(19)30627-7. doi: 10.1016/j.amjcard.2019.05.048. [Epub ahead of print]
PMID: 31272701
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Take Home Points:
• Annual event rate of death or cardiac transplant was 0.9 % in a Mayo Clinic cohort of 465 TOF patients.
• Six independent risk factors associated with death/transplant were age > 42 years, atrial fibrillation, moderate QRS fragmentation, LVEF < 50 %, RVEDP > 16 mmHg, and LVEDP > 16 mmHg.
• Nearly two-fold increased risk of death or transplant per unit increase in number of risk factors.
Commentary from Dr. Timothy Roberts (Melbourne, Australia), section editor of ACHD Journal Watch: The aim of this retrospective analysis from the Mayo Adult Congenital Heart Disease database of 465 adults with repaired TOF was to identify risk factors for death and/or cardiac transplantation.
Patients’ electronic medical records were interrogated to collate data of patient demographics, co-morbidities, medications, heart rhythm, echocardiograms, cardiopulmonary exercise testing, cardiac magnetic resonance imaging, and cardiac catheterization. Data from the first visit and test/procedure was used as the baseline variable. Full datasets were not available for all patients, with a single conditional imputation method used to correct for missing data in the multivariate analysis.
Baseline age was 37- 14 years, 48 % men, and mean age at time of TOF repair was 5 (3 – 10) years. A transannular patch repair was performed in 37 % of the cohort.
The endpoint of death and/or transplant occurred in 57 (12%) patients during a follow-up of 13.6 8.2 years, giving an event rate of 0.9 % per year. Mean age at death (54/57 patients with combined endpoint) was 57 15 years; key causes of death included:
– congestive cardiac failure 23 (43%)
– arrhythmic/sudden cardiac death in 14 (26%)
– malignancy in 5 (9%),
– sepsis/multisystem organ failure in 4 (7%)
– postoperative death following cardiac surgery in 3 (6%)
Three underwent cardiac transplantation at a mean age of 54 5 years, with one dying 13 months later.
A multivariate risk model (Table 3, below), incorporating all statistically significant univariate correlates, identified the following risk factors:
1. age >42 years (HR 1.86, 95% CI 1.24 to 2.03),
2. atrial fibrillation (HR 1.84, 95% CI 1.06 to 3.17),
3. ≥moderate QRS fragmentation (HR 1.92, 95% CI 1.47 to 2.81),
4. left ventricular ejection fraction <50% (HR 1.39, 95% CI 1.08 to 2.31),
5. right ventricular end-diastolic pressure >16 mm Hg (HR 1.41, 95% CI 1.02 to 1.22), and
6. left ventricle end-diastolic pressure >16 mm Hg (HR 1.32, 95% CI 1.11 to 1.89).
Each independent risk factor was assigned one point; of the original 465 patients, 208 (45 %) had no risk factors (0 points), 191 (41 %) were low risk (1-2 points), 59 (13 %) were of intermediate risk (3-4 points), and 7 (1.5 %) were at highest risk (5 or 6 points). Using the patients without risk factors as the reference group, the annual event rate rose incrementally for each additional risk factor (no risk factors, 0.2 % per year; low risk group 1.0% per year; intermediate risk group 2.7% per year; and the high risk group 4.6% per year); see figure 1 (below).
A number of risk factors have previously been described in association with TOF and either all-cause death or sudden cardiac death; examples include the Khairy score which incorporates six risk factors (prior palliative shunt, inducible sustained ventricular tachycardia (VT) during electrophysiology study, QRS duration ≥180 msec, ventriculotomy incision, non-sustained VT, and left ventricular end-diastolic pressure (LVEDP) ≥12 mmHg), and the INDICATOR multi-centre study (right ventricular hypertrophy, right ventricular hypertension, left ventricular dysfunction, and atrial arrhythmia). Not exclusive to TOF, VO2peak parameters have also been demonstrated to carry prognostic information in relation to all-cause mortality in ACHD. Interestingly, the current study only found elevated LVEDP to be an independent risk for death/transplant and not any other of the Khairy score parameters, while neither VO2peak or VE/VCO2 were associated with death or transplant. Contradictions in significant risk factors identified may reflect different population groups studied, and it should be noted that the current study did not have all invasive haemodynamic, exercise, or CMR data. Nonetheless, it offers a relatively straightforward framework of risk factors to consider in the broader TOF population which is less reliant on invasive measurements/procedures such as the Khairy score.
Non-vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: are they safe?
Yang H, Veldtman GR, Bouma BJ, Budts W, Niwa K, Meijboom F, Scognamiglio G, Egbe AC, Schwerzmann M, Broberg C, Morissens M, Buber J, Tsai S, Polyzois I, Post MC, Greutmann M, Van Dijk A, Mulder BJ, Aboulhosn J.
Open Heart. 2019 Jun 3;6(1):e000985. doi: 10.1136/openhrt-2018-000985. eCollection 2019.
PMID: 31245011 Free PMC Article
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Take Home Points:
• The 2014 PACES/HRS gave NOACS a class III recommendation for use in Fontan patients with atrial arrhythmias – this was based on a lack of data rather than reported adverse events.
• This is the first study providing prospective safety data for the use of NOACS in Fontan patients.
• They showed comparable safety and efficacy to VKA’s with an annual rate of 2.9% respectively for thromboembolism and major bleeding.
• Since the mean follow-up in this study was only 1.4 years, longer follow-up data is needed.
Commentary from Dr. Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch: Patients with Fontan circuits are at risk of both thromboembolism and bleeding. Previous recommendations included the use of aspirin or Vitamin K antagonists. The data supporting its use is scanty and derived largely form observational studies. Furthermore, both poor INR control with limited time in therapeutic range as well as aspirin resistance, reduced the efficacies of both these treatment strategies. In the treatment of non-valvular AF, Non- vitamin K antagonist oral anticoagulants (NOACS) have been shown to be as efficacious as vitamin K antagonists (VKA’s) in reducing thromboembolic events with fewer intracranial bleeds and drug interactions as well as obliterating the need for INR monitoring. In Fontan patients with atrial arrhythmias, NOACS currently have a class III recommendation by the PACES/HRS 2014 guideline. This recommendation was based on the lack of adequate available data and not on a documented safety hazard. Therefore, despite this recommendation, NOACS have been used increasingly in this population.
This study focused on the safety and efficacy of NOAC use in Fontan patients with prior atrial arrhythmia. Patients enrolled in the NOTE registry (NOACS for thromboembolic prevention in patients with CHD). The NOTE registry is a worldwide ongoing prospective registry in ACHD patients on NOACS for the prevention of thromboembolism. Recruitment commenced in April 2014 (35 centers in 10 countries including Europe, North America, the Middle East and east Asia). Consecutive patients with Fontan circulation were identified. Patients were followed up at 6 months, 1 year and 2 years.
Primary endpoints were thromboembolism (ischemic CVA, TIA, systemic or pulmonary embolism, intra-cardiac embolism) and major bleeding (defined as bleeding requiring hospitalizations / interventions/the transfusion of 2 or more units of packed cells or a haemoglobin drop > 20 g/L and/or fatal bleeding or bleeding occurring in one of the following critical sites: intracranial, intraspinal, intraocular, pericardial, intra-articular or intra-muscular with compartment syndrome. Minor bleeding was a secondary endpoint.
A total of 74 Fontan patients were identified from a total of 513 ACHD patients on NOACS. The indications for NOACS were atrial arrhythmias (n=52), primary prevention (n=12) and secondary prevention (n=10). The CHA2DS2VASc score was >1 in 66% (n=49) and only 2 patients had high HAS-BLED scores.
During the mean follow-up of 1.4 years (102.4 patient years), 3 thromboembolic events (pulmonary emboli, 1 ischemic cerebrovascular event –) and 3 major bleeds (2 menorrhagia, 1 major GIT bleed)occurred in 5 patients with AP Fontans and one patient with TCPC – annual rate 2,9%, 95% CI 0.7-7.6%, respectively. Minor bleeding occurred in 15 patients (annual rate 15.8%, 95% CI 9.1-25.2%).
Figure 1A below shows the Kaplan-Meier curves of survival free from thromboembolism, major bleeding and minor bleeding.
Two patients died during follow-up – 1 heart failure and 1 cancer death.
Prior to NOAC initiation, 50% (n=37) were on VKA’s, 26% (n=19) were taking aspirin and 24% (n=18) had no antithrombotic treatment. In the subgroup who started NOACS de-novo for primary prevention (i.e. no prior VKA or aspirin use), no thromboembolic events of major bleeding occurred.
Figure 1B above depicts the event free survival of those patients on VKA’s in the 3 years prior to changing to NOACS. In this group, over a 3.5year period, the annual event rate was 6.2% for thromboembolism (95% CI 1.9% to 14.4%) and 0% for major bleeding.
A total of 14 patients stopped NOAC therapy to recommence VKA’s. Reasons included: thromboembolism (n=3), bleeding (n=4), side-effects (n=2), pregnancy (n=2), patient refusal (n=2), Fontan conversion (n=1).
Relation Between New York Heart Association Functional Class and Objective Measures of Cardiopulmonary Exercise in Adults With Congenital Heart Disease.
Das BB, Young ML, Niu J, Mendoza LE, Chan KC, Roth T.
Am J Cardiol. 2019 Jun 1;123(11):1868-1873. doi: 10.1016/j.amjcard.2019.02.053. Epub 2019 Mar 15.
PMID: 30954207
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Take Home Points:
• NYHA class of patients is a simple measure for assessment of functional status, CPET remains an important tool to identify the source of exercise limitation in ACHD patients.
• CPET can provide objective and reproducible assessment of the cardiovascular, respiratory, and muscular systems.
• 3 of CPET parameters (pVO2, OUES, and maximum DP) overall correlate well with NYHA functional class especially between NYHA functional class I and III.
• If CPET cannot be performed, stratification using NYHA class is an acceptable alternative.
Commentary from Dr. Soha Romeih (Aswan, Egypt), section editor of ACHD Journal Watch:
Das et al compared the New York Heart Association (NYHA) functional class in adults with CHD patients with measured cardiopulmonary exercise testing parameters. This was a retrospective study which included 175 ACHD patients who underwent CPET between 2014 till 2018 in a single center. The majority of patients were repaired TOF (26%) followed by Fontan patients (19%) and repaired TGA with arterial or atrial switch (16%).
• The NYHA class was determined for each patient by physician assessment of patients’ self-reported symptoms before the exercise test or estimated from patient narrative from the medical record. Only patients with NYHA class I to III were enrolled in the study, as they were able to participate in CPET.
• Symptoms limited CPET was performed on all patients using treadmill according to modified Bruce protocol (97% of patients) or cycle ergometer ramping protocol (3% of patients). The measured parameters were:
• Peak VO2 max, as the highest 30-second average during exercise.VO2 value was adjusted per ideal body weight the normal was > 20 ml/kg/min.
• The VE/VCO2 nadir was taken as the lowest 30-second average during exercise, the cutoff of 34 (a prognostic tool of heart failure).
• O2 uptake efficient slob (OUES) was measured as relationship between VO2 max and Log VE thought the exercise., cutoff value was 2,000.
• Double product (DP) is the myocardial O2 uptake, defined as the amount of O@ consumed by the myocardium during exercise, was estimated by the product of heart rate and systolic blood pressure, cut off value was 20.000.
Results:
1- Peak VO2 in the overall study population was 26.4 ± 9.8 ml/kg/min or 72.7 ± 22.3% of predicted. There was a progressive decline in pVO2 with increasing severity of CHD (p = 0.001).
There was a strong negative correlation between functional class and pVO2, with a progressive decline from functional Class I to III (r = ¡0.48; p 0.0001). About ¼ of patients had pVO2 <20ml/kg/min and there was an overlap in pVO2 in each NYHA functional class.
However, 75% of NYHA class III had pVO2 <20 ml/kg/min compared with 10% in NYHA class I. Patients in functional class III had a markedly reduced pVO2 of 16.8 ± 4.5ml/kg/ min compared with 26.6 ± 6.1 ml/kg/min in class I (p = 0.0002).There was no significant difference in pVO2 between NYHA class II and III (p = 0.09), whereas the difference between NYHA class I and II and Class I and III are significant (p <0.004 and p <0.00002 respectively.
Figure 1. Relation between Peak VO2 and NYHA class. (A) Spearman correlation between pVO2 and NYHA class, (B) frequency of distribution of pVO2, (C) Relative distribution with a cut-off value of pVO2 20 ml/kg/min, and (D) Box and whisker-plot demonstrating median and quartiles of pVO2 by NYHA class. NYHA = New York Heart Association.
2- The VE/VCO2 slope in the overall study population was 31.7 ± 5.4. Patients in functional class III had the higher VE/VCO2 slopes compared with class I and II, but there was no significant correlation between NYHA class and VE/VCO2 slope (r = 0.14, p = 0.37).
The distribution of VE/VCO2 slope was within upper normal limits in functional class I patients, but one-fifth of these asymptomatic patients (20%) had an abnormal slope > 34. Almost one half of patients in NYHA class II (42%) and III (50%) had a VE/VCO2 slope > 34.There was no significant difference between NYHA class I and II (p = 0.071), or between Class II and III (p = 0.263), whereas VE/VCO2 was significantly different between Class I and III (p = 0.011).
Figure 2 Relation between VE/VCO2 slope and NYHA class. (A) Spearman correlation between VE/VCO2 and NYHA class, (B) frequency of distribution of VE/VCO2, (C) Relative distribution with a cut-off value of VE/VCO2 34, and (D) Box and whisker-plot demonstrating median and quartiles of VE/VCO2 by NYHA class. NYHA = New York Heart Association.
3- The OUES in the overall study population was 2,565 ± 825. There was a progressive decline in OUES with increasing severity of NYHA class (r = ¡0.35, p <0.0001). Majority of ACHD patients had OUES > 2000. There was no significant difference in OUES between NYHA class II and III (p = 0.158), whereas the difference between NYHA class I and II and Class I and III were significant (p < 0.001).
Figure 3. Relation between OUES slope and NYHA class. (A) Spearman correlation between OUES and NYHA class, (B) frequency of distribution of OUES, (C) Relative distribution with a cut-off value of OUES 2,000, and (D) Box and whisker-plot demonstrating median and quartiles of OUES by NYHA class. NYHA = New York Heart Association; OUES = O2 uptake efficient slope.
4- The maximum DP in the overall study population was 26,602 ± 6,127. There was a progressive decline in maximum DP with increasing NYHA functional class (r = ¡0.31, p <0.0001). Majority of patients had DP > 20,000.
There was no significant difference in maximum DP between NYHA class II and III (p = 0.749), whereas the difference between NYHA class I and II and Class I and III were significant (p <0.001 and p<0.05 respectively).
Figure 4. Relation between maximum double product and NYHA class. (A) Spearman correlation between double product (DP) and NYHA class, (B) frequency of distribution of DP, (C) Relative distribution with a cut-off value of DP 20,000, and (D) Box and whisker-plot demonstrating median and quartiles of DP by NYHA class. NYHA = New York Heart Association; OUES = O2 uptake efficient slope.
Conclusions:
NYHA functional class correlates with objective measures of CPET, however there is wide variability in measured exercise capacity in each NYHA classification. Therefore, whereas NYHA class of patients is a simple measure for assessment of functional status, CPET is an important tool to identify the source of exercise limitation in ACHD patients.
X chromosome gene dosage as a determinant of congenital malformations and of age-related comorbidity risk in patients with Turner syndrome, from childhood to early adulthood.
Fiot E, Zénaty D, Boizeau P, Haignere J, Dos Santos S, Léger J; French Turner Syndrome Study Group.
Eur J Endocrinol. 2019 Jun 1;180(6):397-406. doi: 10.1530/EJE-18-0878.
PMID: 30991358
Take Home Points:
• Turner syndrome (TS) patients with 45, X karyotype are most likely to have congenital heart disease when compared with other karyotypes
• TS mosaicism patients were less likely to develop comorbidities as they got older when compared with other karyotypes.
• Patients with a ring X chromosome were more prone to metabolic disorders.
• It is possible that X gene chromosome dosage, particularly for Xp genes, contributes to the risk of developing acquired comorbid conditions.
Commentary by Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch: Turner syndrome (TS) as a condition in which all or part of one X-chromosome Is absent from some or all cells. TS occurs in about 1/2500 live born girls. TS is associated with congenital cardiac and renal malformations. In addition, TS is associated with increased risk of hearing loss and metabolic disease that includes obesity, dyslipidemia, hypertension, abnormal glucose metabolism, and liver dysfunction. An increased risk of autoimmune thyroid disease and celiac disease is also observed. Certain TS karyotypes may increase the associated risk of both congenital and acquired conditions.
Dr. Elodie Fiot and colleagues from France conducted a national, multi-centered, observational study that included all patients with Turner syndrome diagnosed before January 2013 and followed at participating pediatric and adult centers from the French national rare disease network (the French Turner syndrome study group). The prevalence of congenital malformations and the cumulative incidence of subsequent comorbidities was evaluated at five-year intervals, from the ages of 10 to 30 years. The median age was 9.4 years at initial evaluation and 16.8 years at last evaluation with a median follow-up of 4.1 years. This was a young cohort with only about 12% over the age of 30 years at last evaluation.
The TS karyotype associations with congenital malformations were as follows:
- 45, X (n= 549, 36%): cardiac malformations were present in 27% and renal malformations in 22%
- 45, X /46, iso-chromosome Xq (n= 280, 19%): cardiac malformations were present in 12.5% and renal malformations 18.9%
- Ring X chromosome 46, X, r(X)/ 46, XX (n= 106, 7%): cardiac malformations were present in 17% and renal malformations 20.8%.
- 45, X /46, XX Mosaic karyotype (n= 221, 15%): cardiac malformations were present in 13.1% and renal malformations 11.3%.
- Presence of Y chromosome (n= 87, 6%): cardiac malformations were present in 21.8% and renal malformations in 10.3%.
- Other (various mosaicism with triple X, variable Xp for Xq deletions, and various complex rearrangements or translocations within an X chromosome) (n= 258, 17%): cardiac malformation were present in 13.2% and renal malformations in 10.5%.
There were 35 patients with unknown karyotype who were excluded from the study.
Congenital cardiac malformations were more common with 45, X compared with other karyotypes.
As one would expect, TS patients acquired comorbid medical conditions, such as hearing loss, metabolic disease, and autoimmune disease as they got older. However, patients with TS mosaicism were less likely to develop comorbidities when compared with other karyotypes.
This study showed a lower prevalence of comorbidities in TS with 45,X/46,XX mosaicism or TS with a Y chromosome when compared with TS patients with a ring X chromosome or with a 45,X/46,isoXq or 45,X karyotype. This may underscore the role of X chromosome dosage in the occurrence of comorbidities in TS patients.
Patients with a ring X chromosome were more prone to metabolic disorders.
A plausible explanation for these findings is that X chromosome gene dosage influences TS associated conditions and results in higher risk of comorbid conditions in patients with the 45,X, 45,X/46,isoXq and 46,X,r(X)/46,XX karyotypes and a lower risk in patients with mosaic karyotypes, such as 45,X/46,XX or XY mosaic karyotypes, suggesting an important role of Xp genes.
This study adds to the understanding of the age-related increase in the incidence of acquired comorbidities and the relationship between the prevalence of congenital malformations, acquired diseases and karyotype subgroups in TS.
This was a large study but cohort was young and additional studies in older TS patients are needed.
ACHD June 2019
1. Adult congenital heart disease: past, present, future.
Brida M, Gatzoulis MA.
Acta Paediatr. 2019 Jun 29. doi: 10.1111/apa.14921. [Epub ahead of print] Review.
PMID: 31254360
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2. Factors influencing the participation of adolescents and young adults with a congenital heart disease in a transition education program: A prospective multicentre controlled study.
Werner O, Abassi H, Lavastre K, Guillaumont S, Picot MC, Serrand C, Dulac Y, Souletie N, Acar P, Bredy C, Amedro P.
Patient Educ Couns. 2019 Jun 26. pii: S0738-3991(19)30256-3. doi: 10.1016/j.pec.2019.06.023. [Epub ahead of print]
PMID: 31262673
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3. Tetralogy of Fallot with isolated levocardia in a young female.
Sattar Z, Abdullah HM, Roomi S, Ullah W, Khan A, Ghani A, Ahmad A.
J Community Hosp Intern Med Perspect. 2019 Jun 19;9(3):275-278. doi: 10.1080/20009666.2019.1601980. eCollection 2019.
PMID: 31258874 Free PMC Article
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4. Coronary Artery Disease in Adults With Coarctation of Aorta: Incidence, Risk Factors, and Outcomes.
Egbe AC, Rihal CS, Thomas A, Boler A, Mehra N, Andersen K, Kothapalli S, Taggart NW, Connolly HM.
J Am Heart Assoc. 2019 Jun 18;8(12):e012056. doi: 10.1161/JAHA.119.012056. Epub 2019 Jun 14.
PMID: 31195876 Free Article
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5. Turner syndrome: mechanisms and management.
Gravholt CH, Viuff MH, Brun S, Stochholm K, Andersen NH.
Nat Rev Endocrinol. 2019 Jun 18. doi: 10.1038/s41574-019-0224-4. [Epub ahead of print] Review.
PMID: 31213699
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6. Secular trends in pregnancy rates, delivery outcomes, and related health care utilization among women with congenital heart disease.
Bottega N, Malhamé I, Guo L, Ionescu-Ittu R, Therrien J, Marelli A.
Congenit Heart Dis. 2019 Jun 17. doi: 10.1111/chd.12811. [Epub ahead of print]
PMID: 31207185
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7. Postpartum Cardiovascular Outcomes Among Women With Heart Disease from A Nationwide Study.
Lima F, Nie L, Yang J, Owens A, Dianati-Maleki N, Avila C, Stergiopoulos K.
Am J Cardiol. 2019 Jun 15;123(12):2006-2014. doi: 10.1016/j.amjcard.2019.03.012. Epub 2019 Mar 19.
PMID: 30967289
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8. Cardiac Denial and Expectations Associated With Depression in Adults With Congenital Heart Disease.
Huntley GD, Tecson KM, Sodhi S, Saef J, White KS, Ludbrook PA, Cedars AM, Ko JM.
Am J Cardiol. 2019 Jun 15;123(12):2002-2005. doi: 10.1016/j.amjcard.2019.03.011. Epub 2019 Mar 16.
PMID: 30967286
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9. Sacubitril/valsartan for heart failure in adults with complex congenital heart disease.
Maurer SJ, Pujol Salvador C, Schiele S, Hager A, Ewert P, Tutarel O.
Int J Cardiol. 2019 Jun 13. pii: S0167-5273(19)31521-9. doi: 10.1016/j.ijcard.2019.06.031. [Epub ahead of print]
PMID: 31242968
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10. Pregnancy with Heart Disease: Maternal Outcomes and Risk Factors for Fetal Growth Restriction.
Nguyen Manh T, Bui Van N, Le Thi H, Vo Hoang L, Nguyen Si Anh H, Trinh Thi Thu H, Nguyen Xuan T, Vu Thi N, Minh LB, Chu DT.
Int J Environ Res Public Health. 2019 Jun 12;16(12). pii: E2075. doi: 10.3390/ijerph16122075.
PMID: 31212780 Free Article
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11. Genetic counselling and testing in adults with congenital heart disease: A consensus document of the ESC Working Group of Grown-Up Congenital Heart Disease, the ESC Working Group on Aorta and Peripheral Vascular Disease and the European Society of Human Genetics.
De Backer J, Bondue A, Budts W, Evangelista A, Gallego P, Jondeau G, Loeys B, Peña ML, Teixido-Tura G, van de Laar I, Verstraeten A, Roos Hesselink J.
Eur J Prev Cardiol. 2019 Jun 11:2047487319854552. doi: 10.1177/2047487319854552. [Epub ahead of print]
PMID: 31184212
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12. Sarcopenia is common in adults with complex congenital heart disease.
Sandberg C, Johansson K, Christersson C, Hlebowicz J, Thilén U, Johansson B.
Int J Cardiol. 2019 Jun 10. pii: S0167-5273(19)30759-4. doi: 10.1016/j.ijcard.2019.06.011. [Epub ahead of print]
PMID: 31230936
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13. Mechanism and Risk Factors for Death in Adults With Tetralogy of Fallot.
Egbe AC, Kothapalli S, Borlaug BA, Ammash NM, Najam M, Bajwa N, Tarek K, Matthew J, Connolly HM.
Am J Cardiol. 2019 Jun 7. pii: S0002-9149(19)30627-7. doi: 10.1016/j.amjcard.2019.05.048. [Epub ahead of print]
PMID: 31272701
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14. Long-surviving Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta Complicated with Pulmonary Arteriovenous Fistula.
Ueno R, Yagi S, Bando M, Sata M.
Intern Med. 2019 Jun 7. doi: 10.2169/internalmedicine.2455-18. [Epub ahead of print] No abstract available.
PMID: 31178488 Free Article
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15. Transition Readiness in Adolescents and Young Adults with Heart Disease: Can We Improve Quality of Life?
Uzark K, Afton K, Yu S, Lowery R, Smith C, Norris MD.
J Pediatr. 2019 Jun 7. pii: S0022-3476(19)30543-8. doi: 10.1016/j.jpeds.2019.04.060. [Epub ahead of print]
PMID: 31182220
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16. Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review.
Rudienė V, Hjortshøj CMS, Glaveckaitė S, Zakarkaitė D, Petrulionienė Ž, Gumbienė L, Aidietis A, Søndergaard L.
Heart. 2019 Jun 5. pii: heartjnl-2019-314714. doi: 10.1136/heartjnl-2019-314714. [Epub ahead of print] Review.
PMID: 31171629
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17. Left Ventricular Non-compaction (LVNC) Cardiomyopathy.
Singh DP, Patel H.
StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.
2019 Jun 4.
PMID: 30725710 Free Books & Documents
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18. Tetralogy of Fallot.
Diaz-Frias J, Guillaume M.
StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-.
2019 Jun 4.
PMID: 30020660 Free Books & Documents
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Select item 29939590
19. Long-Term Outcomes in Patients With Turner Syndrome: A 68-Year Follow-Up.
Fuchs MM, Attenhofer Jost C, Babovic-Vuksanovic D, Connolly HM, Egbe A.
J Am Heart Assoc. 2019 Jun 4;8(11):e011501. doi: 10.1161/JAHA.118.011501.
PMID: 31131660 Free PMC Article
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20. Non-vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: are they safe.
Yang H, Veldtman GR, Bouma BJ, Budts W, Niwa K, Meijboom F, Scognamiglio G, Egbe AC, Schwerzmann M, Broberg C, Morissens M, Buber J, Tsai S, Polyzois I, Post MC, Greutmann M, Van Dijk A, Mulder BJ, Aboulhosn J.
Open Heart. 2019 Jun 3;6(1):e000985. doi: 10.1136/openhrt-2018-000985. eCollection 2019.
PMID: 31245011 Free PMC Article
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21. Relation Between New York Heart Association Functional Class and Objective Measures of Cardiopulmonary Exercise in Adults With Congenital Heart Disease.
Das BB, Young ML, Niu J, Mendoza LE, Chan KC, Roth T.
Am J Cardiol. 2019 Jun 1;123(11):1868-1873. doi: 10.1016/j.amjcard.2019.02.053. Epub 2019 Mar 15.
PMID: 30954207
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22. Risk assessment and survival of patients with pulmonary hypertension: Multicenter experience in Turkey.
Yaylalı YT, Başarıcı I, Kılıçkıran Avcı B, Meriç M, Sinan ÜY, Şenol H, Küçükoğlu MS, Öngen Z.
Anatol J Cardiol. 2019 Jun;21(6):322-330. doi: 10.14744/AnatolJCardiol.2019.53498.
PMID: 31142721 Free Article
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23. Trends and Outcomes of Infective Endocarditis in Adults With Tetralogy of Fallot: A Review of the National Inpatient Sample Database.
Egbe AC, Vallabhajosyula S, Akintoye E, Connolly HM.
Can J Cardiol. 2019 Jun;35(6):721-726. doi: 10.1016/j.cjca.2019.02.006. Epub 2019 Feb 16.
PMID: 31151707
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24. Nurses’ Perceptions of Quality of Life for Adolescents with Congenital Heart Disease.
Shackleford JL, Minick P, Kelley SJ.
Compr Child Adolesc Nurs. 2019 Jun;42(2):92-108. doi: 10.1080/24694193.2017.1397066. Epub 2017 Dec 13.
PMID: 29236545
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25. Aortopulmonary window in adults: A rare entity leading to Eisenmenger syndrome.
El Dick J, El-Rassi I, Tayeh C, Bitar F, Arabi M.
Echocardiography. 2019 Jun;36(6):1173-1178. doi: 10.1111/echo.14368. Epub 2019 May 22. Review.
PMID: 31116466
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26. A rare case series of mitral valve clefts diagnosed by 3D echocardiography and mini-review of the literature.
Moura-Ferreira S, Sampaio F, Ribeiro J, Fontes-Carvalho R.
Echocardiography. 2019 Jun;36(6):1203-1207. doi: 10.1111/echo.14353. Epub 2019 Apr 26.
PMID: 31025775
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27. Patient empowerment and its correlates in young persons with congenital heart disease.
Acuña Mora M, Sparud-Lundin C, Burström Å, Hanseus K, Rydberg A, Moons P, Bratt EL.
Eur J Cardiovasc Nurs. 2019 Jun;18(5):389-398. doi: 10.1177/1474515119835434. Epub 2019 Mar 5.
PMID: 30834772
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28. X chromosome gene dosage as a determinant of congenital malformations and of age-related comorbidity risk in patients with Turner syndrome, from childhood to early adulthood.
Fiot E, Zénaty D, Boizeau P, Haignere J, Dos Santos S, Léger J.
Eur J Endocrinol. 2019 Jun 1;180(6):397-406. doi: 10.1530/EJE-18-0878.
PMID: 30991358
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29. Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease.
Favoccia C, Constantine AH, Wort SJ, Dimopoulos K.
Expert Rev Cardiovasc Ther. 2019 Jun;17(6):449-459. doi: 10.1080/14779072.2019.1623024. Epub 2019 Jun 6.
PMID: 31120797
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30. Osteopontin plays important roles in pulmonary arterial hypertension induced by systemic-to-pulmonary shunt.
Meng L, Liu X, Teng X, Gu H, Yuan W, Meng J, Li J, Zheng Z, Wei Y, Hu S.
FASEB J. 2019 Jun;33(6):7236-7251. doi: 10.1096/fj.201802121RR. Epub 2019 Mar 20.
PMID: 30893567
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31. Right ventricular fibrosis is associated with cardiac remodelling after pulmonary valve replacement.
Yamamura K, Yuen D, Hickey EJ, He X, Chaturvedi RR, Friedberg MK, Grosse-Wortmann L, Hanneman K, Billia F, Farkouh ME, Wald RM.
Heart. 2019 Jun;105(11):855-863. doi: 10.1136/heartjnl-2018-313961. Epub 2018 Dec 4.
PMID: 30514732
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32. Cardiac risk prediction model for pregnant women with structural heart disease in Eastern China.
Fu Q, Wang XJ, Wang BS, Lin JH.
Int J Gynaecol Obstet. 2019 Jun;145(3):324-330. doi: 10.1002/ijgo.12820. Epub 2019 Apr 23.
PMID: 30932184
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33. The bicuspid aortic valve: Is it an immunological disease process?
Howard C, Picca L, Smith T, Sharif M, Bashir M, Harky A.
J Card Surg. 2019 Jun;34(6):482-494. doi: 10.1111/jocs.14050. Epub 2019 Apr 23.
PMID: 31012137
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34. [Adult Cor Triatriatum Found by Chance at the Onset of Myocardial Infarction].
Toyama M, Nakayama M, Abe T, Shimazu S, Ohno O.
Kyobu Geka. 2019 Jun;72(6):414-417. Japanese.
PMID: 31268012
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35. Occurrence of a primary liver cancer with an unusual histologic appearance as a late Fontan complication.
Matsuyama TA, Ohuchi H, Saito K, Kondo F, Fukusato T, Ishibashi-Ueda H.
Pathol Res Pract. 2019 Jun;215(6):152366. doi: 10.1016/j.prp.2019.02.016. Epub 2019 Feb 27.
PMID: 30857653
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36. Use of ondansetron during pregnancy and the risk of major congenital malformations: A systematic review and meta-analysis.
Kaplan YC, Richardson JL, Keskin-Arslan E, Erol-Coskun H, Kennedy D.
Reprod Toxicol. 2019 Jun;86:1-13. doi: 10.1016/j.reprotox.2019.03.001. Epub 2019 Mar 5.
PMID: 30849498
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37. The Association between Pulmonary Function and Left Ventricular Volume and Function in Duchenne Muscular Dystrophy.
Khokhar A, Nair A, Midya V, Kumar A, Sinharoy A, Ahmad TA, Abu-Hasan M, Mondal P.
Muscle Nerve. 2019 Jun 28. doi: 10.1002/mus.26623. [Epub ahead of print]
PMID: 31250930
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37. Sleep apnea and the impact on cardiovascular risk in patients with Marfan syndrome.
Muiño-Mosquera L, Bauters F, Dhondt K, De Wilde H, Jordaens L, De Groote K, De Wolf D, Hertegonne K, De Backer J.
Mol Genet Genomic Med. 2019 Jun 27:e805. doi: 10.1002/mgg3.805. [Epub ahead of print]
PMID: 31245936 Free Article
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38. Acute impact of an endurance race on cardiac function and biomarkers of myocardial injury in triathletes with and without myocardial fibrosis.
Tahir E, Scherz B, Starekova J, Muellerleile K, Fischer R, Schoennagel B, Warncke M, Stehning C, Cavus E, Bohnen S, Radunski UK, Blankenberg S, Simon P, Pressler A, Adam G, Patten M, Lund GK.
Eur J Prev Cardiol. 2019 Jun 26:2047487319859975. doi: 10.1177/2047487319859975. [Epub ahead of print]
PMID: 31242053
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39. Coronary Artery Disease in Adults With Coarctation of Aorta: Incidence, Risk Factors, and Outcomes.
Egbe AC, Rihal CS, Thomas A, Boler A, Mehra N, Andersen K, Kothapalli S, Taggart NW, Connolly HM.
J Am Heart Assoc. 2019 Jun 18;8(12):e012056. doi: 10.1161/JAHA.119.012056. Epub 2019 Jun 14.
PMID: 31195876 Free Article
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40. Health care stakeholder perspectives regarding the role of a patient navigator during transition to adult care.
Dimitropoulos G, Morgan-Maver E, Allemang B, Schraeder K, Scott SD, Pinzon J, Andrew G, Guilcher G, Hamiwka L, Lang E, McBrien K, Nettel-Aguirre A, Pacaud D, Zwaigenbaum L, Mackie A, Samuel S.
BMC Health Serv Res. 2019 Jun 17;19(1):390. doi: 10.1186/s12913-019-4227-6.
PMID: 31208417 Free PMC Article
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41. Right Ventricular Size and Function in Neonates after Use of Antidepressants during Pregnancy.
Meinel K, Sallmon H, Koestenberger M.
Neonatology. 2019 Jun 11:1-2. doi: 10.1159/000500526. [Epub ahead of print] No abstract available.
PMID: 31185472
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42. The Golden Proportion in the scallop geometry of normal mitral valves. When nature plays with jigsaw puzzles.
Deorsola L, Bellone A.
Echocardiography. 2019 Jun;36(6):1028-1034. doi: 10.1111/echo.14366. Epub 2019 May 16.
PMID: 31095787
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43. An unusual cause of right ventricular outflow tract obstruction.
Vô C, Carbonez K, De Beco G, Poncelet A, Moniotte S.
Eur Heart J Cardiovasc Imaging. 2019 Jun 1;20(6):657. doi: 10.1093/ehjci/jez005. No abstract available.
PMID: 30689777
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44. Four-dimensional flow magnetic resonance imaging-derived blood flow energetics of the inferior vena cava-to-extracardiac conduit junction in Fontan patients.
Rijnberg FM, Elbaz MSM, Westenberg JJM, Kamphuis VP, Helbing WA, Kroft LJ, Blom NA, Hazekamp MG, Roest AAW.
Eur J Cardiothorac Surg. 2019 Jun 1;55(6):1202-1210. doi: 10.1093/ejcts/ezy426.
PMID: 30590476
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45. Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support.
Kubicki R, Stiller B, Kroll J, Siepe M, Beyersdorf F, Benk C, Höhn R, Grohmann J, Fleck T, Zieger B.
Eur J Cardiothorac Surg. 2019 Jun 1;55(6):1194-1201. doi: 10.1093/ejcts/ezy408.
PMID: 30590475
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46. Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis.
Miranda WR, Borlaug BA, Hagler DJ, Connolly HM, Egbe AC.
Eur J Heart Fail. 2019 Jun;21(6):803-809. doi: 10.1002/ejhf.1365. Epub 2019 Jan 23.
PMID: 30672076
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47. Incidentally identified genetic variants in arrhythmogenic right ventricular cardiomyopathy-associated genes among children undergoing exome sequencing reflect healthy population variation.
Headrick AT, Rosenfeld JA, Yang Y, Tunuguntla H, Allen HD, Penny DJ, Kim JJ, Landstrom AP.
Mol Genet Genomic Med. 2019 Jun;7(6):e593. doi: 10.1002/mgg3.593. Epub 2019 Apr 15.
PMID: 30985088 Free PMC Article
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48. Identifying Subclinical Coronary Abnormalities and Silent Myocardial Ischemia After Arterial Switch Operation.
Tsuda T, Baffa JM, Octavio J, Robinson BW, Radtke W, Mody T, Bhat AM.
Pediatr Cardiol. 2019 Jun;40(5):901-908. doi: 10.1007/s00246-019-02085-4. Epub 2019 Mar 9.
PMID: 30852629
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ACHD Electrophysiology Featured Articles
Congenital and Pediatric Cardiac EP Reviews of June 2019 Manuscripts
Different characteristics of postoperative atrial tachyarrhythmias between congenital and non-congenital heart disease.
Kondo M, Fukuda K, Wakayama Y, Nakano M, Hasebe Y, Satake H, Segawa M, Hirano M, Shimokawa H.
J Interv Card Electrophysiol. 2019 Jun 13. doi: 10.1007/s10840-019-00575-2. [Epub ahead of print]
PMID: 31197584
Take Home Points:
• Atrial tachyarrhythmias in ACHD vs. non-ACHD differ with regards to arrhythmia mechanism and substrate.
• Atrial tachyarrhythmias develop at earlier age and following longer duration after cardiac surgery in ACHD patients.
Comment from Dr. Philip Chang (Gainesville, FL), section editor of Congenital Electrophysiology Journal Watch: This study by Kondo et al retrospectively reviewed consecutive adult patients with and without CHD undergoing EP study with catheter ablation for the treatment of drug-refractory atrial tachyarrhythmias. Given a general recognition that atrial arrhythmias differ between CHD and non-CHD adult patients, the authors systematically evaluated arrhythmia substrate and mechanism in CHD and non-CHD cohorts with atrial arrhythmias to further characterize and understand their differences.
The study involved a total of 42 adult patients referred for ablation of drug-refractory atrial tachyarrhythmias in the setting of prior cardiac surgery. Procedures were performed between January 2009 and May 2014. Cohort sizes, types of CHD, and types of non-CHD surgery and cardiac conditions are listed in the table below.
Patients underwent invasive EP testing and catheter ablation with non-irrigated or irrigated catheters and 3D electroanatomic mapping assistance. The authors classified atrial arrhythmias as either cavotricuspid isthmus dependent atrial flutter (AFL), intra-atrial reentrant tachycardia (IART), or focal atrial tachycardia (FAT). Several arrhythmias were classified as Other (including 2 cases of AVNRT and 2 left atrial tachycardias) in the analysis. Arrhythmia evaluation involved determination of (right) atrial chamber surface area, voltage characteristics and distribution (with low voltage defined as <0.5 mV), and activation (and entrainment when feasible) mapping of the arrhythmias. Standard catheter ablation techniques were employed for definitive arrhythmia treatment.
A total of 44 arrhythmias were identified among the 26-patient CHD cohort and 21 arrhythmias in the 16-patient non-CHD cohort. ACHD patients were significantly younger at the time of atrial arrhythmia diagnosis and had longer durations of time between prior surgery and present catheter ablation. Non-ACHD patients had a higher incidence of hospitalization for heart failure. Arrhythmia mechanisms and distribution among the different CHD and non-CHD types are depicted and listed in the figure and table below.
Right atrial surface area was significantly larger in the ACHD cohort (197.1±56.4 cm2 vs. 132.4±41.2 cm2) and area of low voltage was also significantly larger in ACHD patients (40.8±33.3 cm2 vs. 13.6±9 cm2) and frequently involved the posterior right atrium. The ACHD cohort had a higher incidence of multiple tachycardia mechanisms in a single patient, IART, and FAT. Right atrial FAT was only seen in ACHD patients and among these patients, the arrhythmia was mapped most frequently to the posterolateral atrium adjacent to the crista terminalis within low-voltage tissue in the posterior atrium. Cavotricuspid isthmus dependent atrial flutter accounted for nearly 75% of non-ACHD atrial tachyarrhythmias ablated. Acutely successful ablation was achieved in all patients with recurrence in 6/26 ACHD patients and 2/16 non-ACHD patients (23% vs. 12.5%).
The study is limited by small cohort sizes and relatively limited ability to evaluate subgroups of ACHD patients where significant differences and diversity can frequently be found. There was also an under-representation of certain CHD types recognized to have high incidence of atrial arrhythmias and complex arrhythmia substrate (namely, TGA/atrial switch patients and older variants of Fontan palliation). Finally, the authors provided very little information regarding left atrial or pulmonary venous atrial arrhythmias, with primary concentration on the right atrium only. As such, the study is really a more detailed descriptive evaluation of right atrial pathology in ACHD vs. non-ACHD. However, the study does further validate and demonstrate distinct differences in arrhythmia types, distribution, and associated substrate between ACHD and non-ACHD patients. Knowledge of prior surgical history including type, number, and location of surgeries performed is critically important and may even be predictive of type and location of arrhythmias. The posterior right atrial wall is frequently abnormal in ACHD patients with atrial arrhythmias, both as noted in this study as well as from this reviewer’s personal, anecdotal experience. Finally, the study’s authors noted that FAT was absent in their adult TOF patients who did not require pulmonary valve replacement during the follow-up period, which also supports the understanding that ongoing hemodynamic derangements have profound impact on arrhythmias in ACHD. Newer and integrated technologies can further characterize arrhythmia substrates and facilitate ablation performance, with hopefully longer term success.
CHD EP June 2019
1. Long-term follow-up and comparison of techniques in radiofrequency ablation of ventricular arrhythmias originating from the aortic cusps (AVATAR Registry).
Styczkiewicz K, Ludwik B, Śledź J, Lipczyńska M, Zaborska B, Kryński T, Deutsch K, Morka A, Kukla P, Styczkiewicz M, Kułakowski P, Stec SM.
Pol Arch Intern Med. 2019 Jun 28;129(6):399-407. doi: 10.20452/pamw.14861. Epub 2019 Jun 6.
PMID: 31169259 Free Article
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2. 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. 2019 Jun 25. doi: 10.1007/s10286-019-00616-w. [Epub ahead of print]
PMID: 31240423
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3. Congenital heart block: Pace earlier (Childhood) than later (Adulthood).
Manolis AA, Manolis TA, Melita H, Manolis AS.
Trends Cardiovasc Med. 2019 Jun 20. pii: S1050-1738(19)30091-X. doi: 10.1016/j.tcm.2019.06.006. [Epub ahead of print] Review.
PMID: 31262557
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4. Out-of-hospital cardiac arrest and survival in a patient with Noonan syndrome and multiple lentigines: a case report.
Eichhorn C, Voges I, Daubeney PEF.
J Med Case Rep. 2019 Jun 15;13(1):194. doi: 10.1186/s13256-019-2096-6.
PMID: 31208451 Free PMC Article
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5. Different characteristics of postoperative atrial tachyarrhythmias between congenital and non-congenital heart disease.
Kondo M, Fukuda K, Wakayama Y, Nakano M, Hasebe Y, Satake H, Segawa M, Hirano M, Shimokawa H.
J Interv Card Electrophysiol. 2019 Jun 13. doi: 10.1007/s10840-019-00575-2. [Epub ahead of print]
PMID: 31197584
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6. Early postoperative interventional aortic valve closure for severe aortic regurgitation in a neonate after Norwood procedure.
Dilber D, Eicken A, Cleuziou J.
Cardiol Young. 2019 Jun 6:1-3. doi: 10.1017/S1047951119000155. [Epub ahead of print]
PMID: 31169113
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7. Efficacy of catheter ablation for atrial fibrillation in patients with congenital heart disease.
Guarguagli S, Kempny A, Cazzoli I, Barracano R, Gatzoulis MA, Dimopoulos K, Ernst S.
Europace. 2019 Jun 5. pii: euz157. doi: 10.1093/europace/euz157. [Epub ahead of print]
PMID: 31168581
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8. Association of Titin-Truncating Genetic Variants With Life-threatening Cardiac Arrhythmias in Patients With Dilated Cardiomyopathy and Implanted Defibrillators.
Corden B, Jarman J, Whiffin N, Tayal U, Buchan R, Sehmi J, Harper A, Midwinter W, Lascelles K, Markides V, Mason M, Baksi J, Pantazis A, Pennell DJ, Barton PJ, Prasad SK, Wong T, Cook SA, Ware JS.
JAMA Netw Open. 2019 Jun 5;2(6):e196520. doi: 10.1001/jamanetworkopen.2019.6520.
PMID: 31251381
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9. Hidden Complexity in Routine Adult and Pediatric Arrhythmias Interpretation: The Future of Precision Electrocardiology.
Leonelli FM, De Ponti R, Drago F, Baban A, Cortez D, Griselli M, Bagliani G.
Card Electrophysiol Clin. 2019 Jun;11(2):391-404. doi: 10.1016/j.ccep.2019.03.001. Review.
PMID: 31084858
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10. Arrhythmias due to Inherited and Acquired Abnormalities of Ventricular Repolarization.
Locati ET, Bagliani G, Cecchi F, Johny H, Lunati M, Pappone C.
Card Electrophysiol Clin. 2019 Jun;11(2):345-362. doi: 10.1016/j.ccep.2019.02.009. Epub 2019 Apr 10. Review.
PMID: 31084855
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11. Safety and outcomes of catheter ablation for atrial fibrillation in adults with congenital heart disease: A multicenter registry study.
Liang JJ, Frankel DS, Parikh V, Lakkireddy D, Mohanty S, Burkhardt JD, Natale A, Szilagyi J, Gerstenfeld EP, Moore JP, Collins KK, Kay JD, Santangeli P, Marchlinski FE, Sauer WH, Nguyen DT.
Heart Rhythm. 2019 Jun;16(6):846-852. doi: 10.1016/j.hrthm.2018.12.024. Epub 2018 Dec 26.
PMID: 30593868
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12. Lead Extraction With Baffle Stenting in Adults With Transposition of the Great Arteries.
Laredo M, Waldmann V, Chaix MA, Ibrahim R, Casteigt B, Dubuc M, Thibault B, Asgar AW, Dore A, Mongeon FP, Khairy P, Mondésert B.
JACC Clin Electrophysiol. 2019 Jun;5(6):671-680. doi: 10.1016/j.jacep.2019.01.023. Epub 2019 Mar 27.
PMID: 31221353
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13. Long-term outcomes of cardiac resynchronization therapy in adult congenital heart disease.
Leyva F, Zegard A, Qiu T, de Bono J, Thorne S, Clift P, Marshall H, Hudsmith L.
Pacing Clin Electrophysiol. 2019 Jun;42(6):573-580. doi: 10.1111/pace.13670. Epub 2019 Apr 17.
PMID: 30908673
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14. Cardiac resynchronization therapy in adult congenital heart disease.
Sabra M, Refaat MM.
Pacing Clin Electrophysiol. 2019 Jun;42(6):581-582. doi: 10.1111/pace.13656. Epub 2019 Mar 28. No abstract available.
PMID: 30883794
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15. A comparison of oral flecainide and amiodarone for the treatment of recurrent supraventricular tachycardia in children.
Hill AC, Silka MJ, Bar-Cohen Y.
Pacing Clin Electrophysiol. 2019 Jun;42(6):670-677. doi: 10.1111/pace.13662. Epub 2019 Mar 28.
PMID: 30875081
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16. Quinidine-A legacy within the modern era of antiarrhythmic therapy.
Vitali Serdoz L, Rittger H, Furlanello F, Bastian D.
Pharmacol Res. 2019 Jun;144:257-263. doi: 10.1016/j.phrs.2019.04.028. Epub 2019 Apr 23. Review.
PMID: 31026503
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17. Pediatric Post-Cardiac Arrest Care: A Scientific Statement From the American Heart Association.
Topjian AA, de Caen A, Wainwright MS, Abella BS, Abend NS, Atkins DL, Bembea MM, Fink EL, Guerguerian AM, Haskell SE, Kilgannon JH, Lasa JJ, Hazinski MF; American Heart Association Emergency Cardiovascular Care Science Subcommittee; American Heart Association Emergency Cardiovascular Care Pediatric Emphasis Group; Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation; Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; Council on Genomic and Precision Medicine; and Stroke Council.
Circulation. 2019 Jun 27:CIR0000000000000697. doi: 10.1161/CIR.0000000000000697. [Epub ahead of print]
PMID: 31242751
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18. Electrocardiogram in Newborns: Beneficial or Not?
Campuzano O, Sarquella-Brugada G, Cesar S, Garcia-Algar O, Brugada J, Brugada R.
Pediatr Cardiol. 2019 Jun 27. doi: 10.1007/s00246-019-02142-y. [Epub ahead of print] No abstract available.
PMID: 31250047
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19. Validation of a novel automated signal analysis tool for ablation of Wolff-Parkinson-White Syndrome.
Ceresnak SR, Pass RH, Dubin AM, Yang L, Motonaga KS, Hedlin H, Avasarala K, Trela A, McElhinney DB, Janson C, Nappo L, Ling XB, Gates GJ.
PLoS One. 2019 Jun 26;14(6):e0217282. doi: 10.1371/journal.pone.0217282. eCollection 2019.
PMID: 31242221 Free PMC Article
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20. Arrhythmias and fasciculoventricular pathways in patients with Danon disease: A single center experience.
Jhaveri S, Herber J, Zahka K, Boyle GJ, Saarel EV, Aziz PF.
J Cardiovasc Electrophysiol. 2019 Jun 25. doi: 10.1111/jce.14049. [Epub ahead of print]
PMID: 31240821
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21. Slow and Steady or Fast and Furious? Sinus Node Dysfunction in Catecholaminergic Polymorphic Ventricular Tachycardia.
Moore JP.
J Cardiovasc Electrophysiol. 2019 Jun 23. doi: 10.1111/jce.14041. [Epub ahead of print]
PMID: 31231896
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22. Management of Asymptomatic Wolff-Parkinson-White Pattern by Pediatric Electrophysiologists.
Chubb H, Campbell RM, Motonaga KS, Ceresnak SR, Dubin AM.
J Pediatr. 2019 Jun 21. pii: S0022-3476(19)30675-4. doi: 10.1016/j.jpeds.2019.05.058. [Epub ahead of print]
PMID: 31235382
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23. Genetic Testing and Cascade Screening in Pediatric Long QT Syndrome and Hypertrophic Cardiomyopathy.
Knight LM, Miller E, Kovach J, Arscott P, von Alvensleben JC, Bradley D, Valdes SO, Ware SM, Meyers L, Travers CD, Campbell RM, Etheridge SP.
Heart Rhythm. 2019 Jun 20. pii: S1547-5271(19)30572-7. doi: 10.1016/j.hrthm.2019.06.015. [Epub ahead of print]
PMID: 31229680
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24. High burden of premature ventricular contractions in structurally normal hearts: To worry or not in pediatric patients?
Sharma N, Cortez D, Imundo JR.
Ann Noninvasive Electrocardiol. 2019 Jun 14:e12663. doi: 10.1111/anec.12663. [Epub ahead of print]
PMID: 31199031
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25. The QT Interval.
Giudicessi JR, Noseworthy PA, Ackerman MJ.
Circulation. 2019 Jun 11;139(24):2711-2713. doi: 10.1161/CIRCULATIONAHA.119.039598. Epub 2019 Jun 10. No abstract available.
PMID: 31180747
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Select item 31186048
26. Catheter ablation of left posterior fascicular ventricular tachycardia in children with limited fluoroscopy exposure.
Koca S, Akdeniz C, Karacan M, Tuzcu V.
Cardiol Young. 2019 Jun 6:1-7. doi: 10.1017/S1047951119000830. [Epub ahead of print]
PMID: 31169097
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27. Calmodulin mutations and life-threatening cardiac arrhythmias: insights from the International Calmodulinopathy Registry.
Crotti L, Spazzolini C, Tester DJ, Ghidoni A, Baruteau AE, Beckmann BM, Behr ER, Bennett JS, Bezzina CR, Bhuiyan ZA, Celiker A, Cerrone M, Dagradi F, De Ferrari GM, Etheridge SP, Fatah M, Garcia-Pavia P, Al-Ghamdi S, Hamilton RM, Al-Hassnan ZN, Horie M, Jimenez-Jaimez J, Kanter RJ, Kaski JP, Kotta MC, Lahrouchi N, Makita N, Norrish G, Odland HH, Ohno S, Papagiannis J, Parati G, Sekarski N, Tveten K, Vatta M, Webster G, Wilde AAM, Wojciak J, George AL, Ackerman MJ, Schwartz PJ.
Eur Heart J. 2019 Jun 6. pii: ehz311. doi: 10.1093/eurheartj/ehz311. [Epub ahead of print]
PMID: 31170290
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28. 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. 2019 Jun 6. doi: 10.1007/s10840-019-00553-8. [Epub ahead of print]
PMID: 31172420
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29. Hidden Complexity in Routine Adult and Pediatric Arrhythmias Interpretation: The Future of Precision Electrocardiology.
Leonelli FM, De Ponti R, Drago F, Baban A, Cortez D, Griselli M, Bagliani G.
Card Electrophysiol Clin. 2019 Jun;11(2):391-404. doi: 10.1016/j.ccep.2019.03.001. Review.
PMID: 31084858
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Select item 31236178
30. Catheter ablation in ASymptomatic PEDiatric patients with ventricular preexcitation: results from the multicenter “CASPED” study.
Telishevska M, Hebe J, Paul T, Nürnberg JH, Krause U, Gebauer R, Gass M, Balmer C, Berger F, Molatta S, Emmel M, Lawrenz W, Kriebel T, Hessling G.
Clin Res Cardiol. 2019 Jun;108(6):683-690. doi: 10.1007/s00392-018-1397-x. Epub 2018 Dec 5.
PMID: 30519781
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31. Successful biventricular cardiac resynchronization therapy in a failing Fontan patient: Implications of ventriculo-ventricular interdependency in single ventricle physiology.
Udink Ten Cate FEA, Germund I, Goossens SS, Bennink G, Sreeram N.
Echocardiography. 2019 Jun;36(6):1211-1216. doi: 10.1111/echo.14376. Epub 2019 May 31.
PMID: 31148229
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32. Methamphetamine-Induced Tachydysrhythmia in an Adolescent in Diabetic Ketoacidosis.
Vo A, Nguyen MB, Song JL, Cheng AL, Festekjian A.
J Emerg Med. 2019 Jun;56(6):e111-e114. doi: 10.1016/j.jemermed.2019.01.031. Epub 2019 Mar 14.
PMID: 30879847
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Select item 30733156
33. Catheter ablation for supraventricular tachycardia in children ≤ 20 kg using an electroanatomical system.
Koca S, Akdeniz C, Tuzcu V.
J Interv Card Electrophysiol. 2019 Jun;55(1):99-104. doi: 10.1007/s10840-018-0499-8. Epub 2019 Jan 3.
PMID: 30603855
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Select item 31124990
34. Management of Asymptomatic Wolff-Parkinson-White Pattern in Young Patients: Has Anything Changed?
Raposo D, António N, Andrade H, Sousa P, Pires A, Gonçalves L.
Pediatr Cardiol. 2019 Jun;40(5):892-900. doi: 10.1007/s00246-019-02110-6. Epub 2019 May 8. Review.
PMID: 31069431
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35. Routine Detection of Atrial Fibrillation/Flutter Predicts a Worse Outcome in a Cohort of Tetralogy of Fallot Patients During 23 Years of Follow-Up.
de Castilhos GM, Ley ALG, Daudt NS, Horowitz ESK, Leiria TLL.
Pediatr Cardiol. 2019 Jun;40(5):1009-1016. doi: 10.1007/s00246-019-02106-2. Epub 2019 May 6.
PMID: 31062060
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Select item 31049646
36. Ambulatory Arrhythmia Detection with ZIO® XT Patch in Pediatric Patients: A Comparison of Devices.
Pradhan S, Robinson JA, Shivapour JK, Snyder CS.
Pediatr Cardiol. 2019 Jun;40(5):921-924. doi: 10.1007/s00246-019-02089-0. Epub 2019 Apr 1.
PMID: 30937502
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37. Cardiac Rhythm Disturbances in Heterotaxy Syndrome.
Ozawa Y, Asakai H, Shiraga K, Shindo T, Hirata Y, Hirata Y, Inuzuka R.
Pediatr Cardiol. 2019 Jun;40(5):909-913. doi: 10.1007/s00246-019-02087-2. Epub 2019 Mar 14.
PMID: 30877320
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ACHD Interventional Cardiology Featured Articles
Interventional Cardiology Featured Manuscripts of June 2019
Comparison of drug eluting versus bare metal stents for pulmonary vein stenosis in childhood.
Khan A, Qureshi AM, Justino H.
Catheter Cardiovasc Interv. 2019 May 8. doi: 10.1002/ccd.28328. [Epub ahead of print]
PMID: 31067002
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Select item 31066199
Take Home Points:
• Pulmonary vein stenosis is a challenging disease process to manage with poor outcomes when treated surgically and with transcatheter therapies.
• Drug eluting stents show promise in treatment of pulmonary vein stenosis with less lumen loss than standard bare metal stents in short term follow up despite being used in higher risk patients.
• Long term results of pulmonary vein stenosis treated with drug eluting stents remains unknown.
Commentary from Dr. Ryan Romans (Kansas City, MO), section editor of Interventional Cardiology Journal Watch: Pulmonary vein stenosis (PVS) remains a challenging lesion to manage with significant morbidity and mortality despite advances and transcatheter and surgical treatments. Transcatheter intervention with balloon angioplasty, cutting balloon angioplasty and bare-metal stent (BMS) implantation has been shown to be acutely successful but have poor medium and long-term outcomes (large modern era series with 5-year survival of 30-50%), along with high rates of restenosis. Surgical treatment is typically used in patients with ostial lesions and lesions proximal to the first order branching. Again, there is a high rate of restenosis and 5-year survival of approximately 50%. Use of the sutureless technique has improved surgical outcomes. However, lesions beyond the first order division of the pulmonary veins are typically beyond surgeon’s reach and hence not amenable to this technique. Additionally, the sutureless technique tends to be more successful in patients with postoperative pulmonary vein stenosis than in primary pulmonary vein stenosis. Given these poor outcomes, drug eluting stents (DES) are being evaluated as a potential strategy to decrease restenosis and potential loss of pulmonary veins.
Khan et al report on their retrospective single center review of all patients who underwent stent implantation for PVS (native and post-surgical) over a 24 year period. A total of 66 patients underwent stent implantation. 37 patients had 58 lesions treated with 62 BMS and 41 patients had 105 lesions treated with 111 DES. 12 patients were treated with both BMS and DES. Patients in the DES group were significantly younger (16.2 +/- 18.8 months versus 2.9 +/- 3.5 years) and smaller (7.4 +/- 3.9 kg versus 11.8 +/- 8.7 kg) than those in the BMS group. Lesion diameter increased from 2.9 +/- 1.7 mm to 7 +/- 2 mm with BMS implantation and 1.3 +/- 1 mm to 3.8 +/- 0.6 mm with DES implantation (significantly more increase with BMS). Mean gradient decreased from 10.7 +/- 6.5 mmHg to 2.1 +/- 2.8 mmHg in the BMS group and 12.6 +/- 6.2 mmHg to 2.8 +/- 2.7 mmHg in the DES group (no statistically significant difference in pre or post gradient between groups).
Follow up cardiac catheterization was performed on 44/58 lesions treated with BMS at an interval of 6.4 +/- 6.4 months and 86/105 lesions treated with DES at an interval of 6.8 +/- 7.4 months. Absolute lumen loss (mm), fractional lumen loss (% lumen diameter change from time of implantation to follow up catheterization), absolute lumen loss rate (mm/month) and fractional lumen loss rate (%/month) were all significantly lower in the DES group as shown in the table below.
Patients were divided into two groups in an attempt to determine era and treatment strategy effect. The conventional group received BMS only throughout their treatment course, with their first stent implantation prior to 2008. The novel group included all remaining patients. In this group, DES were implanted whenever possible (lesion diameter < 4mm as this was the largest DES available) and BMS were implanted only when DES was not possible (lesion >4 mm). The table below shows outcomes for the two groups. The authors note that their center trended to more transcatheter rather than surgical intervention in these patients over time. Patients in the novel therapy group tended to be younger, have more native than post-operative PVS, and have more veins involved. Despite this, 2 year survival was 74.7% in the novel therapy group and 53.1% in the conventional group.
The authors note that the mechanism of PVS appears to be related to myofibroblast proliferation. DES allow for the benefit of local drug delivery to prevent neo-intimal proliferation. They have been shown to decrease in stent stenosis in adults who undergo stent implantation for coronary artery disease. Systemic drug levels from DES have been evaluated in patients who undergo DES implantation in the ductus arteriosus (PDA stenting for ductal dependent pulmonary blood flow). Sirolimus levels were found to be in an immunosuppressive range but there were no serious infectious complications.
Analysis of patient specific outcomes is challenging as there may be a significant era effect (there was not access to DES in earlier patients). However, patients receiving drug eluting stents were younger, smaller, and had more veins involved indicating a much more aggressive disease process. Also, the diameter at first implant was smaller in the DES group than BMS group making the risk of neointimal proliferation higher. Despite these factors, lesions treated with DES had significantly less lumen loss than those with BMS at the first follow up catheterization. The authors conclude that this must be due to the local delivery of the antiproliferative agents. The study is limited by the fact that it does not take into account additional treatment strategies applied to the later (novel) cohort of patients including more aggressive recanalization of atretic veins, aggressive treatment of restoring complete patency during a single procedure, and scheduled follow up catheterization every 6 months to evaluate for in-stent stenosis and disease progression. Longer term outcomes of patients treated with DES remains unknown.
A Low Residual Pressure Gradient Yields Excellent Long-Term Outcome After Percutaneous Pulmonary Valve Implantation.
Georgiev S, Ewert P, Tanase D, Hess J, Hager A, Cleuziou J, Meierhofer C, Eicken A.
JACC Cardiovasc Interv. 2019 Jun 10. pii: S1936-8798(19)30846-5. doi: 10.1016/j.jcin.2019.03.037. [Epub ahead of print]
PMID: 31202951
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Take Home Points:
• Transcatheter pulmonary valve implantation has excellent long-term outcomes with surgery-free survival 92% at 5 years and 83% at 10 years.
• Significant risk factor for death and valve failure include an RV-PA gradient >15 mmHg. All efforts to adequate prepare the RVOT and minimize residual gradient leads to significant improvement in survival free of repeat interventions.
Commentary from Dr. Wendy Whiteside (Ann Arbor MI), section editor of Congenital Heart Disease Interventions Journal Watch: Since the introduction of percutaneous pulmonary valve implantation (PPVI) in 2000, the use and treatment indications of transcatheter valves has broadened substantially, and in many centers, has become the treatment of choice for rehabilitation of right ventricular outflow tract (RVOT) dysfunction. While there has been significant data to support the short and intermediate outcomes of PPVI, long term outcome data is somewhat limited. Georgiev add to this, a large, prospectively collected dataset of their single center experience with meticulous follow-up of up to 11 years.
Between 2006-2017, 236 patients underwent PPVI at the German Heart Center Munich. Follow-up data were available for an impressive 96% of patients (226 patients) over a median follow-up time of 3.9 years (range 2 months to 11 years). Median age at implant was 18 years, median weight 59 kg, with 14% (32 patients) weighing <30 kg. The majority of patients had surgically placed conduits or bioprosthetic valves with 26 patients (11%) having a native RVOT. Given the later introduction of the Sapiens valve, the majority of patients received Melody valves (220 patients, 92.8%) with only 16 patients (6.8%) receiving a Sapiens valve. Pre-stenting was performed in the vast majority of patients (96%) and post-dilation of the implanted valve with high-pressure balloons was performed in 63% of patients.
Seven patients died during the study period—2 patient deaths were directly related to the procedure (uncontained conduit rupture and coronary artery compression) with 4 patients dying at home 3-7 years post-procedure. Overall survival was 96% at 5 years, and 93% at 10 years. Surgery free survival was 92% at 5 years and 83% at 10 years. At the end of the study period 219 patients (93%) were alive with the initially implanted valve. The remaining 17 patients reached the endpoint of valve failure—10 patients because of stenosis due to infective endocarditis and 7 due to progressive stenosis without infective endocarditis. Of these patients, 11 received new surgical valves and 6 received a second transcatheter valve-in-valve procedure. Significant risk factors for reaching the endpoints of the study (including death or valve failure) were analyzed and the best outcome was seen in patients treated with pre-stenting and who were left with a residual RVOT gradient <15 mmHg. Survival free from intervention in patients who had pre-stenting and a post-interventional residual gradients <15 mmHg was significantly better than those without (88% vs 56%, see Figure). In follow-up MRI imaging, RV dimensions and function improved after 6 months and remained stable, and exercise capacity improved slightly after new valve implantation and remained stable at 5-year follow-up.
This study supports the excellent outcomes following PPVI, even with longer term follow-up up to 11 years. Reinterventions are uncommon and are primarily due to valve stenosis, which occurs both with and without infective endocarditis. This study also supports what many have done in practice—meticulously preparing the landing zone prior to valve implantation with pre-stenting and attention to minimizing residual stenosis—and shows significantly improved outcomes in patients left with RVOT gradient <15 mmHg.
CHD Interventions June 2019
1. Long-term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study.
Hansen RL, Naimi I, Wang H, Atallah N, Smith F, Byrum C, Kveselis D, Leonard G, Devanagondi R, Egan M.
Congenit Heart Dis. 2019 Jun 28. doi: 10.1111/chd.12788. [Epub ahead of print]
PMID: 31250555
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2. Infective Endocarditis in a Patient with Transcatheter Pulmonary Valve Implantation.
Wang C, Li YJ, Ma L, Pan X.
Int Heart J. 2019 Jun 28. doi: 10.1536/ihj.18-497. [Epub ahead of print]
PMID: 31257331 Free Article
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Select item 31264394
3. Molding the shape of congenial and structural interventional cardiology: interviews with directors of major congresses.
Goreczny S, Hijazi ZM, Qureshi SA, Carminati M, Kenny D, Morgan GJ.
Cardiol Young. 2019 Jun 27:1-7. doi: 10.1017/S104795111900132X. [Epub ahead of print]
PMID: 31244446
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4. Economic implications of outpatient cardiac catheterisation in infants with single ventricle congenital heart disease.
Colombo JN, Hainstock MR, Spaeder MC, Vergales JE.
Cardiol Young. 2019 Jun 26:1-7. doi: 10.1017/S1047951119001240. [Epub ahead of print]
PMID: 31241034
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Select item 31242054
5. Left arm structure and function late after subclavian flap repair of aortic coarctation in childhood.
Dennis MR, Cusick A, Borilovic J, Nicholson C, Derwin T, Puranik R, Celermajer DS.
Cardiol Young. 2019 Jun 20:1-6. doi: 10.1017/S1047951119000386. [Epub ahead of print]
PMID: 31218968
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Select item 31230756
6. Very long-term outcomes of transcatheter secundum atrial septal defect closure using intracardiac echocardiography without balloon sizing.
Rigatelli G, Nghia NT, Zuin M, Conte L, D’Elia K, Nanjundappa A.
Clin Radiol. 2019 Jun 20. pii: S0009-9260(19)30257-0. doi: 10.1016/j.crad.2019.05.017. [Epub ahead of print]
PMID: 31230756
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7. Percutaneous Interventional Repositioning of an Inverted Left Atrial Appendage in an Infant.
Georgiev S, Ewert P.
JACC Cardiovasc Interv. 2019 Jun 19. pii: S1936-8798(19)30754-X. doi: 10.1016/j.jcin.2019.03.021. [Epub ahead of print] No abstract available.
PMID: 31255560
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8. Transcatheter closure of atrial septal defect in adults: time-course of atrial and ventricular remodeling and effects on exercise capacity.
Stephensen SS, Ostenfeld E, Kutty S, Steding-Ehrenborg K, Arheden H, Thilén U, Carlsson M.
Int J Cardiovasc Imaging. 2019 Jun 15. doi: 10.1007/s10554-019-01647-0. [Epub ahead of print]
PMID: 31203534
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9. Transcatheter closure of sinus venosus atrial septal defect with anomalous pulmonary venous drainage: Innovative technique with long-term follow-up.
Abdullah HAM, Alsalkhi HA, Khalid KA.
Catheter Cardiovasc Interv. 2019 Jun 14. doi: 10.1002/ccd.28364. [Epub ahead of print]
PMID: 31197932
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10. First European experience of percutaneous closure of ventricular septal defects using a new CE-marked VSD occluder.
Schubert S, Kelm M, Koneti NR, Berger F.
EuroIntervention. 2019 Jun 12;15(3):e242-e243. doi: 10.4244/EIJ-D-18-00867. No abstract available.
PMID: 30585782 Free Article
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11. The experience of transcatheter closure of postoperative ventricular septal defect after total correction.
Kouakou NYN, Song J, Huh J, Kang IS.
J Cardiothorac Surg. 2019 Jun 11;14(1):104. doi: 10.1186/s13019-019-0933-8.
PMID: 31186037 Free PMC Article
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12. A Low Residual Pressure Gradient Yields Excellent Long-Term Outcome After Percutaneous Pulmonary Valve Implantation.
Georgiev S, Ewert P, Tanase D, Hess J, Hager A, Cleuziou J, Meierhofer C, Eicken A.
JACC Cardiovasc Interv. 2019 Jun 10. pii: S1936-8798(19)30846-5. doi: 10.1016/j.jcin.2019.03.037. [Epub ahead of print]
PMID: 31202951
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Select item 31103536
13. Solysafe Device Pushed Away by Amplatzer Septal Occluder After Closure of a Residual Atrial Septal Defect.
Sigler M, Horke A, Paul T, Uhlemann F.
JACC Cardiovasc Interv. 2019 Jun 10;12(11):e95-e96. doi: 10.1016/j.jcin.2019.02.026. Epub 2019 May 15. No abstract available.
PMID: 31103536
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14. Guidewire pacing during transcatheter aortic valve implantation in a patient with complex congenital heart disease.
Follansbee CW, Qureshi AM, Parekh DR, Howard TS, Kim JJ.
Pacing Clin Electrophysiol. 2019 Jun 2. doi: 10.1111/pace.13736. [Epub ahead of print]
PMID: 31155732
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15. What Is the Potential of Tissue-Engineered Pulmonary Valves in Children?
Huygens SA, Rutten-van Mölken MPMH, Noruzi A, Etnel JRG, Corro Ramos I, Bouten CVC, Kluin J, Takkenberg JJM.
Ann Thorac Surg. 2019 Jun;107(6):1845-1853. doi: 10.1016/j.athoracsur.2018.11.066. Epub 2018 Dec 31.
PMID: 30605643
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16. Percutaneous axillary artery approach for ductal stenting in critical right ventricular outflow tract lesions in the neonatal period.
Breatnach CR, Aggarwal V, Al-Alawi K, McMahon CJ, Franklin O, Prendiville T, Oslizlok P, Walsh K, Qureshi AM, Kenny D.
Catheter Cardiovasc Interv. 2019 Jun 1;93(7):1329-1335. doi: 10.1002/ccd.28302. Epub 2019 Apr 24.
PMID: 31020799
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17. First use and limitations of Magmaris® bioresorbable stenting in a low birth weight infant with native aortic coarctation.
Sallmon H, Berger F, Cho MY, Opgen-Rhein B.
Catheter Cardiovasc Interv. 2019 Jun 1;93(7):1340-1343. doi: 10.1002/ccd.28300. Epub 2019 Apr 19.
PMID: 31001884
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18. Utility of the Medtronic microvascular plug™ as a transcatheter implantable and explantable pulmonary artery flow restrictor in a swine model.
Khan AH, Hoskoppal D, Kumar TKS, Bird L, Allen K, Lloyd H, Knott-Craig CJ, Waller BR, Sathanandam S.
Catheter Cardiovasc Interv. 2019 Jun 1;93(7):1320-1328. doi: 10.1002/ccd.28162. Epub 2019 Mar 3.
PMID: 30828988
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19. Intracoronary recombinant tissue plasminogen activator in an infant with hypoplastic left heart syndrome and complete left main coronary artery thrombosis.
Mohammad Nijres B, Huntington JH, Baliulis G, Vettukattil JJ.
Catheter Cardiovasc Interv. 2019 Jun 1;93(7):E381-E384. doi: 10.1002/ccd.28092. Epub 2019 Jan 31.
PMID: 30702202
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20. Novel Use of a 3-Dimensional Virtual Model in Devising an Optimal Approach for the Closure of a Right Ventricular Pseudoaneurysm in a Patient With Complex Congenital Heart Disease.
Tredway HL, Chakravarti SB, Halpern DG, Argilla M, Bhatla P.
Circ Cardiovasc Imaging. 2019 Jun;12(6):e008466. doi: 10.1161/CIRCIMAGING.119.008466. Epub 2019 Jun 6. No abstract available.
PMID: 31167560
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Select item 30451018
21. Surgical and percutaneous pulmonary valve replacement in England over the past two decades.
Larsen SH, Dimopoulos K, Gatzoulis MA, Uebing A, Shore DF, Alonso-Gonzalez R, Kempny A.
Heart. 2019 Jun;105(12):932-937. doi: 10.1136/heartjnl-2018-314102. Epub 2019 Jan 30.
PMID: 30700516
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22. Ambulatory Fontan pressure monitoring: Results from the implantable hemodynamic monitor Fontan feasibility cohort (IHM-FFC).
Bradley EA, Jassal A, Moore-Clingenpeel M, Abraham WT, Berman D, Daniels CJ.
Int J Cardiol. 2019 Jun 1;284:22-27. doi: 10.1016/j.ijcard.2018.10.081. Epub 2018 Oct 28.
PMID: 30420147
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23. Assessment of a novel radiation reduction protocol for pediatric and adult congenital device implantation.
Clark BC, Janson CM, Ceresnak SR, Osei FA, Bonney WJ, Nappo L, Pass RH.
J Interv Card Electrophysiol. 2019 Jun;55(1):93-98. doi: 10.1007/s10840-018-0479-z. Epub 2018 Nov 9.
PMID: 30413993
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24. Balloon valvuloplasty in a dog with congenital bicuspid aortic valve and supravalvar aortic stenosis (atypical Shone’s complex).
Winter RL, Newhard DK, Taylor AR, Johnson JA, Baravik-Munsell ED.
J Vet Cardiol. 2019 Jun;23:88-95. doi: 10.1016/j.jvc.2019.02.002. Epub 2019 Mar 22.
PMID: 31174733
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25. Treatment of caudal cavoatrial junction obstruction in a dog with a balloon-expandable biliary stent.
Rohrbaugh M, Schober KE, Bonagura JD, Cheatham S, Rhinehart J, Berman D.
J Vet Cardiol. 2019 Jun;23:112-121. doi: 10.1016/j.jvc.2019.02.004. Epub 2019 Mar 28.
PMID: 31174721
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Select item 31174720
26. Transcatheter embolization of systemic-to-pulmonary artery fistulas in a dog using embolization coils and silk suture.
Winter RL, Horton JA, Newhard DK, Holland M.
J Vet Cardiol. 2019 Jun;23:104-111. doi: 10.1016/j.jvc.2019.02.003. Epub 2019 Mar 23.
PMID: 31174720
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27. Long-term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study.
Hansen RL, Naimi I, Wang H, Atallah N, Smith F, Byrum C, Kveselis D, Leonard G, Devanagondi R, Egan M.
Congenit Heart Dis. 2019 Jun 28. doi: 10.1111/chd.12788. [Epub ahead of print]
PMID: 31250555
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Select item 31257342
28. Economic implications of outpatient cardiac catheterisation in infants with single ventricle congenital heart disease.
Colombo JN, Hainstock MR, Spaeder MC, Vergales JE.
Cardiol Young. 2019 Jun 26:1-7. doi: 10.1017/S1047951119001240. [Epub ahead of print]
PMID: 31241034
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Select item 31241026
29. Salvage pulmonary thromboembolectomy for massive pulmonary embolism in a child presented with syncope and subsequent cardiac arrest.
Ak K, Birkan Y, Akalın F, Günay D.
Cardiol Young. 2019 Jun 26:1-3. doi: 10.1017/S1047951119001410. [Epub ahead of print]
PMID: 31241026
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30. Milestones in paediatric cardiology: the first therapeutic cardiac catheterisations.
Mazurak M, Kusa J.
Arch Dis Child. 2019 Jun 21. pii: archdischild-2019-316788. doi: 10.1136/archdischild-2019-316788. [Epub ahead of print] No abstract available.
PMID: 31227510
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Select item 31221232
31. Pediatric tricuspid valve replacement with transcatheter bioprosthetic valve: An alternative option in high-risk patients.
Fernandez-Doblas J, Perez-Andreu J, Betrian P, Abella RF.
Semin Thorac Cardiovasc Surg. 2019 Jun 21. pii: S1043-0679(19)30195-9. doi: 10.1053/j.semtcvs.2019.06.006. [Epub ahead of print] No abstract available.
PMID: 31233784
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32. A word of caution: diabolic behaviour of AndraStents®: inflation of supporting balloon leads to “diabolo”-misconfiguration of the stent.
Happel CM, Zunzunegui Martínez JL, Del Cerro MJ, Schranz D, Khalil M, Ballesteros F, Pardeiro CA, Bertram H, Beerbaum P, Haas NA.
Cardiol Young. 2019 Jun 20:1-5. doi: 10.1017/S1047951119001264. [Epub ahead of print]
PMID: 31218985
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33. The standing of percutaneous pulmonary valve implantation compared to surgery in a non-preselected cohort with dysfunctional right ventricular outflow tract – Reasons for failure and contraindications.
Haas NA, Vcasna R, Laser KT, Blanz U, Herrmann FE, Jakob A, Fischer M, Kanaan M, Lehner A.
J Cardiol. 2019 Jun 20. pii: S0914-5087(19)30116-9. doi: 10.1016/j.jjcc.2019.03.021. [Epub ahead of print]
PMID: 31230902
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34. Retraction Note to: Primary Draining Vein Stenting for Obstructive Total Anomalous Pulmonary Venous Connection in Neonates with Right Atrial Isomerism and Functional Single Ventricle Improves Outcome.
Kitano M, Hoashi T, Kakuta T, Fujimoto K, Miyake A, Kurosaki KI, Ichikawa H, Shiraishi I.
Pediatr Cardiol. 2019 Jun 19. doi: 10.1007/s00246-019-02131-1. [Epub ahead of print]
PMID: 31218372
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35. Cardiopulmonary Resuscitation in the Pediatric Cardiac Catheterization Laboratory: A Report From the American Heart Association’s Get With the Guidelines-Resuscitation Registry.
Lasa JJ, Alali A, Minard CG, Parekh D, Kutty S, Gaies M, Raymond TT.
Pediatr Crit Care Med. 2019 Jun 18. doi: 10.1097/PCC.0000000000002038. [Epub ahead of print]
PMID: 31232852
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36. Electrical remodeling after percutaneous atrial septal defect closure in pediatric and adult patients.
Kamphuis VP, Nassif M, Man SC, Swenne CA, Kors JA, Vink AS, Ten Harkel ADJ, Maan AC, Mulder BJM, de Winter RJ, Blom NA.
Int J Cardiol. 2019 Jun 15;285:32-39. doi: 10.1016/j.ijcard.2019.02.020. Epub 2019 Feb 23.
PMID: 30857845 Free Article
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37. Microcatheter-assisted stenting of the tortuous vertical ductus arteriosus via femoral access in a duct-dependent pulmonary circulation.
Haas NA, Fernandez-Rodriguez S, Dalla Pozza R, Fischer M, Ulrich S, Jakob A, Lehner A.
Int J Cardiol. 2019 Jun 15;285:103-107. doi: 10.1016/j.ijcard.2019.01.062. Epub 2019 Jan 25.
PMID: 30851992
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Select item 31203535
38. Transcatheter closure of atrial septal defect in adults: time-course of atrial and ventricular remodeling and effects on exercise capacity.
Stephensen SS, Ostenfeld E, Kutty S, Steding-Ehrenborg K, Arheden H, Thilén U, Carlsson M.
Int J Cardiovasc Imaging. 2019 Jun 15. doi: 10.1007/s10554-019-01647-0. [Epub ahead of print]
PMID: 31203534
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Select item 31199031
39. Computational fluid dynamics simulations as a complementary study for transcatheter endovascular stent implantation for re-coarctation of the aorta associated with minimal pressure drop: an aneurysmal ductal ampulla with aortic isthmus narrowing.
Guillot M, Ascuitto R, Ross-Ascuitto N, Mallula K, Siwik E.
Cardiol Young. 2019 Jun 14:1-9. doi: 10.1017/S1047951119000751. [Epub ahead of print]
PMID: 31198121
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Select item 31198120
40. Embolisation of ceramic-coated PDA devices into the descending thoracic aorta: probable mechanisms and retrieval strategies.
Sen S, Jain S, Dalvi B.
Cardiol Young. 2019 Jun 6:1-3. doi: 10.1017/S104795111900091X. [Epub ahead of print]
PMID: 31169093
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41. Role of cardiovascular magnetic resonance end-systolic 3D-SSFP sequence in repaired tetralogy of Fallot patients eligible for transcatheter pulmonary valve implantation.
Leonardi B, Secinaro A, Perrone MA, Curione D, Napolitano C, Gagliardi MG.
Int J Cardiovasc Imaging. 2019 Jun 3. doi: 10.1007/s10554-019-01630-9. [Epub ahead of print]
PMID: 31161492
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Select item 31113241
42. Comparison of the diameter of coronary arteries between small for gestational age (SGA) and appropriate for gestational age (AGA) newborn infants.
Karagol BS, Kundak AA, Örün UA.
J Matern Fetal Neonatal Med. 2019 Jun 3:1-6. doi: 10.1080/14767058.2019.1622668. [Epub ahead of print]
PMID: 31113241
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43. Vocal cord paralysis after transcatheter patent ductus arteriosus closure with AMPLATZERTM Vascular Plug II.
Tanidir IC, Yukcu B, Ozturk E, Guzeltas A.
Anatol J Cardiol. 2019 Jun;21(6):345-346. doi: 10.14744/AnatolJCardiol.2019.88393. No abstract available.
PMID: 31142726 Free Article
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44. Ventricular Pseudoaneurysms After Perventricular Closure of Ventricular Septal Defect.
Martinez JJ, Verma AJ, Thankavel PP, Jaquiss RD.
Ann Thorac Surg. 2019 Jun;107(6):e397-e398. doi: 10.1016/j.athoracsur.2018.09.055. Epub 2018 Nov 8.
PMID: 30414831
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Select item 30414828
45. Percutaneous device embolization of an aneurysmal pulmonary artery in an infant weighing <3 kilograms.
Reynolds K, Stapleton G, Herbert C, Mauriello D.
Catheter Cardiovasc Interv. 2019 Jun 1;93(7):1336-1339. doi: 10.1002/ccd.28292. Epub 2019 Apr 29.
PMID: 31033192
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Select item 31020799
46. Novel shunt modification with an adjustable stent-embedded “fenestrated” septal occluder in a patient with pulmonary hypertension.
Yadlapati A, Wax D, Rich S, Ricciardi MJ.
Catheter Cardiovasc Interv. 2019 Jun 1;93(7):1382-1384. doi: 10.1002/ccd.28169. Epub 2019 Mar 6.
PMID: 30838741
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Select item 30828988
47. Custom tailoring of medical implants for pulmonary artery bifurcation stenosis.
Michel-Behnke I.
Int J Cardiol. 2019 Jun 1;284:82-83. doi: 10.1016/j.ijcard.2018.11.120. Epub 2018 Nov 29. No abstract available.
PMID: 30527530
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48. Percutaneous Removal of a Cardiac Mass in a Patient with Infective Endocarditis: A Case Report.
Koney N, Benmessaoud C, Cole KY, Bulut Y, Yang EH, Moriarty JM.
J Pediatr Intensive Care. 2019 Jun;8(2):103-107. doi: 10.1055/s-0038-1675194. Epub 2018 Oct 24.
PMID: 31093464
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49. Outcomes of Reinterventions for Children with Postoperative Pulmonary Venous Restenosis.
Kai L, Xiaoyang Z, Jinghao Z, Zhongqun Z, Qi S, Xiaomin H, Zhiwei X, Jinfen L.
Pediatr Cardiol. 2019 Jun;40(5):965-972. doi: 10.1007/s00246-019-02098-z. Epub 2019 Apr 1.
PMID: 30937500
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50. [Transcatheter coil occlusion of patent ductus arteriosus and follow-up results].
Ugan Atik S, Saltık İL.
Turk Kardiyol Dern Ars. 2019 Jun;47(4):265-272. doi: 10.5543/tkda.2019.35405. Turkish.
PMID: 31219441 Free Article
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Select item 31219438
ACHD Surgery
Congenital Heart Surgery Reviews of June 2019 Manuscripts
Extracorporeal membrane oxygenation use in the first 24 hours following pediatric heart transplantation: Incidence, risk factors, and outcomes.
Godown J, Bearl DW, Thurm C, Hall M, Feingold B, Soslow JH, Mettler BA, Smith AH, Profita EL, Singh TP, Dodd DA.
Pediatr Transplant. 2019 Jun;23(4):e13414. doi: 10.1111/petr.13414. Epub 2019 Apr 11.
PMID: 30973190
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Take-Home Points:
• This study between linked SRTR and PHIS databases observed that the incidence of severe primary graft dysfunction necessitating ECMO support in pediatric heart transplant recipients was 7.9%.
• Pre-heart transplant ECMO support was the strongest risk factor for in-hospital mortality in patients with cardiomyopathy or congenital heart disease.
• Post-transplant in-hospital mortality was most strongly affected by the duration of post-transplant ECMO support, though 90% of patients were decannulated within 7 days.
• Post-heart transplant patients supported with ECMO who survive to hospital discharge demonstrated similar long-term survival as unsupported patients.
Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch: The authors aimed to combine the SRTR and PHIS databases in a linked manner to evaluate primary graft dysfunction in pediatric heart transplant recipients. Specifically, they used these databases to analyze the incidence of, risk factors for, and outcomes of pediatric heart transplant recipients who developed severe graft dysfunction requiring ECMO support within one day of the heart transplant across multiple participating institutions. Patients <18 years of age who underwent heart transplantation between 2002 and 2016 were evaluated, and ECMO billing codes were used to identify patients who required ECMO support following transplantation. Logistic regression models were used to identify risk factors for needed ECMO support following transplantation and in-hospital mortality.
A total of 2,820 patients were evaluated, and of these, 224 patients (7.9%) required post-transplant ECMO support. The median time on ECMO was 2 days with 90% of patients being decannulated within 7 days. The longest period of support was 50 days.
When comparing all patients by need for post-transplant ECMO support, patients in the ECMO group tended to be younger, have a diagnosis of congenital heart disease (CHD), be on ECMO at the time of transplant, be listed as UNOS status 1A, have a graft ischemic time >4 hours, and have other physiologic impairments (e.g., higher bilirubin level, lower creatinine clearance, and inhaled nitric oxide utilization). Multivariable logistic regression revealed factors independently associated with post-transplant ECMO support including age less <5 years and ECMO support at the time of transplant (additionally VAD support for CHD patients).
The mortality rate for post-transplant ECMO support was 14.3%. Of the 85.3% who were able to be decannulated, 87.4% survived to hospital discharge. The authors further assessed risk factors for hospital mortality, and when duration of ECMO support was excluded from the multivariable logistic regression model, only a graft ischemic time >4 hours was an independent risk factor. When duration of ECMO support was included, independent risk factors for hospital mortality included dialysis and duration of support. A diagnosis of CHD was not a significant risk factor in either case. The need for post-transplant ECMO support portended worse patient survival with 75% of supported patients surviving to hospital discharge compared to 96% of patients in the unsupported. However, the supported patients who survived demonstrated an actuarial survival curve similar to the unsupported group.
The authors elucidate several limitations of the study, particularly in how ECMO patients were identified. Other potential factors that may have affected the observed outcomes but were not able to be assessed include preservation and implantation techniques, complications of ECMO that may have affected mortality, and transplant procedure volume by center.
This large study provides a helpful snapshot of how frequently severe primary graft dysfunction occurs in pediatric patients as well as potential clinical factors that may adversely affect outcomes. The incidence of severe primary graft dysfunction occurs in pediatric patients is not trivial, especially in the youngest patients. This information may be important for counseling families about the risks of heart transplantation in this age group.
CHD Surgery June 2019
1. Impact of noonan syndrome on admissions for pediatric cardiac surgery.
Kriz C, Flores S, Villarreal EG, Bronicki RA, Loomba RS.
Minerva Pediatr. 2019 Jun 28. doi: 10.23736/S0026-4946.19.05461-6. [Epub ahead of print]
PMID: 31264394
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2. “Double-barrel endocarditis”.
Irabien Á, Gil-Jaurena JM, Pita A, Pérez-Caballero R, González-Pinto Á.
J Card Surg. 2019 Jun 27. doi: 10.1111/jocs.14141. [Epub ahead of print]
PMID: 31250478
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3. A simple measure of the extent of Ebstein valve rotation with cardiovascular magnetic resonance gives a practical guide to feasibility of surgical cone reconstruction.
Hughes ML, Bonello B, Choudhary P, Marek J, Tsang V.
J Cardiovasc Magn Reson. 2019 Jun 27;21(1):34. doi: 10.1186/s12968-019-0546-3.
PMID: 31242903 Free Article
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Select item 31254511
4. Outcomes of Repair of Kommerell’s Diverticulum.
Vinnakota A, Idrees JJ, Rosinski BF, Tucker NJ, Roselli EE, Pettersson GB, Vekstein AM, Stewart RD, Raja S, Svensson LG.
Ann Thorac Surg. 2019 Jun 26. pii: S0003-4975(19)30892-6. doi: 10.1016/j.athoracsur.2019.04.122. [Epub ahead of print]
PMID: 31254511
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5. Neuroimaging findings in newborns with congenital heart disease prior to surgery: an observational study.
Kelly CJ, Arulkumaran S, Tristão Pereira C, Cordero-Grande L, Hughes EJ, Teixeira RPAG, Steinweg JK, Victor S, Pushparajah K, Hajnal JV, Simpson J, Edwards AD, Rutherford MA, Counsell SJ.
Arch Dis Child. 2019 Jun 26. pii: archdischild-2018-314822. doi: 10.1136/archdischild-2018-314822. [Epub ahead of print]
PMID: 31243012 Free Article
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6. Pulmonary artery growth after Modified Blalock-Taussig shunt: A single center experience.
Zhou T, Wang Y, Liu J, Wang Y, Wang Y, Chen S, Zhou C, Dong N.
Asian J Surg. 2019 Jun 26. pii: S1015-9584(19)30284-2. doi: 10.1016/j.asjsur.2019.06.002. [Epub ahead of print]
PMID: 31255465 Free Article
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7. Surgical Treatment for Kommerell’s Diverticulum Associated with a Right-Sided Aortic Arch and an Aberrant Left Subclavian Artery: Endovascular or Hybrid.
Morishita A, Tomioka H, Katahira S, Hoshino T, Hanzawa K.
Ann Vasc Dis. 2019 Jun 25;12(2):228-232. doi: 10.3400/avd.cr.18-00170.
PMID: 31275480 Free PMC Article
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8. Linking world bank development indicators and outcomes of congenital heart surgery in low-income and middle-income countries: retrospective analysis of quality improvement data.
Rahman S, Zheleva B, Cherian KM, Christenson JT, Doherty KE, de Ferranti D, Gauvreau K, Hickey PA, Kumar RK, Kupiec JK, Novick WM, Sandoval NF, Jenkins KJ.
BMJ Open. 2019 Jun 22;9(6):e028307. doi: 10.1136/bmjopen-2018-028307.
PMID: 31230022 Free Article
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9. Predicting Post-Fontan Length of Stay: The Limits of Measured Variables.
Martin BJ, McBrien A, Marchak BE, Atallah J, Al Aklabi M, Mackie AS.
Pediatr Cardiol. 2019 Jun 22. doi: 10.1007/s00246-019-02134-y. [Epub ahead of print]
PMID: 31230092
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10. Doubly Committed Subarterial Ventricular Septal Defects Closure Using Minimal Mid-Partial Sternotomy.
Huang C, Yu T, Zhang X.
Ann Thorac Cardiovasc Surg. 2019 Jun 20;25(3):172-175. doi: 10.5761/atcs.oa.18-00184. Epub 2018 Dec 11.
PMID: 30541999 Free PMC Article
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11. Reconstruction of the pulmonary artery by a novel biodegradable conduit engineered with perinatal stem cell-derived vascular smooth muscle cells enables physiological vascular growth in a large animal model of congenital heart disease.
Ghorbel MT, Jia H, Swim MM, Iacobazzi D, Albertario A, Zebele C, Holopherne-Doran D, Hollander A, Madeddu P, Caputo M.
Biomaterials. 2019 Jun 20;217:119284. doi: 10.1016/j.biomaterials.2019.119284. [Epub ahead of print]
PMID: 31255979 Free Article
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12. A Comparative Analysis of Equations to Estimate Patient Energy Requirements Following Cardiopulmonary Bypass for Correction of Congenital Heart Disease.
Roebuck N, Fan CS, Floh A, Harris ZL, Mazwi ML.
JPEN J Parenter Enteral Nutr. 2019 Jun 17. doi: 10.1002/jpen.1610. [Epub ahead of print]
PMID: 31209916
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13. Pediatric Cardiac Surgery in Low-and Middle-Income Countries: Present Status and Need for a Paradigm Shift.
Murala JSK, Karl TR, Pezzella AT.
Front Pediatr. 2019 Jun 13;7:214. doi: 10.3389/fped.2019.00214. eCollection 2019.
PMID: 31263686 Free PMC Article
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14. Surgical ventricular restoration and mitral valve replacement in a pediatric patient with complex congenital heart disease and malignant ventricular arrhythmias.
Saitto G, Castelvecchio S, Arcidiacono C, Menicanti L.
J Thorac Cardiovasc Surg. 2019 Jun 13. pii: S0022-5223(19)31034-7. doi: 10.1016/j.jtcvs.2019.04.095. [Epub ahead of print] No abstract available.
PMID: 31204132
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Select item 31206501
15. Plasma Neutrophil Gelatinase-Associated Lipocalin Is Associated With Acute Kidney Injury and Clinical Outcomes in Neonates Undergoing Cardiopulmonary Bypass.
Schroeder LW, Buckley JR, Stroud RE, Martin RH, Nadeau EK, Barrs R, Graham EM.
Pediatr Crit Care Med. 2019 Jun 13. doi: 10.1097/PCC.0000000000002035. [Epub ahead of print]
PMID: 31206501
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16. Repair of partial atrioventricular canal defect in adult patients: two-year follow-up outcomes of a retrospective study.
Song L, Ling Y, An Q.
J Cardiothorac Surg. 2019 Jun 11;14(1):106. doi: 10.1186/s13019-019-0931-x.
PMID: 31186038 Free PMC Article
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17. [Risk factors for acute kidney injury after pediatric congenital heart surgery].
Yang YQ, Yuan LY, Chen S, Cui MB, Liu L, Zha Y.
Zhonghua Yi Xue Za Zhi. 2019 Jun 11;99(22):1717-1721. doi: 10.3760/cma.j.issn.0376-2491.2019.22.007. Chinese.
PMID: 31216818
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18. Successful combined bilateral lung transplant and congenital heart disease repair in a pediatric patient.
Lozano-Balseiro M, Martínez-Bendayán I, Pato-López O, De la Torre-Bravos M, Fynn-Thompson F, Bautista-Hernández V.
Rev Esp Cardiol (Engl Ed). 2019 Jun 8. pii: S1885-5857(19)30152-5. doi: 10.1016/j.rec.2019.03.013. [Epub ahead of print] English, Spanish. No abstract available.
PMID: 31182299
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19. Humanitarian Mission in Pediatric Cardiothoracic Surgery: A Recipient’s Perspective.
Mohd Zain MR, Shamsuddin AM, Mamat AZ, Mokhtar AM, Ali S, Chen YC, Corno AF.
Front Pediatr. 2019 Jun 7;7:230. doi: 10.3389/fped.2019.00230. eCollection 2019.
PMID: 31231625 Free PMC Article
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20. Comparison of inhospital outcomes of pediatric heart transplantation between single ventricle congenital heart disease and cardiomyopathy.
Bradford TT, Daily JA, Lang SM, Gossett JM, Tang X, Collins RT 2nd.
Pediatr Transplant. 2019 Jun 6:e13495. doi: 10.1111/petr.13495. [Epub ahead of print]
PMID: 31169342
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Select item 31176798
21. State of the Art: Tissue Engineering in Congenital Heart Surgery.
Boyd R, Parisi F, Kalfa D.
Semin Thorac Cardiovasc Surg. 2019 Jun 6. pii: S1043-0679(19)30069-3. doi: 10.1053/j.semtcvs.2019.05.023. [Epub ahead of print]
PMID: 31176798
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22. Catheter-associated bloodstream infection incidence and outcomes in congenital cardiac surgery.
Haughey BS, White SC, Seckeler MD.
Congenit Heart Dis. 2019 Jun 5. doi: 10.1111/chd.12809. [Epub ahead of print]
PMID: 31166073
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23. Cardiopulmonary bypass method with blood delivery via femoral artery cannulation for pediatric aortic arch repair.
Yamamoto T, Schindler E.
Paediatr Anaesth. 2019 Jun 5. doi: 10.1111/pan.13681. [Epub ahead of print]
PMID: 31166055
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24. Gastrointestinal complications following on-pump cardiac surgery-A propensity matched analysis.
Marsoner K, Voetsch A, Lierzer C, Sodeck GH, Fruhwald S, Dapunt O, Mischinger HJ, Kornprat P.
PLoS One. 2019 Jun 5;14(6):e0217874. doi: 10.1371/journal.pone.0217874. eCollection 2019.
PMID: 31166962 Free PMC Article
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25. Effect of ultrafiltration on extravascular lung water assessed by lung ultrasound in children undergoing cardiac surgery: a randomized prospective study.
Elayashy M, Madkour MA, Mahmoud AAA, Hosny H, Hussein A, Nabih A, Lofty A, Hamza HM, Hassan P, Wagih M, Mohamed AK.
BMC Anesthesiol. 2019 Jun 4;19(1):93. doi: 10.1186/s12871-019-0771-1.
PMID: 31159739 Free PMC Article
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26. Perioperative serum albumin and its influence on clinical outcomes in neonates and infants undergoing cardiac surgery with cardiopulmonary bypass: a multi-centre retrospective study.
Henry BM, Borasino S, Ortmann L, Figueroa M, Rahman AKMF, Hock KM, Briceno-Medina M, Alten JA.
Cardiol Young. 2019 Jun 4:1-7. doi: 10.1017/S1047951119000738. [Epub ahead of print]
PMID: 31159896
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27. Ventricular-Arterial Coupling in Children and Infants With Congenital Heart Disease After Cardiopulmonary Bypass Surgery: Observational Study.
Marinari E, Rizza A, Iacobelli R, Iodice F, Favia I, Romagnoli S, Di Chiara L, Ricci Z.
Pediatr Crit Care Med. 2019 Jun 4. doi: 10.1097/PCC.0000000000001982. [Epub ahead of print]
PMID: 31169761
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28. Short stature among children undergoing cardiac surgery for congenital heart defects.
Le Roy C, Larios G, Clavería C, Springmüller D.
Arch Argent Pediatr. 2019 Jun 1;117(3):e211-e217. doi: 10.5546/aap.2019.eng.e211. English, Spanish.
PMID: 31063303 Free Article
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29. Extracorporeal membrane oxygenation in pediatric cardiovascular care: Experience of a center in Argentina.
Pilan ML, Moreno G, Krynski M, Ponce G, Montonati M, Lenz M, Rodríguez R, Cornelis J, Barreta J, Magliola R, Quiroz R, García Delucis P.
Arch Argent Pediatr. 2019 Jun 1;117(3):157-163. doi: 10.5546/aap.2019.eng.157. English, Spanish.
PMID: 31063299 Free Article
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Select item 30536407
30. Current Challenges and Emergent Technologies for Manufacturing Artificial Right Ventricle to Pulmonary Artery (RV-PA) Cardiac Conduits.
Manavitehrani I, Ebrahimi P, Yang I, Daly S, Schindeler A, Saxena A, Little DG, Fletcher DF, Dehghani F, Winlaw DS.
Cardiovasc Eng Technol. 2019 Jun;10(2):205-215. doi: 10.1007/s13239-019-00406-5. Epub 2019 Feb 14. Review.
PMID: 30767113
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31. Short-term outcomes of en bloc combined heart and liver transplantation in the failing Fontan.
Vaikunth SS, Concepcion W, Daugherty T, Fowler M, Lutchman G, Maeda K, Rosenthal DN, Teuteberg J, Woo YJ, Lui GK.
Clin Transplant. 2019 Jun;33(6):e13540. doi: 10.1111/ctr.13540. Epub 2019 Apr 11.
PMID: 30891780
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32. Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.
Slieker MG, Meza JM, Devlin PJ, Burch PT, Karamlou T, DeCampli WM, McCrindle BW, Williams WG, Morgan CT, Fleishman CE, Mertens L.
Eur Heart J Cardiovasc Imaging. 2019 Jun 1;20(6):658-667. doi: 10.1093/ehjci/jey141.
PMID: 30339206
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Select item 30624624
33. Surgical repair of a ruptured congenital sinus of Valsalva aneurysm: 10-year experience with 286 cases.
Luo X, Zhang D, Li B, Qi L, Gong L, Tang Y, Sun H.
Eur J Cardiothorac Surg. 2019 Jun 1;55(6):1211-1218. doi: 10.1093/ejcts/ezy437.
PMID: 30624624
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Select item 30590475
34. Acquired von Willebrand syndrome in paediatric patients during mechanical circulatory support.
Kubicki R, Stiller B, Kroll J, Siepe M, Beyersdorf F, Benk C, Höhn R, Grohmann J, Fleck T, Zieger B.
Eur J Cardiothorac Surg. 2019 Jun 1;55(6):1194-1201. doi: 10.1093/ejcts/ezy408.
PMID: 30590475
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Select item 30561567
35. Preoperative predictability of right ventricular failure following surgery for Ebstein’s anomaly.
Mrad Agua K, Burri M, Cleuziou J, Beran E, Meierhofer C, Nagdyman N, Lange R.
Eur J Cardiothorac Surg. 2019 Jun 1;55(6):1187-1193. doi: 10.1093/ejcts/ezy425.
PMID: 30561567
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36. The conversion to Rastelli’s type operation from Patrick-McGoon’s procedure of an adult with Taussig-Bing heart: a case report.
Fujiwara K, Yoshizawa K, Ohno N, Sakazaki H.
Gen Thorac Cardiovasc Surg. 2019 Jun;67(6):551-553. doi: 10.1007/s11748-018-0942-x. Epub 2018 Jun 11.
PMID: 29948796
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37. Ventricular assist device in a patient with congenitally corrected transposition of the great arteries and situs inversus totalis.
Wieloch R, Sipahi NF, Boeken U, Albert A, Akhyari P, Westenfeld R, Lichtenberg A, Saeed D.
Int J Artif Organs. 2019 Jun;42(6):321-322. doi: 10.1177/0391398818823768. Epub 2019 Jan 10.
PMID: 30630389
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Select item 30420147
38. Minimally invasive surgery for atrial septal defects: a 20-year experience at a single centre.
Vida VL, Zanotto L, Zanotto L, Tessari C, Padalino MA, Zanella F, Pittarello D, Stellin G.
Interact Cardiovasc Thorac Surg. 2019 Jun 1;28(6):961-967. doi: 10.1093/icvts/ivz017.
PMID: 30726938
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39. Left thorax approach to repair doubly committed juxta-arterial ventricular septal defect with Da Vinci robotic system.
Hu Y, Deng J, Zhao S, Zhong Q.
J Card Surg. 2019 Jun;34(6):495-498. doi: 10.1111/jocs.14036. Epub 2019 Apr 13.
PMID: 30981213
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Select item 31159401
40. Incidence, predictors, and outcomes after severe primary graft dysfunction in pediatric heart transplant recipients.
Profita EL, Gauvreau K, Rycus P, Thiagarajan R, Singh TP.
J Heart Lung Transplant. 2019 Jun;38(6):601-608. doi: 10.1016/j.healun.2019.01.1310. Epub 2019 Jan 24.
PMID: 30733156
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Select item 30413993
41. Early Neurodevelopmental Outcomes in Children Supported with ECMO for Cardiac Indications.
Sadhwani A, Cheng H, Stopp C, Rollins CK, Jolley MA, Dunbar-Masterson C, Wypij D, Newburger J, Ware J, Thiagarajan RR.
Pediatr Cardiol. 2019 Jun;40(5):1072-1083. doi: 10.1007/s00246-019-02115-1. Epub 2019 May 11.
PMID: 31079193
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Select item 31065757
42. Surgical Management of Tetralogy of Fallot with Unilateral Absence of the Pulmonary Artery.
Yang T, Sun J, Xu H, Yan J, Li S, Wang Q, Zhang J.
Pediatr Cardiol. 2019 Jun;40(5):1026-1034. doi: 10.1007/s00246-019-02109-z. Epub 2019 May 2.
PMID: 31049646
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Select item 30982075
43. Reverse Remodeling of Pulmonary Arterioles After Pulmonary Artery Banding in Patients ≥ 2 Years Old with Severe Pulmonary Arterial Hypertension and Congenital Heart Disease.
Liu C, Cheng P, Liu A, Li B, Yang Y, Wang Z, Su J.
Pediatr Cardiol. 2019 Jun;40(5):958-964. doi: 10.1007/s00246-019-02097-0. Epub 2019 Apr 13.
PMID: 30982075
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44. Morbidity During Adult Congenital Heart Surgery Admissions.
Setton M, He W, Benavidez OJ.
Pediatr Cardiol. 2019 Jun;40(5):987-993. doi: 10.1007/s00246-019-02103-5. Epub 2019 Apr 11.
PMID: 30976885
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45. Readmissions Following Congenital Heart Surgery in Infants and Children.
Benavidez OJ, He W, Lahoud-Rahme M.
Pediatr Cardiol. 2019 Jun;40(5):994-1000. doi: 10.1007/s00246-019-02104-4. Epub 2019 Apr 11.
PMID: 30976884
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46. Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease.
Foulks MG, Meyer RML, Gold JI, Herrington CS, Kallin K, Menteer J.
Pediatr Cardiol. 2019 Jun;40(5):943-949. doi: 10.1007/s00246-019-02093-4. Epub 2019 Apr 1.
PMID: 30937501
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47. Is Sutureless Technique Beneficial in the Primary Repair of Total Anomalous Pulmonary Venous Connection? A Systematic Review and Meta-Analysis.
Wu Y, Xin L, Zhou Y, Kuang H, Jin X, Li Y, Wu C.
Pediatr Cardiol. 2019 Jun;40(5):881-891. doi: 10.1007/s00246-018-1948-y. Epub 2018 Sep 8. Review.
PMID: 30196381
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48. Common Arterial Trunk: Physiology, Imaging, and Management.
Chikkabyrappa S, Mahadevaiah G, Buddhe S, Alsaied T, Tretter J.
Semin Cardiothorac Vasc Anesth. 2019 Jun;23(2):225-236. doi: 10.1177/1089253218821382. Epub 2018 Dec 29.
PMID: 30596352
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49. Efficacy of different analgesic or sedative drug therapies in pediatric patients with congenital heart disease undergoing surgery: a network meta-analysis.
Liu RZ, Li BT, Zhao GQ.
World J Pediatr. 2019 Jun;15(3):235-245. doi: 10.1007/s12519-019-00252-4. Epub 2019 Apr 23.
PMID: 31016566
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50. Patient-specific three-dimensional printed heart models benefit preoperative planning for complex congenital heart disease.
Xu JJ, Luo YJ, Wang JH, Xu WZ, Shi Z, Fu JZ, Shu Q.
World J Pediatr. 2019 Jun;15(3):246-254. doi: 10.1007/s12519-019-00228-4. Epub 2019 Feb 22.
PMID: 30796731
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51. Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity.
DiLorenzo MP, Santo A, Rome JJ, Zhang H, Faerber JA, Mercer-Rosa L, Hopper RK.
Semin Thorac Cardiovasc Surg. 2019 Summer;31(2):266-273. doi: 10.1053/j.semtcvs.2018.09.027. Epub 2018 Sep 29.
PMID: 30278272
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52. Postoperative Serum Troponin Trends in Infants Undergoing Cardiac Surgery.
Su JA, Kumar SR, Mahmoud H, Bowdish ME, Toubat O, Wood JC, Kung GC.
Semin Thorac Cardiovasc Surg. 2019 Summer;31(2):244-251. doi: 10.1053/j.semtcvs.2018.08.010. Epub 2018 Sep 6.
PMID: 30194978
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53. A long-term mechanical cavopulmonary support device for patients with Fontan circulation.
Granegger M, Thamsen B, Hubmann EJ, Choi Y, Beck D, Valsangiacomo Buechel E, Voutat M, Schweiger M, Meboldt M, Hübler M.
Med Eng Phys. 2019 Jun 29. pii: S1350-4533(19)30111-0. doi: 10.1016/j.medengphy.2019.06.017. [Epub ahead of print]
PMID: 31266678
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54. An investigation of layer-specific tissue biomechanics of porcine atrioventricular valve anterior leaflets.
Kramer KE, Ross CJ, Laurence DW, Babu AR, Wu Y, Towner RA, Mir A, Burkhart HM, Holzapfel GA, Lee CH.
Acta Biomater. 2019 Jun 28. pii: S1742-7061(19)30471-4. doi: 10.1016/j.actbio.2019.06.049. [Epub ahead of print]
PMID: 31260822
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Select item 31250782
55. Anomalous single trunk coronary artery from the right pulmonary artery.
Donmez YN, Aykan HH, Yilmaz M.
Cardiol Young. 2019 Jun 28:1-3. doi: 10.1017/S1047951119001379. [Epub ahead of print]
PMID: 31250782
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Select item 31250773
56. Impact of noonan syndrome on admissions for pediatric cardiac surgery.
Kriz C, Flores S, Villarreal EG, Bronicki RA, Loomba RS.
Minerva Pediatr. 2019 Jun 28. doi: 10.23736/S0026-4946.19.05461-6. [Epub ahead of print]
PMID: 31264394
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Select item 31250930
57. Impact of Left Atrial Decompression on Patient Outcomes During Pediatric Venoarterial Extracorporeal Membrane Oxygenation: A Case-Control Study.
Alghanem F, Balasubramanian S, Zampi JD.
Pediatr Cardiol. 2019 Jun 27. doi: 10.1007/s00246-019-02147-7. [Epub ahead of print]
PMID: 31250046
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58. Endothelial-Dependent Vasomotor Dysfunction in Infants After Cardiopulmonary Bypass.
Krispinsky LT, Stark RJ, Parra DA, Luan L, Bichell DP, Pietsch JB, Lamb FS.
Pediatr Crit Care Med. 2019 Jun 26. doi: 10.1097/PCC.0000000000002049. [Epub ahead of print]
PMID: 31246738
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Select item 31242221
59. Standardized Approach to Intervention for Intestinal Malrotation in Single Ventricle Patients with Heterotaxy Syndrome: Impact on Interstage Attrition and Time to Superior Cavopulmonary Connection.
Mathis L, Shafer B, Crethers D, Polime Nakos AC.
Pediatr Cardiol. 2019 Jun 25. doi: 10.1007/s00246-019-02136-w. [Epub ahead of print]
PMID: 31240371
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Select item 31240370
60. Linking world bank development indicators and outcomes of congenital heart surgery in low-income and middle-income countries: retrospective analysis of quality improvement data.
Rahman S, Zheleva B, Cherian KM, Christenson JT, Doherty KE, de Ferranti D, Gauvreau K, Hickey PA, Kumar RK, Kupiec JK, Novick WM, Sandoval NF, Jenkins KJ.
BMJ Open. 2019 Jun 22;9(6):e028307. doi: 10.1136/bmjopen-2018-028307.
PMID: 31230022 Free Article
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61. Predicting Post-Fontan Length of Stay: The Limits of Measured Variables.
Martin BJ, McBrien A, Marchak BE, Atallah J, Al Aklabi M, Mackie AS.
Pediatr Cardiol. 2019 Jun 22. doi: 10.1007/s00246-019-02134-y. [Epub ahead of print]
PMID: 31230092
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62. Use of bivalirudin as a primary anticoagulant in a child during Berlin Heart EXCOR ventricular assist device support.
Medar SS, Hsu DT, Lamour JM, Bansal N, Peek GJ.
Perfusion. 2019 Jun 21:267659119855853. doi: 10.1177/0267659119855853. [Epub ahead of print]
PMID: 31223064
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63. Thoracic Aortic, Aortic Valve and Mitral Valve Surgery in Pediatric and Young Adult Patients with Marfan Syndrome: Characteristics and Outcomes.
Knadler JJ, LeMaire S, McKenzie ED, Moffett B, Morris SA.
Semin Thorac Cardiovasc Surg. 2019 Jun 21. pii: S1043-0679(19)30196-0. doi: 10.1053/j.semtcvs.2019.06.005. [Epub ahead of print]
PMID: 31233783
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64. Arterial Pressure Monitoring in Pediatric Patients Undergoing Cardiac Surgery: An Observational Study Comparing Invasive and Non-invasive Measurements.
Ricci Z, Brogi J, De Filippis S, Caccavelli R, Morlacchi M, Romagnoli S.
Pediatr Cardiol. 2019 Jun 20. doi: 10.1007/s00246-019-02137-9. [Epub ahead of print]
PMID: 31222376
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65. Perioperative Assessment of Cerebral Oxygen Metabolism in Infants With Functionally Univentricular Hearts Undergoing the Bidirectional Cavopulmonary Connection.
Neunhoeffer F, Michel J, Nehls W, Marx M, Mustafi M, Magunia H, Schuhmann M, Schlensak C, Hofbeck M.
Pediatr Crit Care Med. 2019 Jun 19. doi: 10.1097/PCC.0000000000002016. [Epub ahead of print]
PMID: 31232848
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66. Modeling sensitivity and uncertainties in platelet activation models applied on centrifugal pumps for extracorporeal life support.
Fuchs G, Berg N, Broman LM, Prahl Wittberg L.
Sci Rep. 2019 Jun 19;9(1):8809. doi: 10.1038/s41598-019-45121-2.
PMID: 31217491 Free PMC Article
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67. Ecthyma gangrenosum in a 3-year-old boy post-heart transplantation.
Fukui KO, Shoji K, Nagai Y, Shindo T, Hikosaka M, Kuwahara K, Ishiguro A, Miyairi I.
Transpl Infect Dis. 2019 Jun 19:e13131. doi: 10.1111/tid.13131. [Epub ahead of print]
PMID: 31216602
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68. Surgical Outcomes in Syndromic Tetralogy of Fallot: A Systematic Review and Evidence Quality Assessment.
Athanasiadis DI, Mylonas KS, Kasparian K, Ziogas IA, Vlachopoulou D, Sfyridis PG, Schizas D, Spartalis E, Nikiteas N, Hemmati P, Kalangos A, Avgerinos DV.
Pediatr Cardiol. 2019 Jun 18. doi: 10.1007/s00246-019-02133-z. [Epub ahead of print] Review.
PMID: 31214731
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69. A Comparative Analysis of Equations to Estimate Patient Energy Requirements Following Cardiopulmonary Bypass for Correction of Congenital Heart Disease.
Roebuck N, Fan CS, Floh A, Harris ZL, Mazwi ML.
JPEN J Parenter Enteral Nutr. 2019 Jun 17. doi: 10.1002/jpen.1610. [Epub ahead of print]
PMID: 31209916
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70. Quality of life in young patients after cone reconstruction for Ebstein anomaly.
Sessions KL, Van Dorn C, Dearani JA, Warring S, Leopold K, Wackel PL, Cetta F, Johnson JN.
Cardiol Young. 2019 Jun 14:1-5. doi: 10.1017/S1047951119000726. [Epub ahead of print]
PMID: 31198119
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71. Long-term Outcomes of the Fontan Operation in Patients with Total Anomalous Pulmonary Venous Drainage.
Yong MS, Zhu MZL, du Plessis K, Weintraub RG, Hornung T, Winlaw D, Alphonso N, d’Udekem Y, Konstantinov IE.
Ann Thorac Surg. 2019 Jun 12. pii: S0003-4975(19)30824-0. doi: 10.1016/j.athoracsur.2019.04.073. [Epub ahead of print]
PMID: 31201782
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72. Serial Changes in Right Ventricular Systolic Function Among Rejection-Free Children and Young Adults After Heart Transplantation.
Harrington JK, Richmond ME, Woldu KL, Pasumarti N, Kobsa S, Freud LR.
J Am Soc Echocardiogr. 2019 Jun 12. pii: S0894-7317(19)30614-5. doi: 10.1016/j.echo.2019.04.413. [Epub ahead of print]
PMID: 31202590
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73. At Ross-Konno Operation in an Infant with a Quadricuspid Pulmonary Valve and Anomalous Aortic Origin of the Right Coronary Artery.
Bayle KM, Boston U, Sainathan S, Naik R, Knott-Craig CJ.
Ann Thorac Surg. 2019 Jun 7. pii: S0003-4975(19)30739-8. doi: 10.1016/j.athoracsur.2019.04.061. [Epub ahead of print]
PMID: 31181204
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74. Regenerating Hearts by Arresting Development With Hypothyroidism.
Lal S, Kühn B.
Circ Res. 2019 Jun 7;124(12):1725-1726. doi: 10.1161/CIRCRESAHA.119.315106. Epub 2019 Jun 6. No abstract available.
PMID: 31170042
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75. New Versatile Dual-Support Pediatric Heart Pump.
Fox C, Sarkisyan H, Stevens R, Arabia F, Fischer W, Rossano J, Throckmorton A.
Artif Organs. 2019 Jun 4. doi: 10.1111/aor.13507. [Epub ahead of print]
PMID: 31162850
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76. Perioperative serum albumin and its influence on clinical outcomes in neonates and infants undergoing cardiac surgery with cardiopulmonary bypass: a multi-centre retrospective study.
Henry BM, Borasino S, Ortmann L, Figueroa M, Rahman AKMF, Hock KM, Briceno-Medina M, Alten JA.
Cardiol Young. 2019 Jun 4:1-7. doi: 10.1017/S1047951119000738. [Epub ahead of print]
PMID: 31159896
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77. Ross operation early and mid-term results in children and young adults.
Korun O, Selcuk A, Yurdakök O, Altın HF, Cicek M, Kılıç Y, Kudsioglu ST, Bulut MO, Aydemir NA, Sasmazel A.
Anatol J Cardiol. 2019 Jun;22(1):21-25. doi: 10.14744/AnatolJCardiol.2019.45751.
PMID: 31264658 Free Article
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78. Complete Atrioventricular Septal Defect: Evolution of Results in a Single Center During 50 Years.
Airaksinen R, Mattila I, Jokinen E, Salminen J, Puntila J, Lehtinen ML, Ojala T, Rautiainen P, Rahkonen O, Suominen P, Pätilä T.
Ann Thorac Surg. 2019 Jun;107(6):1824-1830. doi: 10.1016/j.athoracsur.2019.01.020. Epub 2019 Feb 13.
PMID: 30771324
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79. Readmission After Pediatric Cardiothoracic Surgery: An Analysis of The Society of Thoracic Surgeons Database.
Kogon BE, Oster ME, Wallace A, Chiswell K, Hill KD, Cox ML, Jacobs JP, Pasquali S, Karamlou T, Jacobs ML.
Ann Thorac Surg. 2019 Jun;107(6):1816-1823. doi: 10.1016/j.athoracsur.2019.01.009. Epub 2019 Feb 8.
PMID: 30742819
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80. Damus-Kaye-Stansel: Valuable Option for Retraining of Left Ventricle in Late Arterial Switch for Transposition of the Great Arteries.
Bishnoi AK, Patel K, Agrawal P, Ananthanarayanan C, Garg P, Pandya H.
Ann Thorac Surg. 2019 Jun;107(6):e389-e391. doi: 10.1016/j.athoracsur.2018.10.044. Epub 2018 Nov 24.
PMID: 30481515
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81. Hybrid Palliation as a Bridge to Biventricular Repair in Critical Aortic Stenosis With Coarctation.
Handler SS, Buelow M, Bergstrom C, Frommelt PC, Hraska V.
Ann Thorac Surg. 2019 Jun;107(6):e395-e396. doi: 10.1016/j.athoracsur.2018.09.051. Epub 2018 Nov 8.
PMID: 30414828
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82. Age-Dependent Impact of Pre-Transplant Intensive Care Unit Stay on Mortality in Heart Transplant Recipients.
Sims T, Tumin D, Hayes D, Tobias JD.
Cardiol Res. 2019 Jun;10(3):157-164. doi: 10.14740/cr870. Epub 2019 Jun 7.
PMID: 31236178 Free PMC Article
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83. Novel Use of a 3-Dimensional Virtual Model in Devising an Optimal Approach for the Closure of a Right Ventricular Pseudoaneurysm in a Patient With Complex Congenital Heart Disease.
Tredway HL, Chakravarti SB, Halpern DG, Argilla M, Bhatla P.
Circ Cardiovasc Imaging. 2019 Jun;12(6):e008466. doi: 10.1161/CIRCIMAGING.119.008466. Epub 2019 Jun 6. No abstract available.
PMID: 31167560
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84. Short-term outcomes of en bloc combined heart and liver transplantation in the failing Fontan.
Vaikunth SS, Concepcion W, Daugherty T, Fowler M, Lutchman G, Maeda K, Rosenthal DN, Teuteberg J, Woo YJ, Lui GK.
Clin Transplant. 2019 Jun;33(6):e13540. doi: 10.1111/ctr.13540. Epub 2019 Apr 11.
PMID: 30891780
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85. Pre-intervention morphologic and functional echocardiographic characteristics of neonates with critical left heart obstruction: a Congenital Heart Surgeons Society (CHSS) inception cohort study.
Slieker MG, Meza JM, Devlin PJ, Burch PT, Karamlou T, DeCampli WM, McCrindle BW, Williams WG, Morgan CT, Fleishman CE, Mertens L.
Eur Heart J Cardiovasc Imaging. 2019 Jun 1;20(6):658-667. doi: 10.1093/ehjci/jey141.
PMID: 30339206
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86. Preoperative predictability of right ventricular failure following surgery for Ebstein’s anomaly.
Mrad Agua K, Burri M, Cleuziou J, Beran E, Meierhofer C, Nagdyman N, Lange R.
Eur J Cardiothorac Surg. 2019 Jun 1;55(6):1187-1193. doi: 10.1093/ejcts/ezy425.
PMID: 30561567
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87. The Janus-faced Fontan circulation: unravelling its elusive pathophysiology.
Ridderbos FS, Hoendermis ES, Berger RMF, van Melle JP.
Eur J Heart Fail. 2019 Jun;21(6):810-812. doi: 10.1002/ejhf.1415. Epub 2019 Feb 7. No abstract available.
PMID: 30730092
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88. Rescue Nuss procedure for inferior vena cava compression syndrome following posterior scoliosis surgery in Marfan syndrome.
Löhnhardt M, Hättich A, Andresen A, Stangenberg M, Mir TS, Reinshagen K, Dreimann M.
Eur Spine J. 2019 Jun;28(Suppl 2):31-36. doi: 10.1007/s00586-018-5772-0. Epub 2018 Oct 5. Review.
PMID: 30291489
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89. Incidence, predictors, and outcomes after severe primary graft dysfunction in pediatric heart transplant recipients.
Profita EL, Gauvreau K, Rycus P, Thiagarajan R, Singh TP.
J Heart Lung Transplant. 2019 Jun;38(6):601-608. doi: 10.1016/j.healun.2019.01.1310. Epub 2019 Jan 24.
PMID: 30733156
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90. Successful Truncal Valve Replacement After Truncal Valve Repairs.
Watanabe T, Nishigaki K, Kawahira Y, Kagisaki K.
Pediatr Cardiol. 2019 Jun 1. doi: 10.1007/s00246-019-02126-y. [Epub ahead of print]
PMID: 31152185
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91. Early Neurodevelopmental Outcomes in Children Supported with ECMO for Cardiac Indications.
Sadhwani A, Cheng H, Stopp C, Rollins CK, Jolley MA, Dunbar-Masterson C, Wypij D, Newburger J, Ware J, Thiagarajan RR.
Pediatr Cardiol. 2019 Jun;40(5):1072-1083. doi: 10.1007/s00246-019-02115-1. Epub 2019 May 11.
PMID: 31079193
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92. Post-Extubation Inhaled Nitric Oxide Therapy via High-Flow Nasal Cannula After Fontan Procedure.
Tominaga Y, Iwai S, Yamauchi S, Kyogoku M, Kugo Y, Hasegawa M, Kayatani F, Takahashi K, Aoki H, Takeuchi M, Tachibana K, Kawata H.
Pediatr Cardiol. 2019 Jun;40(5):1064-1071. doi: 10.1007/s00246-019-02122-2. Epub 2019 May 7.
PMID: 31065760
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93. Preoperative Clinical and Echocardiographic Factors Associated with Surgical Timing and Outcomes in Primary Repair of Common Atrioventricular Canal Defect.
Burstein DS, Gray PE, Griffis HM, Glatz AC, Cohen MS, Gaynor JW, Goldberg DJ.
Pediatr Cardiol. 2019 Jun;40(5):1057-1063. doi: 10.1007/s00246-019-02116-0. Epub 2019 May 7.
PMID: 31065759
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94. Surgical Management of Tetralogy of Fallot with Unilateral Absence of the Pulmonary Artery.
Yang T, Sun J, Xu H, Yan J, Li S, Wang Q, Zhang J.
Pediatr Cardiol. 2019 Jun;40(5):1026-1034. doi: 10.1007/s00246-019-02109-z. Epub 2019 May 2.
PMID: 31049646
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95. Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease.
Foulks MG, Meyer RML, Gold JI, Herrington CS, Kallin K, Menteer J.
Pediatr Cardiol. 2019 Jun;40(5):943-949. doi: 10.1007/s00246-019-02093-4. Epub 2019 Apr 1.
PMID: 30937501
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96. Extracorporeal membrane oxygenation use in the first 24 hours following pediatric heart transplantation: Incidence, risk factors, and outcomes.
Godown J, Bearl DW, Thurm C, Hall M, Feingold B, Soslow JH, Mettler BA, Smith AH, Profita EL, Singh TP, Dodd DA.
Pediatr Transplant. 2019 Jun;23(4):e13414. doi: 10.1111/petr.13414. Epub 2019 Apr 11.
PMID: 30973190
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97. Comparative pharmacokinetics of tacrolimus in stable pediatric allograft recipients converted from immediate-release tacrolimus to prolonged-release tacrolimus formulation.
Rubik J, Debray D, Iserin F, Vondrak K, Sellier-Leclerc AL, Kelly D, Czubkowski P, Webb NJA, Riva S, D’Antiga L, Marks SD, Rivet C, Tönshoff B, Kazeem G, Undre N.
Pediatr Transplant. 2019 Jun;23(4):e13391. doi: 10.1111/petr.13391. Epub 2019 Apr 1.
PMID: 30932313
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Fetal Cardiology Featured Articles
Fetal Cardiology Featured Manuscripts of June 2019
Feasibility of Non-invasive Fetal Electrocardiographic Interval Measurement in the Outpatient Clinical Setting.
Doshi AN, Mass P, Cleary KR, Moak JP, Funamoto K, Kimura Y, Khandoker AH, Krishnan A.
Pediatr Cardiol. 2019 Jun 6. doi: 10.1007/s00246-019-02128-w. [Epub ahead of print]
PMID: 31172229
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Take Home Points:
• Fetal EKG scanning in a routine clinical setting may be feasible.
• Further research, automation, and assessment of cost effectiveness will likely be necessary.
Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: As most fetal cardiologists know, an easy way to obtain a fetal EKG could be a holy grail for diagnosis and management of fetal arrhythmias and possibly prevention of hydrops and fetal demise. While echocardiography can indirectly assess certain rhythm abnormalities, measurement of the QRS complex or QT interval is not possible. There have been past attempts for obtaining a fetal EKG, but the ability to extract the low amplitude fetal signals from the maternal abdominal signals has been poor. Fetal magnetocardiography is currently the gold standard for assessment of fetal arrhythmias, but this is limited to a few large centers that have the equipment necessary for this. This study group used equipment that utilizes blind source separation, a novel signal processing technique that has been shown useful to obtain low amplitude signals and is not dependent on precise electrode positioning. Their objective was to determine the feasibility of measuring standard EKG intervals.
55 fetuses (with a few twin gestations) were studied after 5 were removed from analysis due to a non-functional signal amplifier. Gestational age (GA) ranged from 18-37 weeks (median 23). There were 13 during the vernix period (weeks 25-32), a period known to present difficulty in fetal EP analysis due to the electrical insulating properties of the vernix caseosa, the waxy coating of the fetal skin most prominent at that time. 10 minutes of data were recorded using a prototype fetal EKG monitor using blind source separation as described in a previous paper. Specialized electrodes were placed on the maternal abdomen with one additional electrode on the back as a reference and one on the upper chest to capture the maternal EKG. A magnetic interference shield was draper over the mother and all electrodes were connected to a signal amplifier. Averaged fetal EKG waveforms were processed off-line (see paper for description). Post-processing time was reported as taking between 5-30 minutes, shorter in normal patients, longer in those with rhythm abnormalities (see figure 2).
50 of the 55 fetuses had interpretable results (91%), with 11/13 (85%) in the vernix period. Figure 3 shows the median and interquartile ranges of the EKG intervals at each gestational group. 6 fetuses had abnormal rhythms on fetal echo, and fetal EKG tracings were able to be obtained from 5 of 6, all of which demonstrated the same rhythm abnormality. The authors report that their fetal EKG interval values were similar to those reported when using fetal magnetocardiography.
This was an interesting feasibility study. While the authors report relatively quick and ease of interpretation of the fetal EKG, this would need to be confirmed in larger and more diverse clinical setting before widespread use could be considered. Additionally, how this data will impact clinical management would need to be further elucidated.
Congenital Cardiovascular Anesthesia and Critical Care Featured Articles
CHD Anesthesia June 2019
1. Intraoperative oxygenation and ventilation in children – a U.K. survey of current practice.
Elgie L, Miskovic A, Nandi R.
Paediatr Anaesth. 2019 Jun 24. doi: 10.1111/pan.13688. [Epub ahead of print]
PMID: 31233674
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2. Factors Impacting Physician Recommendation for Tracheostomy Placement in Pediatric Prolonged Mechanical Ventilation: A Cross-Sectional Survey on Stated Practice.
Meyer-Macaulay CB, Dayre McNally J, O’Hearn K, Lynne Katz S, Thébaud B, Vaccani JP, Barrowman N, Harrison MA, Jouvet P.
Pediatr Crit Care Med. 2019 Jun 19. doi: 10.1097/PCC.0000000000002046. [Epub ahead of print]
PMID: 31246744
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3. Continuous spinal anaesthesia for partial gastrectomy in an adult patient with unrepaired tetralogy of Fallot.
Corda Teixeira JS, Correia MJD, Haas A, Tralhão A.
Cardiol Young. 2019 Jun 4:1-2. doi: 10.1017/S1047951119000994. [Epub ahead of print]
PMID: 31159910
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4. Pediatric Ambulatory Anesthesia Challenges.
Butz SF.
Anesthesiol Clin. 2019 Jun;37(2):289-300. doi: 10.1016/j.anclin.2019.01.002. Epub 2019 Mar 15. Review.
PMID: 31047130
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5. The Year in Review: Anesthesia for Congenital Heart Disease 2018.
Ing RJ, Twite M.
Semin Cardiothorac Vasc Anesth. 2019 Jun;23(2):205-211. doi: 10.1177/1089253219845414.
PMID: 31064317
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6. Perioperative and Anesthetic Management of Coarctation of the Aorta.
Fox EB, Latham GJ, Ross FJ, Joffe D.
Semin Cardiothorac Vasc Anesth. 2019 Jun;23(2):212-224. doi: 10.1177/1089253218821953. Epub 2019 Jan 7.
PMID: 30614372
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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
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