Congenital heart disease (CHD) is a well-established risk factor for inferior waitlist
and post-heart transplant survival in children. Differences in outcomes between CHD
subgroups are understudied. The present study compared outcomes for palliated hypoplastic
left heart syndrome (HLHS) patients to other non-single ventricle CHD (non-SVCHD)
and non-CHD patients. United Network for Organ Sharing was used to identify children
(age < 18) listed for heart transplant in the United States between 2016 and 2021.
CHD sub-diagnoses were only available for United Network for Organ Sharing status
1a after 2015, thereby defining the cohort. Waitlist outcomes were studied using competing-risk
time-to-event analysis for transplantation, mortality/decompensation, and alive-on-waitlist.
Multivariable Cox proportional hazards regression analyses were used to identify factors
associated with inferior post-transplant survival. Patients included: palliated-HLHS
(n = 477), non-SVCHD (n = 686), and non-CHD (n = 1261). At listing, Palliated-HLHS patients were older than non-SVCHD (median 2-year
[IQR 0-8] vs median 0-year [0-3], respectively) and younger than non-CHD (median 7-year
[0-14]) (P < 0.001 vs both), and were more likely to be white (P < 0.01 vs both). Upon time-to-event analysis, rates of waitlist mortality/decompensation
rates were greater among non-SVCHD than palliated-HLHS. Post-transplant survival was
comparable between palliated-HLHS and non-SVCHD (P = 0.920) but worse compared to non-CHD (P < 0.001). Both palliated-HLHS (HR 2.40 [95% CI 1.68-3.42]) and non-SVSCHD (2.04 [1.39-2.99])
were independently associated with post-transplant mortality. Palliated-HLHS patients
with heart failure experience significantly worse post-transplant outcomes than non-CHD
but, compared to other CHD patients, experience superior waitlist and comparable post-transplant
survival. While a high-risk cohort, HLHS patients can achieve gratifying waitlist
and post-transplant survival.
Graphical Abstract
Graphical Abstract
Keywords
Abbreviations:
CHD (congenital heart disease), CI (confidence interval), ECMO (extracorporeal membrane oxygenation), HLHS (hypoplastic left heart syndrome), HR (hazards ratio), IQR (interquartile range), palliated HLHS (operated (palliated) hypoplastic left heart syndrome patients), other-CHD (patients with other congenital heart disease lesions), non-SV CHD (patients with non-single-ventricle congenital heart disease lesions), UNOS (United Network for Organ Sharing), VAD (ventricular assist device)To read this article in full you will need to make a payment
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References
- Congenital heart disease epidemiology in the United States: Blindly feeling for the charging elephant.Circulation. 2016; 134: 110-113https://doi.org/10.1161/circulationaha.116.023370
- Congenital Heart defects in the United States: Estimating the magnitude of the affected population in 2010.Circulation. 2016; 134: 101-109https://doi.org/10.1161/circulationaha.115.019307
- Heart failure in pediatric patients with congenital heart disease.Circ Res. 2017; 120: 978-994https://doi.org/10.1161/circresaha.116.308996
- Indications for heart transplantation in pediatric heart disease.Circulation. 2007; 115: 658-676https://doi.org/10.1161/circulationaha.106.180449
- Outcomes from three decades of infant and pediatric heart transplantation.Asaio J. 2020; (Publish Ahead of Print): 1051-1059https://doi.org/10.1097/mat.0000000000001312
- A risk-prediction model for in-hospital mortality after heart transplantation in US children.Am J Transplant. 2012; 12: 1240-1248https://doi.org/10.1111/j.1600-6143.2011.03932.x
- The reality of limping to pediatric heart transplantation.J Thorac Cardiovasc Surg. 2020; 159 (e1): 2418-2425https://doi.org/10.1016/j.jtcvs.2019.10.077
- Transplantation for congenital heart disease: Focus on the impact of functionally univentricular versus biventricular circulation.World J Pediatric Congenit Hear Surg. 2021; 12: 352-359https://doi.org/10.1177/2150135121990650
- Pediatric cardiac waitlist mortality—Still too high.Pediatr Transplant. 2020; 24: e13671https://doi.org/10.1111/petr.13671
- The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease.J Am Coll Cardiol. 2009; 54: 160-165https://doi.org/10.1016/j.jacc.2009.04.020
- Outcomes and risk factors for heart transplantation in children with congenital heart disease.J Thorac Cardiovasc Surg. 2015; 150 (e3): 1455-1462https://doi.org/10.1016/j.jtcvs.2015.06.029
- Trends in the indications and survival in pediatric heart transplants: A 24-year single-center experience in 307 patients.Ann Thorac Surg. 2012; 94: 807-816https://doi.org/10.1016/j.athoracsur.2012.02.052
- Lessons learned from 119 consecutive cardiac transplants for pediatric and congenital heart disease.Ann Thorac Surg. 2011; 91: 1248-1255https://doi.org/10.1016/j.athoracsur.2010.10.083
- Prevalence of congenital heart defects in Metropolitan Atlanta, 1998-2005.J Pediatrics. 2008; 153: 807-813https://doi.org/10.1016/j.jpeds.2008.05.059
- Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period.J Thorac Cardiovasc Surg. 2010; 139: 119-127https://doi.org/10.1016/j.jtcvs.2009.04.061
- Mortality in first 5 years in infants with functional single ventricle born in Texas, 1996 to 2003.Circulation. 2010; 121: 644-650https://doi.org/10.1161/circulationaha.109.881904
- Results of orthotopic heart transplantation for failed palliation of hypoplastic left heart.Eur J Cardio-thorac. 2012; 43: 597-603https://doi.org/10.1093/ejcts/ezs326
- Results of heart transplantation following failed staged palliation of hypoplastic left heart syndrome and related single ventricle anomalies.Eur J Cardio-thorac. 2015; 48: 792-799https://doi.org/10.1093/ejcts/ezu547
- Validation of the revised Schwartz estimating equation in a predominantly non-CKD population.Pediatr Nephrol. 2010; 25: 2321-2326https://doi.org/10.1007/s00467-010-1598-7
- Hepatic dysfunction and survival after orthotopic heart transplantation: Application of the MELD scoring system for outcome prediction.J Hear Lung Transplant. 2012; 31: 591-600https://doi.org/10.1016/j.healun.2012.02.008
- Transplant-Free survival and interventions at 6 years in the SVR trial.Circulation. 2018; 137: 2246-2253https://doi.org/10.1161/circulationaha.117.029375
- The improved prognosis of hypoplastic left heart: A population-based register study of 343 cases in England and Wales.Front Pediatr. 2021; 9635776https://doi.org/10.3389/fped.2021.635776
- Hypoplastic left heart syndrome: from fetus to Fontan.Cardiol Young. 2018; 28: 1275-1288https://doi.org/10.1017/s104795111800135x
- Current therapy for hypoplastic left heart syndrome and related single ventricle lesions.Circulation. 2016; 134: 1265-1279https://doi.org/10.1161/circulationaha.116.022816
- Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: Lessons learned from 115 consecutive patients.Circulation. 2002; 106: I82-I89
- Hypoplastic left heart syndrome current considerations and expectations.J Am Coll Cardiol. 2012; 59: S1-S42https://doi.org/10.1016/j.jacc.2011.09.022
- Fifth annual pediatric interagency registry for mechanical circulatory support (pedimacs) report.Ann Thorac Surg. 2021; 112: 1763-1774https://doi.org/10.1016/j.athoracsur.2021.10.001
- An overview of mechanical circulatory support in single-ventricle patients.Transl Pediatrics. 2018; 7: 151-161https://doi.org/10.21037/tp.2018.03.03
- Selection and management of pediatric and congenital heart disease patients on ventricular assist devices.J Heart Lung Transplant. 2021; 40: 709-732https://doi.org/10.1016/j.healun.2021.04.015
- Incidence and risk factors for mortality in infants awaiting heart transplantation in the USA.J Heart Lung Transplant. 2009; 28: 1292-1298https://doi.org/10.1016/j.healun.2009.06.013
Article info
Publication history
Published online: November 28, 2022
Publication stage
In Press Journal Pre-ProofFootnotes
Read at the AATS 102nd Annual Meeting, May 14-17, 2022, Boston, MA.
Meeting Presentation: Presented at the American Association for Thoracic Surgery 102nd Annual Meeting, May 16th, 2022, Boston, MA, USA.
IRB: #2018-6837; date of approval: 10/29/2018.
The authors declare no conflicts of interest.
National Institutes of Health, R01HL147957: “Novel Methods to Grow the Impact of Pediatric Thoracic Transplantation;” Principal investigators: David L. S. Morales, MD, Farhan Zafar, MD, MS.
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