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Comparative Study
. 2024 Summer;36(2):230-241.
doi: 10.1053/j.semtcvs.2022.08.019. Epub 2022 Nov 29.

Palliated Hypoplastic Left Heart Syndrome Patients Experience Superior Waitlist and Comparable Post-Heart Transplant Survival to Non-Single Ventricle Congenital Heart Disease Patients

Jason W Greenberg  1 , Muhammad Aanish Raees  2 , Alia Dani  2 , Haleh C Heydarian  2 , Clifford Chin  2 , Farhan Zafar  2 , David G Lehenbauer  2 , David L S Morales  2
Affiliations
Comparative Study

Palliated Hypoplastic Left Heart Syndrome Patients Experience Superior Waitlist and Comparable Post-Heart Transplant Survival to Non-Single Ventricle Congenital Heart Disease Patients

Jason W Greenberg et al. Semin Thorac Cardiovasc Surg. 2024 Summer.

Abstract

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.

Keywords: Clinical outcomes; Congenital cardiac surgery; Congenital heart disease; Heart transplantation; Hypoplastic left heart syndrome; Norwood procedure; Quality improvement.

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Conflict of interest statement

Disclosures: The authors declare no conflicts of interest.

Figures

Figure 1.
Figure 1.
Time-to-event, competing-risk analysis of waitlist outcomes for surgically palliated hypoplastic left heart syndrome (HLHS) patients.
Figure 2.
Figure 2.
Time-to-event, competing-risk analysis of waitlist outcomes for non-single ventricle congenital heart disease (non-SV CHD) patients.
Figure 3.
Figure 3.
Time-to-event, competing-risk analysis of waitlist outcomes for non-congenital heart disease (non-CHD) patients.
Figure 4.
Figure 4.
Kaplan-Meier survival curves (with 95% confidence intervals shown) of post-transplant survival by diagnosis group. *HLHS, previously palliated hypoplastic left heart syndrome patients; non-SV CHD, non-single-ventricle congenital heart disease; non-CHD, non-congenital heart disease.
Figure 5.
Figure 5.
Graphical abstract, pictorially illustrating the methods, results, and implications of the study.
See this image and copyright information in PMC

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