Determinants of survival following heart transplantation in adults with congenital heart disease

• Published in: Journal of cardiothoracic surgery Vol. 19; no. 1; p. 83
• Main Authors: Sicim, Hüseyin, Noly, Pierre Emmanuel, Naik, Suyash, Sood, Vikram, Ohye, Richard G, Haft, Jonathan W, Aaronson, Keith D, Pagani, Francis D, Si, Ming-Sing, Tang, Paul C
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 10.02.2024; BioMed Central; BMC
• Subjects: Adults; Analysis; Blood groups; Cardiomyopathy; Cardiovascular disease; Cardiovascular diseases; Cerebrovascular diseases; Complexity; Congenital diseases; Congenital heart disease; Dialysis; Extracorporeal membrane oxygenation; Fatalities; Genetic disorders; Heart; Heart diseases; Heart failure; Heart transplant; Heart transplantation; Heart transplants; Hemodialysis; Hospitals; Ischemia; Liver; Males; Malignancy; Medical research; Medicine, Experimental; Mortality; Online databases; Outcomes; Oxygenation; Patients; Physiology; Pulmonary arteries; Stroke; Surgery; Survival; Survival analysis; Transplantation; Transplants; Variance analysis; Veins & arteries; Ventricle; Ventricular assist devices; Heart failure; Congenital heart disease; Heart transplant; Outcomes
• ISSN: 1749-8090; 1749-8090
• DOI: 10.1186/s13019-024-02509-0

Adult patients surviving with congenital heart disease (ACHD) is growing. We examine the factors associated with heart transplant outcomes in this challenging population with complex anatomy requiring redo-surgeries. We reviewed the United Network for Organ Sharing-Standard Transplant Analysis and Research database and analyzed 35,952 heart transplants from January 1st, 2000, to September 30th, 2018. We compared transplant characteristics for ischemic cardiomyopathy (ICM) (n = 14,236), nonischemic cardiomyopathy (NICM) (n = 20,676), and ACHD (n = 1040). Mean follow-up was 6.20 ± 4.84 years. Kaplan-Meier survival curves and Cox-proportional hazards analysis were used to analyze survival data. Multivariable analysis confirmed that ACHD was associated greater in-hospital death compared to ICM (HR = 0.54, P < 0.001) and NICM (HR = 0.46, P < 0.001). Notable factors associated with increased mortality were history of cerebrovascular disease (HR = 1.11, P = 0.026), prior history of malignancy (HR = 1.12, P = 0.006), pre-transplant biventricular support (HR = 1.12, P = 0.069), postoperative stroke (HR = 1.47, P < 0.001) and postoperative dialysis (HR = 1.71, P < 0.001). ACHD transplants had a longer donor heart ischemic time (P < 0.001) and trend towards more deaths from primary graft dysfunction (P = 0.07). In-hospital deaths were more likely with ACHD and use of mechanical support such as use of right ventricular assist device (HR = 2.20, P = 0.049), biventricular support (HR = 1.62, P < 0.001) and extracorporeal membrane oxygenation (HR = 2.36, P < 0.001). Conditional survival after censoring hospital deaths was significantly higher in ACHD (P < 0.001). Heart transplant in ACHD is associated with a higher post-operative mortality given anatomical complexity but a better long-term conditional survival. Normothermic donor heart perfusion may improve outcomes in the ACHD population by reducing the impact of longer ischemic times.