Referral for heart transplantation and combined heart-liver transplantation in a contemporary cohort of adults with congenital heart disease

Abstract

Introduction

Adults with congenital heart disease (ACHD) may develop advanced heart failure with associated liver disease from chronic right-sided heart or Fontan failure. Thus, CHD with subsequent irreversible liver dysfunction is an increasing indication for combined heart-liver transplant (CHLT). A systematic liver function assessment is therefore essential prior to listing for heart transplantation (HTx). We aimed to describe clinical characteristics, underlying cardiac defects, surgical history, perioperative issues, and outcomes in a contemporary cohort of ACHD referred for HTx or CHLT.

Method

We prospectively included all ACHD patients referred to our centre for HTx between August 2021 and February 2025. Liver function and disease were evaluated systematically before listing. Inclusions criteria were 1) CHD; 2) HT/CHLT referral; and 3) age > 18 years at the time of referral.

Results

A total of 22 ACHD patients were referred for HT/CHLT to our centre between August 2021 and February 2025. Mean age at referral was 36 ± 24 years. Twelve patients (55%) had univentricular physiology, and all were in New York Heart Association (NYHA) functional class III or IV at the time of referral. Following initial evaluation, 5 patients were listed for CHLT, 7 patients for HTx (including 1 for combined heart-kidney transplantation). The main indications for CHLT were hepatocellular carcinoma and refractory ascites. Three patients died before listing, one of whom received Carmat artificial heart. Prior to transplantation, two patients required inotropic support, two were supported with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) (Figure 1). The median time from evaluation to listing or death prior to listing was 146 days (IQR 28–598 days). Among listed patients, the median time from listing to transplantation or death was 150 days (IQR 55–210 days). Among the 9 transplanted patients, no one died during the study period. No patients required VA-ECMO after transplantation, and no major post-transplant complications were reported.

Conclusion

In this contemporary cohort, patients with HF referred for HT/CHLT are heterogeneous also in terms of severity. Results after CHLT in our Centre are encouraging. Times from evaluation to listing and to transplantation are long. This supports the importance of early referral.