Background <p>The Fontan procedure improves survival in single-ventricle patients, but some progress to Fontan circulatory failure (FCF) with limited treatment options. Fontan-associated liver disease (FALD) further complicates management and raises uncertainty regarding the choice between isolated heart transplantation (HT) and combined heart-liver transplantation (CHLT). Data from Asian populations are limited, and this study aimed to provide additional evidence on transplantation outcomes in this population.</p> Methods <p>A single-center retrospective study in China, describing the clinical characteristics, surgical procedures, and outcomes of six consecutive HT patients and one CHLT patient with FCF. The study was conducted between August 2017 and October 2025.</p> Results <p>There were no perioperative deaths and 30‑day survival was 100%. One patient died on postoperative day 60 due to severe pulmonary infection with multiorgan failure, resulting in overall survival of 85.7%, with a median follow-up duration of 71 months (IQR, 6.5–77.0). Cardiac graft function was preserved in all survivors, with improvement in New York Heart Association (NYHA) class from IV preoperatively to I–II. The CHLT recipient experienced initial cardiac graft dysfunction that required intra-aortic balloon pump (IABP) support and renal dialysis. The patient subsequently recovered, with stable function of the cardiac and hepatic allografts, and preserved native renal function. Liver function tests normalized, and protein-losing enteropathy resolved.</p> Conclusions <p>This study demonstrates that both HT and CHLT are feasible for carefully selected patients with FCF. Favorable outcomes likely reflect timely referral, rigorous candidate selection with systematic evaluation of FALD and end-organ function, and proactive management of comorbidities.</p> Clinical trial number <p>Not applicable.</p>