Outcomes and complications of combined heart-liver transplantation in patients with failing Fontan physiology: A systematic review

BACKGROUND Single-ventricle congenital heart disease often requires the Fontan procedure, which can lead to Fontan-associated liver disease (FALD) and multi-organ failure. Combined heart-liver transplantation (CHLT) is a potential lifesaving option for these patients. AIM To investigate the outcomes and complications of CHLT in patients with failing Fontan physiology. METHODS Seven retrospective studies of 121 patients undergoing CHLT were systematically reviewed. Quality was assessed with the Newcastle-Ottawa Scale. A meta-analysis using random-effects models to calculate odds ratios (ORs) or mean differences (MDs) with 95% confidence intervals. RESULTS The pooled 30-day, 1-year, 5-year, and 10-year survival rates after CHLT were 92.6%, 86.78%, 81.17%, and 77.8%, respectively. The mean intensive care unit and total hospital lengths of stay were 8.46 and 28.16 days. Mean ischemic time was 267.29 minutes, while cardiopulmonary bypass time was 260.27 minutes. Infections (30%), renal replacement therapy (36.84%), and graft rejection (12.34%) were notable complications. Compared to orthotopic heart transplantation (OHT), CHLT significantly reduced mortality (OR: 0.30, P = 0.009) and ischemic time (MD: –65.93 minutes), with no major differences in perioperative morbidity. CONCLUSION CHLT offers a survival advantage over OHT for patients with FALD and failing Fontan physiology. Future prospective studies are warranted to refine eligibility and improve long-term survival.