Bulhões, Elísio

Background. The Fontan procedure has transformed the management of congenital heart defects characterized by single ventricle physiology, yet it predisposes individuals to Fontan-associated liver disease. Combined heart and liver transplantation (CHLT) emerges as a therapeutic option, but evidence of its efficacy and safety remains limited. This study aimed to comprehensively evaluate CHLT in Fontan patients, focusing on patient characteristics, perioperative outcomes, and posttransplant morbidity and mortality. Methods. Following Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines, a systematic search of PubMed, Embase, and the Cochrane Central Register of Controlled Trials was conducted. Studies meeting the intervention of CHLT in Fontan patients were included, and data were collected and synthesized using proportion meta-analysis techniques. Statistical analysis was carried out using R software. Results. Four studies met inclusion criteria, comprising 67 Fontan patients undergoing CHLT. All included studies were observational retrospective cohorts performed in the United States. The 1-y survival rate post-CHLT was 88% (95% confidence interval [CI], 70%-98%). Liver graft rejection rates were low, 4% (95% CI, 0%-22%), and no heart graft rejection greater than mild was reported. Postoperative complications included acute kidney injury 75% (95% CI, 50%-93%), temporary dialysis 27% (95% CI, 9%-51%), neurologic events 7% (95% CI, 0%-26%), infection 23% (95% CI, 3%-55%), and unplanned medical procedures 40% (95% CI, 23%-59%). Conclusions. CHLT in Fontan patients demonstrates promising survival rates, but graft rejection and postoperative complications pose challenges. The rate of renal complications is particularly notable and requires further evaluation. Future research should prioritize comparative different management strategies and long-term follow-up to refine protocols and optimize outcomes.

Background: The Fontan procedure has transformed the management of congenital heart defects (CHD) characterized by single ventricle physiology, yet it predisposes individuals to Fontan-associated liver disease (FALD), potentially leading to end-stage liver disease (ESLD). Combined heart and liver transplantation (CHLT) emerges as a therapeutic option, but evidence on its efficacy, safety, and outcomes remains limited. Objectives: This systematic review and single-arm meta-analysis aims to comprehensively evaluate the literature on CHLT in Fontan patients, focusing on patient characteristics, perioperative outcomes, and post-transplant morbidity and mortality. Methods: A systematic search of PubMed, Embase, and Cochrane Central Register of Controlled Trials was conducted following PRISMA guidelines. Studies meeting the intervention of CHLT in Fontan patients were included and data were collected and synthesized using proportion meta-analysis techniques. Statistical analysis was carried out using R software. Results: Four studies met inclusion criteria, comprising 67 Fontan patients undergoing CHLT. All included studies were observational retrospective cohorts performed in the United States. One-year survival rate post-CHLT was 84% (95% CI 66%-94%), Figure 1A. Rates of liver and heart graft rejection were low 10% (95% CI 3%-33%) and 8% (95% CI 2%-31%), respectively, Figure 1B. Postoperative complications included acute kidney injury 72% (95% CI 46%-88%), temporary dialysis 31% (95% CI 14%-55%), neurologic events 14% (95% CI 5%-34%), infection 24% (95% CI 6%-63%), and unplanned medical procedures 41% (95% CI 24%-60%), Figure 1C. Conclusion: CHLT in Fontan patients demonstrates promising survival rates, but graft rejection and postoperative complications pose challenges. The rate of renal complications is particularly notable and requires further evaluation. Future research should prioritize comparative different management strategies and long-term follow-up to refine protocols and optimize outcomes.