Heart Transplantation Following Fontan Failure: Long-Term Survival Analysis

Area of Science:

• Cardiology
• Pediatric Cardiology
• Cardiac Surgery

Background:

• Fontan circulation is a palliative procedure for complex congenital heart disease.
• Patients with Fontan circulation often experience multi-organ system deterioration, leading to heart transplantation (HTX).
• Outcomes of HTX in Fontan patients are not well-established, with concerns about increased early mortality.

Purpose of the Study:

• To evaluate the long-term results of heart transplantation in patients with univentricular congenital heart disease previously palliated with Fontan circulation.
• To compare HTX outcomes between patients with failing Fontan circulation and those with biventricular congenital heart disease.

Main Methods:

• Retrospective analysis of 66 patients who underwent HTX for congenital heart disease between 1987 and 2018.
• Patients were divided into two groups: Failing Fontan Group (FFG) and Biventricular Congenital Group (BCG).
• Comparison of early and late outcomes, including survival rates, between the two groups.

Main Results:

• No statistically significant difference in 30-day mortality between FFG (29 patients) and BCG (37 patients).
• Comparable long-term survival rates between FFG and BCG at 1, 5, 10, and 15 years post-HTX.
• Cox-regression analysis indicated no impact of prior Fontan surgery or transplantation era on long-term survival.

Conclusions:

• Heart transplantation following Fontan palliation presents higher early post-operative risks but comparable medium- and long-term survival rates to biventricular patients.
• Despite challenges, heart transplantation is a reasonable treatment option for patients with failing Fontan circulation.
• Fontan patients represent a challenging but manageable cohort for heart transplantation.