Contemporary Waitlist and Post-Heart Transplant Outcomes for Fontan patients: An International Multi-Institutional...

Area of Science:

• Pediatric Cardiology
• Transplantation Medicine
• Congenital Heart Disease

Background:

• Fontan circulation patients are a growing, high-risk group for pediatric heart transplantation.
• Limited contemporary data exists on Fontan patients' waitlist and post-transplant outcomes.

Purpose of the Study:

• To analyze waitlist and post-transplant outcomes for pediatric heart transplant candidates with Fontan circulation.
• Compare outcomes between Fontan patients, non-Fontan congenital heart disease (CHD), and cardiomyopathy (CMP) groups.
• Evaluate trends in outcomes across different eras.

Main Methods:

• Retrospective analysis of children (2 to <18 years) listed for heart transplant (1993-2023).
• Categorization into Fontan circulation, non-Fontan CHD, and cardiomyopathy (CMP) groups.
• Analysis of outcomes in early (1993-2014) and current (2015-2023) eras using Cox proportional hazards models.

Main Results:

• Fontan patients comprised 22.3% of listed patients, becoming the majority of CHD listings in the current era.
• Waitlist survival improved and showed no difference among groups in the current era.
• Fontan patients had a 2-fold higher risk of post-transplant graft loss and increased infection/malignancy rates compared to CMP.
• Ventricular assist device (VAD) support was less common in Fontan patients but associated with similar post-transplant survival compared to no support and better survival than extracorporeal membrane oxygenation.

Conclusions:

• Fontan patients are the largest CHD subgroup listed for heart transplantation.
• While waitlist outcomes have improved, post-transplant outcomes for Fontan patients remain inferior.
• Selective and timely VAD support may improve survival for high-risk Fontan patients awaiting heart transplant.