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Fontan Patient Survival After Pediatric Heart Transplantation Has Improved in the Current Era
Kathleen E Simpson1, Elizabeth Pruitt2, James K Kirklin2
1Department of Pediatrics, St. Louis Children's Hospital, St. Louis, Missouri.
Insights
Survival rates for heart transplant recipients with a prior Fontan procedure have significantly improved. Fontan patients now have comparable post-transplant survival to other congenital heart disease patients.
Area of Science:
- Cardiology
- Transplantation Medicine
- Pediatric Congenital Heart Disease
Background:
- Historically, Fontan procedure patients faced higher mortality risk after heart transplant (HT).
- Improved pediatric HT survival trends raise questions about Fontan patient outcomes in the current era.
Purpose of the Study:
- To evaluate the change in post-heart transplant survival for Fontan procedure patients.
- To compare outcomes between early and late eras and with non-Fontan congenital heart disease patients.
Main Methods:
- Analysis of Pediatric Heart Transplant Study database data.
- Comparison of Fontan patients from early (1993-2006) and late (2007-2014) eras.
- Comparison of Fontan and non-Fontan congenital heart disease patients.
Main Results:
- Fontan patient survival post-HT significantly improved from the early to late era (p=0.02).
- One-year survival increased from 77% (early era) to 89% (late era).
- Late era Fontan patient survival is now comparable to non-Fontan congenital heart disease patients (92%).
Conclusions:
- Fontan patient survival after heart transplant has markedly improved.
- Fontan patients now demonstrate similar post-HT survival rates to other congenital heart disease patients.
- Excluding Fontan patients from HT consideration based solely on prior procedure is unwarranted.
Background:
Historically, patients with a prior Fontan procedure for complex congenital heart disease (CHD) have been considered at higher risk for death after heart transplant (HT) compared with other HT transplant candidates. With the overall trend of improved survival of pediatric HT recipients, it is unclear of Fontan patient post-HT survival has also improved in the current era.
Methods:
Data from the Pediatric Heart Transplant Study database for Fontan patients who underwent HT was compared between the early era (1993 to 2006, n = 150) and late era (2007 to 2014, n = 252). Post-HT survival and pre-HT characteristics were compared among eras and also with non-Fontan CHD patients.
Results:
At time of HT, Fontan patients in the late era were more likely to require inotropic support, have protein-losing enteropathy, have failure to thrive, and be further from time of Fontan, although less likely to be on ventilator support. Only ventilator support and earlier year of HT were significant risk factors for death in the multivariate analysis. Post-HT Fontan patient survival significantly improved from the early to late era (p = 0.02), particularly in the early phase, with 1-year survival of 77% in the early era and 89% in the late era. Late era non-Fontan CHD patient 1-year post-HT survival was similar to Fontan patients at 92%.
Conclusions:
Survival of Fontan patients after HT has significantly improved in the current era. Currently, expected post-HT survival for Fontan patients is on par with other CHD patients. Fontan patients should not be excluded from consideration for HT solely on a history of Fontan.

