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Updated: Jul 15, 2025
Wireless Telemetry Device Implantation in a Fontan Ovine Model for Continuous and Long-Term Hemodynamic Monitoring
Published on: May 2, 2025
The majority of pediatric Fontan patients have excellent post-transplant survival
Kevin Kulshrestha1, Jason W Greenberg1, John T Kennedy1
1Division of Cardiothoracic Surgery, Cincinnati Children's Hospital Medical Center, Heart Institute, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Insights
Pediatric Fontan patients undergoing heart transplant show promising survival rates, especially those not decompensated at transplant. Older age at transplant is linked to better outcomes in this understudied group.
Area of Science:
- Pediatric Cardiology
- Transplant Surgery
- Congenital Heart Disease
Background:
- The Fontan procedure is a palliative surgery for single-ventricle congenital heart defects.
- Many Fontan survivors develop heart failure and require heart transplantation.
- Data on pediatric Fontan patients undergoing heart transplant is limited due to registry identification challenges.
Purpose of the Study:
- To characterize survival outcomes in a large cohort of pediatric patients who underwent heart transplantation after a Fontan procedure.
- To identify factors influencing post-transplant survival in this specific patient population.
Main Methods:
- Utilized the United Network for Organ Sharing (UNOS) and Pediatric Health Information System (PHIS) databases.
- Identified 241 pediatric Fontan patients (age <18) transplanted between 2005-2022.
- Defined decompensation based on criteria including ECMO, ventilation, organ dysfunction, and TPN dependency.
Main Results:
- Median age at transplant was 9 years; median waitlist time was 107 days.
- Overall 1- and 5-year survival rates were 89% and 74%, respectively.
- Non-decompensated patients had significantly better 1- and 5-year survival (93% and 88%) and lower in-hospital mortality (4% vs 11%).
- Decompensation at transplant predicted worse survival (HR 2.47), while older age predicted better survival (HR 0.89/year).
Conclusions:
- Pediatric Fontan patients have promising post-transplant outcomes, but early mortality remains a concern.
- Non-decompensated Fontan patients without end-organ disease (>63% of cohort) experience excellent survival, comparable to the general pediatric transplant population.
- Careful patient selection and management of pre-transplant status are crucial for optimizing outcomes.
Objective:
Many pediatric Fontan patients require heart transplant, but this cohort is understudied given the difficulty in identifying these patients in national registries. We sought to characterize survival post-transplant in a large cohort of pediatric patients undergoing the Fontan.
Methods:
The United Network for Organ Sharing and Pediatric Health Information System were used to identify Fontan heart transplant recipients aged less than 18 years (n = 241) between 2005 and 2022. Decompensation was defined as the presence of extracorporeal membrane oxygenation, ventilation, hepatic/renal dysfunction, paralytics, or total parenteral nutrition at transplant.
Results:
Median age at transplant was 9 (interquartile range, 5-12) years. Median waitlist time was 107 (37-229) days. Median volume across 32 center was 8 (3-11) cases. Approximately half (n = 107, 45%) of recipients had 1A/1 initial listing status. Sixty-four patients (28%) were functionally impaired at transplant, 10 patients (4%) were ventilated, and 18 patients (8%) had ventricular assist device support. Fifty-nine patients (25%) had hepatic dysfunction, and 15 patients (6%) had renal dysfunction. Twenty-one patients (9%) were dependent on total parenteral nutrition. Median postoperative stay was 24 (14-46) days, and in-hospital mortality was 7%. Kaplan-Meier analysis showed 1- and 5-year survivals of 89% (95% CI, 85-94) and 74% (95% CI, 81-86), respectively. Kaplan-Meier of Fontan patients without decompensation (n = 154) at transplant demonstrated 1- and 5-year survivals of 93% (95% CI, 88-97) and 88% (95% CI, 82-94), respectively. In-hospital mortality was higher in decompensated patients (11% vs 4%, P = .023). Multivariable analysis showed that decompensation predicted worse post-transplant survival (hazard ratio, 2.47; 95% CI, 1.16-5.22; P = .018), whereas older age at transplant predicted superior post-transplant survival (hazard ratio, 0.89/year; 95% CI, 0.80-0.98; P = .019).
Conclusions:
Pediatric Fontan post-transplant outcomes are promising, although early mortality remains high. For nondecompensated pediatric patients at transplant without end-organ disease (>63% of cohort), early mortality is circumvented and post-transplant survival is excellent and similar to all pediatric transplantation.
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