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Published on: June 23, 2014
Heart Transplantation Following Fontan Failure: Long-Term Survival Analysis
Michele D'Alonzo1, Federico Brunelli2, Francesco Seddio2
1Cardiac Surgery Unit, Cardio-Thoracic Department, University of Brescia, 25123 Brescia, Italy.
Insights
Heart transplantation in patients with Fontan circulation shows comparable long-term survival rates to those with biventricular congenital heart disease. This study demonstrates that heart transplantation is a viable treatment option for failing Fontan patients.
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.
Abstract:
Objectives: Fontan circulation presents significant challenges for patients with congenital heart disease, often necessitating heart transplantation (HTX) due to deteriorating functionality across multiple organ systems. However, the impact of prior Fontan palliation on HTX outcomes remains poorly understood, with early mortality rates suggesting a heightened risk. The aim of our study is to evaluate the long-term results after heart transplantation in patients with univentricular congenital heart disease previously palliated with Fontan circulation. Methods: A retrospective analysis was conducted on patients who underwent HTX for congenital heart disease. Patients were categorized into two groups based on the pre-HTX circulation pathway: the Failing Fontan Group (FFG) and the Biventricular Congenital Group (BCG). Data were collected from patients between 1987 and 2018. Early and late outcomes, including survival rates, were assessed and critically analyzed. Results: Of the 66 patients, 29 (43%) had a failing Fontan palliation (FFG), and 37 had biventricular congenital diseases (BCG) before heart transplantation. Early mortality (30-day) was not statistically different between the two group. The overall survival rate was 82.6 ± 13.9% at 1 year, 79.0 ± 14.9% at 5 years, 67.2 ± 17.6% at 10 years and 63.2 ± 18.2 ± at 15 years for the FFG, and 86.1 ±11.4% at 1 year, 79.5 ± 13.7% at 5 years, 75.7 ± 14.9% at 10 years, 75.7 ± 14.9% at 15 years for the BCG, with no statistically significant difference (Mantel Cox p value: 0.69, 0.89, 0.52 and 0.39, respectively). Regarding Cox-regression analysis, the long-term survival rate was not affected either by previous Fontan surgery or by the era of heart transplantation (before vs. after the year 2000). Conclusions: Although heart transplantation after Fontan palliation showed a higher risk in the early post-operative period, the medium- and long-term survival rates are comparable with biventricular circulation patients. Despite the failing Fontan patients being a challenging set of candidates for transplantation, it is a reasonable option in their treatment.
