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.
