Heart Transplantation Outcomes for Fontan Patients When Using Contemporary Strategies: Heart-Liver Transplantation and Ventricular Assist Device Therapy

Abstract

Background

With an increasing number of Fontan patients surviving into adulthood, the burden of end-stage heart failure is increasing. Prior studies have reported suboptimal heart transplantation (HTx) outcomes. Therefore, the authors describe their institutional experience of HTx in patients with Fontan circulation failure, including en-bloc heart-liver transplantation (HLTx) and pretransplant systemic ventricular assist device (SVAD) therapies.

Methods

All consecutive Fontan-palliated patients undergoing HTx or HLTx between 2013 and 2022 at the authors’ institution were included. Pretransplant characteristics and posttransplant outcomes were recorded.

Results

Twenty-six transplant recipients, including 5 (19%) HLTx recipients, were identified. The majority (n = 16, 62%) of patients had a cardiac diagnosis of hypoplastic left heart syndrome. Seven (26%) patients were bridged to transplant on an SVAD; the median duration of support was 104 [IQR 39-543] days. Transplantation occurred at a median of 10.6 [IQR 6.8-15.6] years post-Fontan. Fourteen (54%) patients had ≥1 in-hospital complication and 4 (15%) patients required an in-hospital reoperation. Postdischarge reinterventions included tricuspid valve repair (n = 1, 4%) and retransplantation (n = 1, 4%). Two (8%) in-hospital mortalities [22 days (post-HLTx), 2.9 months (post-HTx] and 4 (15%) postdischarge mortalities [5.4 months, 3.1 years, 5.7 years, 7.1 years (all post-HTx)] occurred. Overall survival was excellent, with in-hospital, one-year, and five-year actuarial survival being 92%, 89%, and 80%, respectively.

Conclusions

The current series demonstrates that excellent posttransplant outcomes are achievable for patients with Fontan circulation failure using a comprehensive approach including SVAD and HLTx therapies. Pretransplant optimization, sometimes including SVAD implantation, and meticulous operative planning are imperative strategies for successful patient outcomes.