Fontan-Associated Liver Disease and the Double Organ Transplant: Addressing the Heart of the Argument

Describe the history, background, histopathology, and current practices of Fontan-associated liver disease (FALD), a universal sequelae of Fontan physiology. Build awareness and insight into the risk factors for disease progression, its impact on long-term mortality, and implications for heart transplant (HT) alone versus combined heart liver transplant (CHLT). Highlight an era in which > 90% of patients with single ventricle physiology are reaching adulthood, manifesting the sequelae of multi-organ dysfunction including FALD and highlighting the urgent need to create evidence-based practices to help support this patient population.

Patients with advanced FALD are at higher risk of adverse events and even suboptimal outcomes. Multiple centers are now reporting comparable, if not superior, outcomes for patients post-Fontan with compensated FALD who undergo CHLT compared to HT.

In the setting of organ scarcity, it is especially important to identify high risk patients with FALD who may benefit from CHLT, as the dual organ transplant may optimize long-term survival, quality of life, and reduce sequelae of allograft rejection for patients with single ventricle physiology CHD.