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

Purpose of Review: 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. Recent Findings: 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. Summary: 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. © The Author(s), under exclusive licence to Springer Nature Switzerland AG 2024.