Hepatic Abnormalities in Patients with Repaired Tetralogy of Fallot: Emerging Evidence and Implications for Transitional Care
Year: 2026
Type: article
Abstract: Repair of tetralogy of Fallot (TOF) preserves a biventricular circulation, which differs fundamentally from Fontan physiology. Clinically important chronic liver disease is not established as a common complication of routine TOF repair, and its prevalence remains inadequately quantified. Hepatic abnormalities are most plausible in patients with sustained residual pulmonary or tricuspid regurgitation, right ventricular dysfunction, elevated right-sided filling pressures, or impaired forward flow. This narrative review examines TOF-specific evidence, distinguishing it from Fontan and general heart-failure observations. Published TOF studies, predominantly in adolescents and adults, report heterogeneous biochemical and imaging findings and occasional histologically documented fibrosis in selected patients. The aspartate aminotransferase-to-platelet ratio index (APRI), matrix markers, liver stiffness and T1 mapping may reflect congestion and cannot independently establish irreversible fibrosis. Pediatric evidence and the absolute risk of advanced liver disease remain uncertain. A risk-based approach to liver assessment is proposed for patients with significant residual hemodynamic abnormalities, heart failure, abnormal liver tests, hepatomegaly, or concerning imaging findings. Cardiac assessment should accompany hepatic evaluation, with consideration of metabolic, viral, autoimmune and drug-related causes. This framework is not a validated screening protocol, and no universal starting age or testing interval is established. Isolated reports of hepatocellular carcinoma (HCC) do not justify TOF-specific HCC surveillance without established cirrhosis or another recognized indication.
Source: Children
Institution: Niigata University
Language: English
Subfield: Cardiology and Cardiovascular Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,896
OpenAlex ID: W7225390594