[III-JCKAP8-1] The Unique Characteristics of Liver Function in Tetralogy of Fallot
Keywords:ACHD, Tetralogy of Fallot, Liver fibrosis
Fontan associated liver disease (FALD) and liver cancer emerge as late complications following the Fontan procedure. Similarly, patients with repaired tetralogy of Fallot (TOF) exhibit hepatic congestion and fibrosis due to right ventricular (RV) failure due to pulmonary valve dysfunction. Liver cancer attributable to hepatic congestion has been documented in TOF patients. Nonetheless, the potential reversibility of liver fibrosis post pulmonary valve replacement (PVR) remains uncertain. Therefore, we elucidated the reversibility of hepatic fibrosis post-PVR and identified associated predictors. Thirty-three TOF patients (median age 24.9 years) undergoing PVR were enrolled. Liver function tests and fibrosis markers (hyaluronic acid, type-IV collagen) were assessed pre- and one year post-PVR. While hyaluronic acid levels remained unchanged, type-IV collagen levels decreased post-PVR. Patients with normalized type-IV collagen levels post-PVR exhibited shorter baseline QRS duration, greater reduction in RV end-diastolic volume, and tended to have better baseline RV ejection fraction compared to those without normalized type-IV collagen levels. Despite hyaluronic acid's persistence, a reduction in RV volume correlated with decreased type-IV collagen, an early fibrosis marker. Baseline RV ejection fraction and QRS duration may serve as predictors for liver fibrosis reversibility. These findings emphasize the importance of adequate timing of PVR in patients with repaired TOF.