Skeletonized Handmade Valved Conduit: A Novel Approach for Transannular Repair in Absent Pulmonary Valve Syndrome

Abstract

Absent pulmonary valve syndrome in neonates and infants presents challenges due to airway compression from dilated pulmonary arteries and limited anatomical space for right ventricular outflow tract (RVOT) reconstruction. This study introduces a half-skeletonized handmade valved conduit for transannular repair in a 6-month-old boy with absent pulmonary valve syndrome, trisomy 21, and laryngomalacia. The conduit, made from expanded polytetrafluoroethylene, provided effective RVOT reconstruction while minimizing airway obstruction. Postoperative imaging confirmed bronchial decompression, and echocardiography showed sustained valve function at 1 year. This technique addresses homograft limitations and may have broader applicability in congenital heart defects requiring RVOT reconstruction.