Right atrial appendage for neopulmonary valve reconstruction in a child with tetralogy of Fallot with absent pulmonary valve.

Abstract

Absent pulmonary valve is a rare congenital heart anomaly frequently associated with tetralogy of Fallot. We present the case of a 12-year-old child who came with symptoms of cyanosis and a heart murmur. Echocardiographic evaluation revealed severe infundibular pulmonary stenosis and a large subaortic ventricular septal defect. Intraoperative findings confirmed the presence of a rudimentary pulmonary valve, with thin, chordae-like structures connected to the pulmonary leaflet remnants. The child underwent successful intracardiac repair, which involved the reconstruction of a neopulmonary valve utilizing the right atrial appendage tissue.