Innovation in Pulmonary Atresia Repair Using Right Atrial Appendage as Pulmonary Valve: A Case Report

A four-year girl with a ventricular septal defect (VSD) and pulmonary valve atresia (PVA) underwent a total correction surgery. Preoperative findings revealed a severe right-sided dilation, a right ventricular hypertrophy (RVH), a large conoventricular VSD, a slightly hypoplastic main pulmonary artery (MPA), and appropriately-sized pulmonary arteries. The surgery began with a midline sternotomy, patent ductus arteriosus (PDA) ligation, and initiation of cardiopulmonary bypass (CPB). The pericardium was harvested, and the right atrial appendage (RAA) was shaped into a bi-leaflet valve. A myomectomy was performed, and the VSD was closed with a continuous Dacron patch. Continuity between the right ventricular outflow tract (RVOT) and pulmonary artery (PA) was achieved using a transannular patch from autologous pericardium, and the RAA valve was implanted. A 15 mm Hegar dilator confirmed smooth blood flow. The patient showed satisfactory intraoperative transesophageal echocardiography (TEE) findings, including good ventricular function, an RVOT gradient of 20-25 mmHg, and no residual defects. The procedure achieved successful outcomes.