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.