Pulmonary atresia with restrictive ventricular septal defect is a rare congenital cardiac anomaly. A Blalock-Taussig shunt and surgical perforation of the atretic pulmonary valve is often performed as the initial palliation. We present our experience of utilizing both transesophageal and epicardial echocardiography during surgical pulmonary valvuloplasty in a 22-day-old neonate with pulmonary atresia with restrictive ventricular septal defect. The atretic pulmonary valve was perforated using a sheath introduced through the pulmonary artery.
