Neonatal Cone Valvuloplasty for Pulmonary Atresia/Intact Ventricular Septum and Ebsteinoid Valve

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare lesion with wide anatomic variability. A rare variant exists, marked by right-sided dilatation, an Ebsteinoid tricuspid valve, and severe tricuspid regurgitation. Neonatal cone valvuloplasty allows for a biventricular circulation and avoids complications of prosthetic valves; however, this technique inevitably requires reintervention to exchange the right ventricle-to-pulmonary artery conduit as the patient grows. We present a successful complete repair cone tricuspid valvuloplasty in a 9-day-old (2.8 kg) neonate with trisomy 21, pulmonary atresia with intact ventricular septum, and an Ebsteinoid tricuspid valve.