Pulmonary atresia with intact ventricular septum causing severe left ventricular outflow tract obstruction

We describe an infant with pulmonary atresia with intact ventricular septum (PAIVS) and severe left ventricular outflow tract (LVOT) obstruction secondary to a suprasystemic right ventricle causing leftward displacement of the interventricular septum. Imaging demonstrated an aneurysmal dilation at the base of the proximal main pulmonary artery (MPA) with no forward flow from the right ventricle. During transannular patch and central shunt placement, the communication between the pulmonary artery and the right ventricle was enlarged to ensure adequate decompression. We report this successful palliation and resulting complete elimination of the LVOT obstruction in a very unique presentation of PAIVS in a newborn.