Unusual case of pulmonary valve atresia

Abstract

We report on a 12-year-old boy with a rare form of pulmonary valve atresia with a ventricular septal defect (VSD) and anomalous origin of the left pulmonary artery arising from the aortic arch. He also has an absent right pulmonary artery, the right lung being supplied by small major aorto-pulmonary collaterals (MAPCAs). He has decreased pulmonary blood flow to his right lung and increased pulmonary blood flow with irreversible pulmonary hypertension (PHT) on the left. Due to the late presentation, he was deemed inoperable due to the severe PHT of the left lung.