Unroofed Coronary Sinus Syndrome Presenting with Early Pulmonary Hypertension

Isolated partial or complete absence of the coronary sinus roof hemodynamically behaves like an atrial septal defect and rarely causes pulmonary hypertension. We report a case of unroofed coronary sinus (URCS) presenting with post-operative cyanosis due to pulmonary hypertensive crisis following surgical repair of a ventricular septal defect at 6 months of age. He had progressively decreased stamina with gradual increase in pulmonary pressure requiring surgical closure of the URCS by 6 years of age with resolution of symptoms and normalization of the RV pressure.