Surgical repair of an aortopulmonary window and subaortic stenosis in an adolescent: case report

Background: Aortopulmonary windows (APWs) are rare congenital heart defects that can rapidly cause pulmonary arterial disease. Most patients with APWs present in the neonatal or infant period. We describe the successful surgical management of an adolescent female who presented with an APW and severe subaortic stenosis (SAS). Case Description: A 16-year-old asymptomatic female with known SAS presented to establish care. Transthoracic echocardiogram (TTE) revealed a subaortic membrane extending into the left-ventricular-outflow-tract with a peak gradient of 114 mmHg. TTE also revealed a dilated aorta. Cardiac catheterization and computerized tomography was significant for a large APW (3.4-by-3.6 cm), elevated pulmonary-vascular-resistance (PVR) of 15 wood units, and a pulmonary to systemic blood flow of 1:1. Pulmonary to systemic blood flow increased to 3.2:1 and the PVR dropped to 3.2 wood units with 100% FiO2 and 80 parts per million of nitric oxide. As acute vasodilative testing was positive, the patient underwent surgical APW repair, subaortic membrane resection, and atrial septal defect (ASD) creation. At 8-month follow-up, the patient was doing well and able to exercise. Conclusions: We describe a delayed presentation of a large left-to-right shunt APW with SAS. As pulmonary reactivity was preserved, despite the delayed presentation, the patient underwent successful surgical repair.