A Rare Case of a Large Aortopulmonary Window with Anomalous Right Pulmonary Artery From Ascending Aorta in a Young Adult

Abstract

Background: The aortopulmonary window, a rare congenital condition, is an abnormal communication between the ascending aorta and the major pulmonary artery. This anomaly often necessitates early surgical intervention because its prognosis is unfavorable. Cases of adult survival without treatment are exceedingly rare. This condition is typically fatal in childhood if left untreated. In rare instances, it may manifest in adults, presenting symptoms similar to those of pulmonary hypertension. These patients exhibit clinical presentations that are indistinguishable from those of more common conditions characterized by a left-to-right shunt. Through careful transthoracic echocardiography, it is possible to identify the defect in the aortopulmonary septum. Results: We present a unique case of a 23-year-old adult diagnosed with a large, unrepaired aortopulmonary window, illustrating the challenges in diagnosing and managing such rare adult presentations.