Title : Unilateral pulmonary artery agenesis with singular coronary artery: A case report and literature review
Abstract:
Unilateral Agenesis of the Pulmonary Artery (UAPA) is a rare congenital disorder occurring in 1 in 200,000 individuals.1 the anomaly results from malformation of the sixth aortic arch of the affected side during embryogensis. 2 We are presenting a case of a 44 year old Caucasian male with absence of the left pulmonary artery, left lung congenital hypoplasia, right sided aortic arch, and singular coronary artery with initial presentation of acute hypoxic respiratory failure. Diagnosis of unilateral pulmonary artery agenesis with right sided aortic arch was established with CT angiogram with IV contrast. On subsequent presentation with congestive heart failure and concern for ischemia, heart catheterization reveal singular left coronary artery. The patient was treated with triple Coronary Artery Bypass Graft (CABG) by cardiothoracic surgery however the patient ultimately had complications including prolonged intubation with ventilator associated pneumonia and ischemic stroke. Although unilateral pulmonary artery agenesis can present asymptomatically in adulthood complications, additional anomalies as in this case can lead to adverse outcomes. We present an unusual case of unilateral pulmonary artery agenesis as the patient additionally had anomaly of a single coronary artery requiring CABG.