Isolated unilateral pulmonary artery atresia in an adult presenting with cor pulmonale

Abstract

Unilateral pulmonary artery atresia (UPAA) is a rare embryonic vascular malformation, leading to general presentations of exertional dyspnoea, pneumonia and haemoptysis. Our patient, a man in his early 30s, presented with a history of progressive breathlessness over a period of 2 years. History showed multiple admissions for pneumonia over his childhood and adolescence. Physical examination revealed a loud P2, an ejection systolic murmur and coarse crepitations in multiple lung fields. Severe pulmonary artery hypertension was noted on two-dimensional echocardiography. CT studies confirmed right pulmonary artery atresia with aspergilloma and bronchiectasis. With this diagnosis of UPAA, this case report intends to raise awareness among clinicians to consider this as a rare cause of cor pulmonale in an adult.