Unilateral hypoplasia or absence of a branch of the pulmonary artery is a rare anomaly usually associated with other congenital cardiac disorders. Isolated hypoplasia of unilateral pulmonary artery is still rare. Patients present with varied clinical manifestations with varied age at presentation, younger age or in adulthood with pulmonary hypertension (PAHT). Early diagnosis with definitive treatment prevents development of PAHT in adulthood. Here we present an elderly female who presented with PAHT with right heart failure secondary to chronic pulmonary thromboembolism was diagnosed to have hypoplastic left pulmonary artery.

Keywords: Pulmonary Artery; Pulmonary Hypertension; Pulmonary Angiography.