Unilateral agenesis of pulmonary artery (UAPA) is a rare congenital anomaly that occurs due to malformation of the sixth aortic arch and about 70% of the patients have associated cardiovascular anomalies. However, UAPA without any associated cardiovascular anomalies is termed isolated UAPA. Cases of isolated UAPA may remain asymptomatic and survive into adulthood. Majority of the adults are asymptomatic and can present with recurrent respiratory tract infections. Presence of pulmonary hypertension carries a poor prognosis. There is no specific treatment for UAPA. We report two patients of UAPA with adult presentation.
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