Unilateral absence of pulmonary artery presenting with pulmonary hypertension in two adults

Unilateral absence of pulmonary artery (UAPA) is a rare congenital abnormality that occurs during embryonic development. Isolated UAPA has a prevalence of about 1 in 200 000. The clinical presentation varies based on age, but manifestations include recurrent pneumonia, haemoptysis, pulmonary hypertension or an incidental finding, among others. Here, we present two adult patients with isolated UAPA who were diagnosed with pulmonary hypertension and have been medically managed at our institution.