The absence of one pulmonary artery combined with partial obliteration of one of the pulmonary veins is a rare condition that concerns the developmental failure of the 6th brachial arch during the period of the 6th -16th weeks of gestation and failed connection of the common pulmonary vein to the left atrium.

About 20% of young patients with hemoptysis may present vascular abnormalities; one of the most significant is atresia of the pulmonary artery. There is no general agreement about treatment. The asymptomatic population must be followed up to monitor for pulmonary hypertension.