Pulmonary artery agenesis is an infrequent pulmonary arteriovenous malformation, an abnormality of the lung caused by abnormal communications between arteries and veins in the lungs. The disease is present at birth due to a malformation in the development of the heart. The condition may manifest with total or partial absence of the lung on the same side with the presence of an unusual contracting lung. In children, the affected side of the chest is usually decreased in size, with a reduction in inspiratory amplitude and diminished breath sounds.
Pulmonary artery agenesis is usually well tolerated and manifests with repeated infections and chest pain, among other symptoms. Tolerance becomes complicated if the condition is moreover associated to compression of vessels carrying blood or other malformations.