Unilateral pulmonary artery agenesia complicated by massive haemoptysis in an adult female.

Isolated unilateral pulmonary artery agenesis is a rare congenital abnormality. We report a case of right pulmonary artery agenesis in an adult female without other cardiovascular anomalies. The patient presented with massive haemoptysis four years after the original diagnosis. The patient underwent right pneumonectomy with stapled suture of the main bronchus covered by an intercostal muscle flap. The clinical course was complicated by a bronchopleural fistula one month after the first operation. The fistula was successfully treated with a videothoracoscopic omentoplasty and serratus muscle flap. In these patients the surgical approach can be particularly complex because of the high risk of bleeding from the highly vascularised and extensive adhesions between the lung and the chest wall, associated with hyperplasia of the bronchial and intercostal arterial trees. In spite of these difficulties, access to the pulmonary veins and the main bronchus during pneumonectomy is not challenging.