[Unilateral agenesis of pulmonal artery in adults: a rare disease frequently undiagnosed].

Introduction: Unilateral absence of a pulmonary artery (UAPA) in adults without any other cardiovascular anomalies is a very rare clinical entity. Usually UAPA in adults remains undetected because of the symptom-free clinical course. The most common symptoms are hemoptysis and recurrent pulmonary infections.

Patients And Therapy: During 2006 - 2014 four adult patients with UAPA were diagnosed and treated in our institution. Recurrent pulmonary infections in combination with existing bronchiectasis and hemoptysis led to hospital treatment for three of the patients. In two cases, because of persevering hemoptysis and pathologically enlarged systemic arteries (intercostal, bronchial, diaphragm), pneumonectomy was indicated. Preoperative embolization of the enlarged arteries reduced the systemic arterial perfusion of the lung and led to minimal intraoperative blood loss.

Discussion: UAPA in the adulthood can frequently lead to hypertrophic systemic arterial perfusion of the lung. This abnormal systemic perfusion in combination with the co-existing bronchiectasis and persevering hemoptysis can cause a life-threatening clinical scenario. A combined interdisciplinary treatment through pneumology, thoracic surgery and radiology is therefore indicated.