Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5th aortic arch with a systemic-to-pulmonary arterial connection

• Published in: Cardiology in the young 2000-10, Vol.10 (4), p.419-422
• Main Authors: Serra, Astolfo, Chamie, Francisco, Freedom, R.M.
• Format: Article
• Language: English
• Subjects: Aorta, Thoracic - abnormalities; Aorta, Thoracic - diagnostic imaging; Aorta, Thoracic - physiopathology; Aortography; artery; atresia; Brief Reports; Cardiac Catheterization; Coronary Angiography; Cyanosis - etiology; Echocardiography; Failure to Thrive - etiology; Fatal Outcome; Hemodynamics; Humans; Infant; Pulmonary; Pulmonary Artery - abnormalities; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - physiopathology; Pulmonary Atresia - complications; Pulmonary Atresia - diagnostic imaging; Pulmonary Atresia - physiopathology; Pulmonary Circulation; ventricular septum
• ISSN: 1047-9511; 1467-1107
• DOI: 10.1017/S1047951100009768

Major abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.