Agenesia of right pulmonary artery associated with high output coronary fistula for superior vena cava and intrapulmonary artery branches: Case report

Unilateral Pulmonary Artery Agenesis (UPAA) is a rare congenital anomaly that occurs due to malformation of the sixth aortic arch of the affected side during embryogenesis, and may occur in isolation or in combination with other cardiovascular anomalies.1,2 Diagnosis usually occurs in adolescence, but individuals may be asymptomatic and receive late diagnosis. We report a case of symptomatic patient with right pulmonary artery agenesis accompanied by high-output fistula of the Circumflex Artery (CXA) to the Superior Vena Cava (SVC). (author)