Anomalous Origin of Left Pulmonary Artery From the Aorta: A Rare Entity in Congenital Heart Disease.


Division of Pediatric Cardiology, Washington University in St. Louis School of Medicine and St. Louis Children's Hospital, St. Louis, Missouri. Electronic address: [Email]


Anomalous origin of the left pulmonary artery (ALPA) from the aorta is a rare form of congenital heart disease, leading to long-term morbidity if not treated early. There is a reported association of this anomaly with 22q11 microdeletion syndrome. Surgical repair is commonly performed by direct reimplantation to the main pulmonary artery with or without patch or flap techniques. This report presents two unique cases of ALPA, describing embryologic considerations for morphogenesis of ALPA with 22q11 microdeletion syndrome and a novel surgical technique involving unroofing of the unique anatomy of the common wall between the left and main pulmonary arteries.