Abnormal origin of the left pulmonary artery from the descending aorta and heterotaxy syndrome: an undescribed phenotypic association

Abstract

AbstractExtensive screening in a newborn with prenatal suspicion of VACTERL syndrome identified an anomalous origin of the left pulmonary artery from the descending aorta with an arterial duct and left aortic arch, and normal intra-cardiac anatomy. Other anatomical anomalies suggested heterotaxy syndrome. At one-month-old, re-implantation of the 3.5 mm left pulmonary artery was performed by direct tension-low anastomosis. Post-operative course was complicated by severe left pulmonary atelectasis, and the patient died 20 days later.