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

Cardiol Young. 2021 Jul;31(7):1193-1196. doi: 10.1017/S1047951121002183. Epub 2021 Jun 4.

Abstract

Extensive 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.

Keywords: CHD; anomalous left pulmonary artery; descending aorta; heterotaxy syndrome; vascular surgery.

MeSH terms

  • Aorta, Thoracic / diagnostic imaging
  • Aorta, Thoracic / surgery
  • Ductus Arteriosus*
  • Female
  • Heterotaxy Syndrome* / diagnosis
  • Heterotaxy Syndrome* / diagnostic imaging
  • Humans
  • Infant
  • Infant, Newborn
  • Lung
  • Pregnancy
  • Pulmonary Artery / diagnostic imaging