Prenatal diagnosis of anomalous origin of pulmonary artery

Prenat Diagn. 2018 Apr;38(5):310-317. doi: 10.1002/pd.5235. Epub 2018 Mar 23.

Abstract

Background: Anomalous origin of the pulmonary arteries is a rare congenital pulmonary vascular malformation that includes unilateral absence of the pulmonary artery (UAPA), anomalous origin of unilateral pulmonary artery (AOPA), and left pulmonary artery sling (LPAS).

Methods: We analyze 15 cases of fetal pulmonary artery abnormalities from 2011 to 2017, detected via prenatal ultrasound at our center.

Results: The 15 cases include UAPA (5), AOPA (6), and LPAS (4). Of the UAPA cases, 2 had pulmonary atresia and 3 had pulmonary artery stenosis. In 5 cases, the descending aorta issued collateral vessels to the left lung. Of the AOPA cases, 2 had left pulmonary artery abnormalities originating in the ascending aorta and 4 showed right pulmonary artery abnormalities originating in the ascending aorta. Of the LPAS cases, 2 were type IA, 1 was type IIA, and 1 was type IIB. Two of the LPAS were associated with right lung dysplasia.

Conclusion: Abnormal origin of the pulmonary artery has a characteristic ultrasonographic appearance. The branch of the pulmonary artery cross section can provide important clues to the diagnosis of abnormal pulmonary artery origin. Pulmonary artery abnormalities are often associated with pulmonary atresia or stenosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Female
  • Heart Defects, Congenital / diagnostic imaging*
  • Humans
  • Pregnancy
  • Pulmonary Artery / abnormalities*
  • Pulmonary Artery / diagnostic imaging
  • Retrospective Studies
  • Ultrasonography, Prenatal
  • Young Adult