Restoring Fetal Circulation as a Means of Bridging Treatment Prior to Surgical Repair of Anomalous Origin of the Right Pulmonary Artery from the Ascending Aorta with Persistent Pulmonary Hypertension of the Newborn

Pediatr Cardiol. 2018 Apr;39(4):848-851. doi: 10.1007/s00246-018-1849-0. Epub 2018 Mar 2.

Abstract

Anomalous origin of one pulmonary artery from the ascending aorta is a rare congenital anomaly. Even more rarely reported is its presence in conjunction with persistent pulmonary hypertension of the newborn (PPHN). We present a case of a full-term infant, initially thought to have PPHN and later found to have anomalous origin of the right pulmonary artery from the ascending aorta. We discuss our management concept which included use of PgE1 infusion to restore fetal circulation prior to surgical treatment in this unique clinical scenario.

Keywords: Anomalous pulmonary artery; Fetal circulation; Persistent pulmonary hypertension of the newborn; Prostaglandin.

MeSH terms

  • Alprostadil / therapeutic use*
  • Aorta / abnormalities
  • Aorta / surgery
  • Computed Tomography Angiography
  • Echocardiography
  • Female
  • Heart Defects, Congenital / diagnosis*
  • Heart Defects, Congenital / drug therapy
  • Heart Defects, Congenital / surgery
  • Humans
  • Hypertension, Pulmonary / drug therapy
  • Hypertension, Pulmonary / etiology*
  • Imaging, Three-Dimensional
  • Infant, Newborn
  • Preoperative Care / methods
  • Pulmonary Artery / abnormalities*
  • Pulmonary Artery / surgery
  • Vasodilator Agents / therapeutic use*

Substances

  • Vasodilator Agents
  • Alprostadil

Supplementary concepts

  • Familial anomalous origin of right pulmonary artery