Combination of PGE1 and Pulmonary Vasodilator Therapy in Managing a Challenging Case of Severe PAH Secondary to CDH

World J Pediatr Congenit Heart Surg. 2020 Jul;11(4):525-527. doi: 10.1177/2150135120912678.

Abstract

Congenital diaphragmatic hernia (CDH) is a rare disease, which affects 1 in 2,500 newborns. Congenital diaphragmatic hernia can interfere with the normal development of the pulmonary parenchyma and vascular bed, and in severe cases, it can lead to the development of severe pulmonary arterial hypertension (PAH) and right ventricular failure. We present a neonate with CDH who developed severe PAH and right ventricular dysfunction and was managed with a unique strategy combining venoarterial extracorporeal membrane oxygenation, prostaglandin E1, and a variety of PAH therapies.

Publication types

  • Case Reports

MeSH terms

  • Administration, Inhalation
  • Alprostadil / therapeutic use*
  • Drug Therapy, Combination
  • Echocardiography
  • Endothelium-Dependent Relaxing Factors / administration & dosage
  • Extracorporeal Membrane Oxygenation / methods*
  • Female
  • Hernias, Diaphragmatic, Congenital / complications*
  • Hernias, Diaphragmatic, Congenital / diagnosis
  • High-Frequency Ventilation / methods
  • Humans
  • Infant, Newborn
  • Nitric Oxide / administration & dosage*
  • Pulmonary Arterial Hypertension / etiology
  • Pulmonary Arterial Hypertension / physiopathology
  • Pulmonary Arterial Hypertension / therapy*
  • Pulmonary Artery / physiopathology
  • Pulmonary Wedge Pressure / physiology*
  • Severity of Illness Index
  • Vasodilator Agents / therapeutic use

Substances

  • Endothelium-Dependent Relaxing Factors
  • Vasodilator Agents
  • Nitric Oxide
  • Alprostadil