Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension

Semin Perinatol. 2005 Apr;29(2):123-8. doi: 10.1053/j.semperi.2005.04.008.


Pulmonary hypertension complicates the course of many newborns with congenital diaphragmatic hernia. In the most severe cases, the fetal condition of markedly elevated pulmonary vascular resistance persists after birth and is associated with hypoxemic respiratory failure and severe disturbances in cardiac performance. Late pulmonary hypertension (weeks to months after birth) is increasingly recognized in this population, and chronic pulmonary vascular abnormalities (months to years after birth) are now being discovered. In this review, we will discuss the pathophysiology of acute, late, and chronic pulmonary hypertension in patients with congenital diaphragmatic hernia. We will also review the role of currently available pulmonary vasoactive drugs in the management of pulmonary hypertension in this population.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Acute Disease
  • Administration, Inhalation
  • Bronchodilator Agents / administration & dosage*
  • Chronic Disease
  • Hernia, Diaphragmatic / complications
  • Hernias, Diaphragmatic, Congenital*
  • Humans
  • Hypertension, Pulmonary / complications
  • Hypertension, Pulmonary / drug therapy*
  • Infant, Newborn
  • Nitric Oxide / administration & dosage*
  • Persistent Fetal Circulation Syndrome / complications
  • Persistent Fetal Circulation Syndrome / drug therapy


  • Bronchodilator Agents
  • Nitric Oxide