Persistent Pulmonary Hypertension of the Newborn

J Formos Med Assoc. 2013 Apr;112(4):177-84. doi: 10.1016/j.jfma.2012.11.007. Epub 2013 Jan 3.


Persistent pulmonary hypertension of the newborn (PPHN) is a severe pulmonary disorder which occurs at a rate of one in every 500 live births. About 10-50% of the victims will die of the problem and 7-20% of the survivors develop long-term impairments such as hearing deficit, chronic lung disease, and intracranial bleed. Most adult survivors show evidence of augmented pulmonary vasoreactivity, suggesting a phenotypical change. Several animal models have been used to study the pathophysiology and help to develop new therapeutic modality for PPHN. The etiology of PPHN can be classified into three groups: (1) abnormally constricted pulmonary vasculature as a result of parenchymal diseases; (2) hypoplastic pulmonary vasculature; and (3) normal parenchyma with remodeled pulmonary vasculature. Impaired vasorelaxation of pulmonary artery and reduced blood vessel density in lungs are two characteristic findings in PPHN. Medical treatment includes sedation, oxygen, mechanical ventilation, vasorelaxants (inhaled nitric oxide, inhaled or intravenous prostacyclin, intravenous prostaglandin E1, magnesium sulfate), and inotropic agents. Phosphodiesterase inhibitors have recently been studied as another therapeutic agent for PPHN. Endothelin-1 (ET-1) inhibitors have been studied in animals and a case of premature infant with PPHN successfully treated with an ET-I inhibitor has been reported in the literature. Surfactants have been reported as an adjunct treatment for PPHN as a complication of meconium aspiration syndrome. Even with the introduction of several new therapeutic modalities there has been no significant change in survival rate. Extracorporeal membrane oxygenator is used when medical treatment fails and the patient is considered to have a recoverable cause of PPHN.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Animals
  • Disease Models, Animal
  • Extracorporeal Membrane Oxygenation
  • Fetal Growth Retardation / physiopathology
  • Humans
  • Nitric Oxide Synthase Type III / physiology
  • Persistent Fetal Circulation Syndrome / diagnosis
  • Persistent Fetal Circulation Syndrome / etiology*
  • Persistent Fetal Circulation Syndrome / therapy
  • Respiration, Artificial
  • Risk Factors


  • Nitric Oxide Synthase Type III