Persistent Pulmonary Hypertension

Robin H Steinhorn, Steven H. Abman

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations


Persistent pulmonary hypertension of the newborn (PPHN) can occur with parenchymal disease, with lung hypoplasia, or without associated lung disease (idiopathic) and has an incidence of 0.2% in term infants and up to 2% in preterm infants. Maternal factors such as diabetes, high body mass index, smoking, use of selective serotonin receptor inhibitors or nonsteroidal antiinflammatory drugs, and cesarean delivery increase the risk of PPHN. Postnatal factors include perinatal asphyxia, hyperoxia, hypoxia, infection, and lung inflammation. Medical management of PPHN requires careful optimization of right and left ventricular function. Lung recruitment strategies should be applied in patients with parenchymal lung disease. Inhaled nitric oxide improves oxygenation and reduces the need for extracorporeal membrane oxygenation support in term and near-term infants with PPHN. Extracorporeal membrane oxygenation support is indicated for term and near-term neonates with severe pulmonary hypertension and/or hypoxemia that is refractory to inhaled nitric oxide therapy and optimization of respiratory and cardiac function. Chronic pulmonary hypertension occurs in a subset of infants with congenital diaphragmatic hernia and bronchopulmonary dysplasia and increases morbidity and mortality. Infants who survive moderate to severe PPHN are at a high risk of neurodevelopmental impairment and should undergo neuroimaging and neurodevelopmental follow-up.

Original languageEnglish (US)
Title of host publicationAvery's Diseases of the Newborn
Subtitle of host publicationTenth Edition
PublisherElsevier Inc.
ISBN (Electronic)9780323401722
ISBN (Print)9780323401395
StatePublished - Dec 27 2017
Externally publishedYes


  • Lung Development
  • Nitric Oxide
  • Pulmonary Hypertension
  • Pulmonary vasculature
  • Pulmonary vasodilators

ASJC Scopus subject areas

  • Medicine(all)


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