Pediatric pulmonary hypertension

D. Dunbar Ivy, Steven H. Abman, Robyn J. Barst, Rolf M F Berger, Damien Bonnet, Thomas R. Fleming, Sheila G. Haworth, J. Usha Raj, Erika B. Rosenzweig, Ingram Schulze Neick, Robin H Steinhorn, Maurice Beghetti

Research output: Contribution to journalArticlepeer-review

287 Scopus citations


Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.

Original languageEnglish (US)
JournalJournal of the American College of Cardiology
Issue number25 SUPPL.
StatePublished - Dec 24 2013


  • congenital heart disease
  • pediatrics
  • pulmonary hypertension

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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