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 journalArticle

234 Scopus citations

Abstract

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
Volume62
Issue number25 SUPPL.
DOIs
StatePublished - Dec 24 2013

Keywords

  • congenital heart disease
  • pediatrics
  • pulmonary hypertension

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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    Ivy, D. D., Abman, S. H., Barst, R. J., Berger, R. M. F., Bonnet, D., Fleming, T. R., Haworth, S. G., Raj, J. U., Rosenzweig, E. B., Schulze Neick, I., Steinhorn, R. H., & Beghetti, M. (2013). Pediatric pulmonary hypertension. Journal of the American College of Cardiology, 62(25 SUPPL.). https://doi.org/10.1016/j.jacc.2013.10.028