Pediatric Pulmonary Arterial Hypertension

Pediatr Clin North Am. 2020 Oct;67(5):903-921. doi: 10.1016/j.pcl.2020.06.005.

Abstract

Pulmonary hypertension (PH), the syndrome of increased pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children and is associated with a variety of potential underlying causes. Several pulmonary arterial hypertension-targeted therapies have become available to reduce pulmonary artery pressure and improve outcome, but there is still no cure for most patients. This review provides a description of select causes of PH encountered in pediatrics and an update on the most recent data pertaining to evaluation and management of children with PH. Available evidence for specific classes of PH-targeted therapies in pediatrics is discussed.

Keywords: Acute vasoreactivity testing; Atrial septal defect; Congenital heart disease; Operability; Pediatric pulmonary hypertension; Pulmonary vasodilators.

Publication types

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

MeSH terms

  • Child
  • Diagnostic Imaging*
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / diagnosis
  • Humans
  • Prognosis
  • Pulmonary Arterial Hypertension* / diagnosis
  • Pulmonary Arterial Hypertension* / etiology
  • Pulmonary Arterial Hypertension* / physiopathology
  • Pulmonary Artery / physiopathology*
  • Pulmonary Wedge Pressure / physiology*