Pulmonary arterial hypertension in children

Pediatr Pulmonol. 2004 Jul;38(1):2-22. doi: 10.1002/ppul.20051.


Pulmonary arterial hypertension is a serious progressive condition with a poor prognosis if not identified and treated early. Because the symptoms are nonspecific and the physical findings can be subtle, the disease is often diagnosed in its later stages. Remarkable progress has been made in the field of pulmonary arterial hypertension over the past several decades. The pathology is now better defined, and significant advances have occurred in understanding the pathobiologic mechanisms. Risk factors have been identified, and the genetics have been characterized. Advances in technology allow earlier diagnosis as well as better assessment of disease severity. Therapeutic modalities such as new drugs, e.g., epoprostenol, treprostinil, and bosentan, and surgical/interventional options, e.g., transplantation and atrial septostomy, which were unavailable several decades ago, have had a significant impact on prognosis and outcome. Thus, despite our inability to cure pulmonary arterial hypertension, advances in medical treatments over the past two decades have resulted in significant improvement in outcomes for children with various forms of pulmonary arterial hypertension. This report is a review the current state of the art for pulmonary arterial hypertension in 2004, with an emphasis on childhood pulmonary arterial hypertension and specific recommendations for current practice and future directions.

Publication types

  • Review

MeSH terms

  • Anticoagulants / therapeutic use
  • Calcium Channel Blockers / therapeutic use
  • Cardiac Catheterization
  • Cardiac Surgical Procedures / methods
  • Cause of Death*
  • Child
  • Child, Preschool
  • Drug Therapy, Combination
  • Epoprostenol / antagonists & inhibitors*
  • Epoprostenol / therapeutic use
  • Female
  • Genetic Therapy / methods
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / therapy*
  • Infant
  • Male
  • Nitric Oxide / therapeutic use
  • Physical Examination
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index
  • Survival Analysis


  • Anticoagulants
  • Calcium Channel Blockers
  • Nitric Oxide
  • Epoprostenol