Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society

Circulation. 2015 Nov 24;132(21):2037-99. doi: 10.1161/CIR.0000000000000329. Epub 2015 Nov 3.


Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension.

Keywords: AHA Scientific Statements; bronchopulmonary dysplasia; congenital diaphragmatic hernia; congenital heart disease; genetics; persistent pulmonary hypertension of the newborn; sickle cell disease.

Publication types

  • Practice Guideline

MeSH terms

  • Cardiovascular Agents / therapeutic use
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Diagnostic Imaging / methods
  • Disease Management
  • Extracorporeal Membrane Oxygenation
  • Genetic Counseling
  • Heart Defects, Congenital / complications
  • Heart Defects, Congenital / therapy
  • Hernias, Diaphragmatic, Congenital / complications
  • Hernias, Diaphragmatic, Congenital / therapy
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / genetics
  • Hypertension, Pulmonary / therapy*
  • Infant
  • Infant, Newborn
  • Lung / embryology
  • Lung Transplantation
  • Nitric Oxide / administration & dosage
  • Nitric Oxide / therapeutic use
  • Oxygen Inhalation Therapy
  • Persistent Fetal Circulation Syndrome / diagnosis
  • Persistent Fetal Circulation Syndrome / therapy
  • Postoperative Complications / therapy
  • Respiration, Artificial / adverse effects
  • Respiration, Artificial / methods
  • Ventilator-Induced Lung Injury / prevention & control


  • Cardiovascular Agents
  • Nitric Oxide