Pulmonary hypertension associated with congenital heart disease: a practical review for the pediatric cardiologist

Congenit Heart Dis. 2012 Nov-Dec;7(6):575-83. doi: 10.1111/chd.12012. Epub 2012 Oct 18.


Pulmonary hypertension (PH) is a complex condition with a broad range of etiologies that result in a common outcome--elevated pulmonary arterial pressure. For the pediatric cardiologist, this entity provides a manifest demonstration of the interrelationship of cardiac and pulmonary physiology. A thoughtful approach must be applied to each patient with PH in order to achieve a thorough understanding of the nature of disease and to proceed with individualized therapy in an appropriate and timely manner. This article aims to provide a clinically relevant, practical review of current literature related to PH in patients with congenital heart disease, including the causes, diagnostic considerations, and an overview of management.

Publication types

  • Review

MeSH terms

  • Age Factors
  • Arterial Pressure*
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / physiopathology
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Prognosis
  • Pulmonary Artery / physiopathology*
  • Risk Factors
  • Vasoconstriction