Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome

Cardiol Clin. 2015 Nov;33(4):599-609, ix. doi: 10.1016/j.ccl.2015.07.003. Epub 2015 Aug 13.


Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient's hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology, diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations.

Keywords: Adult congenital heart disease; Humans vasodilator agents/diagnostic use/therapeutic use; Pulmonary arterial hypertension disease management; Pulmonary hypertension.

Publication types

  • Review

MeSH terms

  • Adult
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / physiopathology*
  • Heart Defects, Congenital / surgery
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / therapy*