Pulmonary arterial hypertension associated with congenital heart disease: recent advances and future directions

Int J Cardiol. 2014 Dec 15;177(2):340-7. doi: 10.1016/j.ijcard.2014.09.024. Epub 2014 Sep 28.


Congenital heart disease (CHD), the most common inborn defect, affects approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to a dramatic improvement in patients' quality of life and long-term survival prospects. However, recently it has been realised that many of these patients are affected by ongoing and life-long cardiac issues, namely residual and progressive haemodynamic lesions, arrhythmia and sudden cardiac death, as well as the development of chronic heart failure and pulmonary arterial hypertension (PAH) - all of which merit tertiary care. Unfortunately, many patients with CHD are lost to follow-up, due to the assumption that their initial response to surgical and or catheter intervention in childhood led to cure. Furthermore, there are many patients with undiagnosed or unoperated CHD in the developing world coming to medical attention during adulthood. Our article focuses on advances in the management of PAH associated with CHD, a common association with an adverse impact on quality of life and survival prospects that affects approximately 10% of patients with CHD. Much of the recent progress in PAH-CHD has focused on the extreme end of the disease spectrum, namely on Eisenmenger syndrome. Herein we discuss this progress and future directions for this emerging cardiovascular field.

Keywords: Congenital heart disease; Eisenmenger syndrome; Fontan circulation; Left-to-right shunts; Pulmonary arterial hypertension.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Eisenmenger Complex / diagnosis
  • Eisenmenger Complex / physiopathology
  • Eisenmenger Complex / therapy
  • Forecasting
  • Heart Defects, Congenital / diagnosis*
  • Heart Defects, Congenital / physiopathology*
  • Heart Defects, Congenital / therapy
  • Hemodynamics / physiology
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / physiopathology*
  • Hypertension, Pulmonary / therapy