Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2014 Dec 15;177(2):340-7.
doi: 10.1016/j.ijcard.2014.09.024. Epub 2014 Sep 28.

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

Michael A Gatzoulis  1 , Maurice Beghetti  2 , Michael J Landzberg  3 , Nazzareno Galiè  4
Affiliations
Review

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

Michael A Gatzoulis et al. Int J Cardiol. .

Abstract

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.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

Publication types

MeSH terms

LinkOut - more resources