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Pulmonary arterial hypertension in congenital heart diseases.

Authors
  • Beghetti, Maurice
  • Tissot, Cecile
Type
Published Article
Journal
Seminars in Respiratory and Critical Care Medicine
Publisher
Georg Thieme Verlag KG
Publication Date
Aug 01, 2009
Volume
30
Issue
4
Pages
421–428
Identifiers
DOI: 10.1055/s-0029-1233311
PMID: 19634081
Source
Medline
License
Unknown

Abstract

Pulmonary hypertension complicates the course of many children and adults with congenital heart diseases (CHDs). The increase in pulmonary pressure associated with CHD is secondary to either increased pulmonary blood flow or increased postcapillary pressures. Pulmonary arterial hypertension is in the vast majority associated with congenital cardiac shunts. Despite major advances in the understanding of the regulation of the pulmonary vascular bed and the pulmonary endothelial lesions leading to pulmonary vascular disease, despite the advances in surgical repair and the discovery of potential therapies in the pre- and postoperative period, pulmonary hypertension still carries a significant mortality and morbidity in patients with CHD. The recent introduction of targeted therapies in other forms of pulmonary arterial hypertension has led to a renewed interest in pulmonary hypertension associated with CHD and this particularly for the most advanced form, the so-called Eisenmenger syndrome (ES). This review summarizes the current knowledge on pulmonary hypertension associated with CHD, focusing on the pathophysiology and treatment of ES.

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