Congenital heart disease and pulmonary hypertension.
Journal Article (Journal Article;Review)
Many patients with congenital heart disease and systemic-to-pulmonary shunts develop pulmonary arterial hypertension (PAH), particularly if the cardiac defect is left unrepaired. A persistent increase in pulmonary blood flow may lead to obstructive arteriopathy and increased pulmonary vascular resistance, a condition that can lead to reversal of shunt and cyanosis (Eisenmenger syndrome). Cardiac catheterization is crucial to confirm diagnosis and facilitate treatment. Bosentan is the only medication to date to be compared with placebo in a randomized controlled trial specifically targeting congenital heart disease-associated PAH. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation is reserved for recalcitrant cases.
Full Text
Duke Authors
Cited Authors
- Gupta, V; Tonelli, AR; Krasuski, RA
Published Date
- July 2012
Published In
Volume / Issue
- 8 / 3
Start / End Page
- 427 - 445
PubMed ID
- 22748904
International Standard Serial Number (ISSN)
- 1551-7136
Digital Object Identifier (DOI)
- 10.1016/j.hfc.2012.04.002
Language
- eng
Conference Location
- United States