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


  • eng

Conference Location

  • United States