Pulmonary Hypertension in Adults with Congenital Heart Disease.

Journal Article (Journal Article;Review)

Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD) affects 5% to 10% of adults with CHD and is associated with significant morbidity and mortality. PAH-CHD develops as a consequence of intracardiac or extracardiac systemic-to-pulmonary shunts that lead to pulmonary vascular remodeling through a pathologic process that is similar to other causes of PAH. Eisenmenger syndrome is the most severe phenotype of PAH-CHD and is characterized by severe elevation in pulmonary vascular resistance, with shunt reversal causing hypoxemia and central cyanosis. The primary management strategy for most patients with PAH-CHD is medical therapy, although defect closure is considered in select cases.

Full Text

Duke Authors

Cited Authors

  • Goldstein, SA; Krasuski, RA

Published Date

  • February 2022

Published In

Volume / Issue

  • 40 / 1

Start / End Page

  • 55 - 67

PubMed ID

  • 34809917

Electronic International Standard Serial Number (EISSN)

  • 1558-2264

Digital Object Identifier (DOI)

  • 10.1016/j.ccl.2021.08.006


  • eng

Conference Location

  • Netherlands