Pulmonary Hypertension and Congenital Heart Disease

Todd S Roth; Jamil A Aboulhosn

doi:10.1016/j.ccl.2016.04.002

Pulmonary Hypertension and Congenital Heart Disease

Cardiol Clin. 2016 Aug;34(3):391-400. doi: 10.1016/j.ccl.2016.04.002.

Authors

Todd S Roth¹, Jamil A Aboulhosn²

Affiliations

¹ Memorial Cardiac and Vascular Institute, Joe DiMaggio Children's Hospital Adult Congenital Heart Disease Center, 3501 Johnson Street, Hollywood, FL 33021, USA. Electronic address: troth@mhs.net.
² Ahmanson/UCLA Adult Congenital Heart Disease Center, Medicine and Pediatrics David Geffen School of Medicine at UCLA, 100 UCLA Medical Plaza, Suite 630, Los Angeles, CA 90095, USA.

PMID: 27443136
DOI: 10.1016/j.ccl.2016.04.002

Abstract

Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is a frequent complication in adults with congenital heart disease. Regardless of etiology, the optimal treatment strategy for this difficult population is challenging. The new frontier of targeted PAH therapies has demonstrated improved functional capacity in the various phenotypes of PAH-CHD, with work currently in progress scrutinizing outcomes. In those who fail conventional medical therapy, heart and heart-lung (block) transplantation become the final therapeutic options, with the role of ventricular assist devices and the total artificial heart still under investigation in this group.

Keywords: Adults with congenital heart disease (ACHD); Eisenmenger syndrome (ES); Pulmonary arterial hypertension (PAH); Targeted, catheter-based, surgical therapies.

Publication types

Review

MeSH terms

Global Health
Heart Defects, Congenital / complications*
Heart Defects, Congenital / epidemiology
Humans
Hypertension, Pulmonary / epidemiology
Hypertension, Pulmonary / etiology*
Incidence