Pulmonary arterial hypertension associated with congenital heart disease

Usha Krishnan; Erika B Rosenzweig

doi:10.1016/j.ccm.2013.08.011

Pulmonary arterial hypertension associated with congenital heart disease

Clin Chest Med. 2013 Dec;34(4):707-17. doi: 10.1016/j.ccm.2013.08.011. Epub 2013 Oct 17.

Authors

Usha Krishnan¹, Erika B Rosenzweig

Affiliation

¹ Columbia University Medical Center, Pulmonary Hypertension Center, Division of Pediatric Cardiology, 3959 Broadway, CH-2N, New York, NY 10032, USA.

PMID: 24267300
DOI: 10.1016/j.ccm.2013.08.011

Abstract

In the past decade, there have been more patients with congenital heart disease (CHD) surviving to adulthood; whether due to late repair, or complex underlying CHD, many of these patients will be faced with pulmonary arterial hypertension (PAH) associated with CHD (APAH-CHD). In this review, the authors discuss the most commonly encountered forms of APAH-CHD, how to interpret the hemodynamic data, and how to classify the patients into meaningful subgroups that have similar management strategies. The current state of targeted medical treatments available to patients with APAH-CHD is also discussed.

Keywords: Congenital heart disease; Eisenmenger syndrome; Fontan; Operability; Pulmonary arterial hypertension.

Publication types

Review

MeSH terms

Combined Modality Therapy
Familial Primary Pulmonary Hypertension
Heart Defects, Congenital / classification
Heart Defects, Congenital / complications*
Heart Defects, Congenital / surgery
Hemodynamics
Humans
Hypertension, Pulmonary / etiology*
Hypertension, Pulmonary / physiopathology
Hypertension, Pulmonary / therapy