Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome

Eric V Krieger; Peter J Leary; Alexander R Opotowsky

doi:10.1016/j.ccl.2015.07.003

Pulmonary Hypertension in Congenital Heart Disease: Beyond Eisenmenger Syndrome

Cardiol Clin. 2015 Nov;33(4):599-609, ix. doi: 10.1016/j.ccl.2015.07.003. Epub 2015 Aug 13.

Authors

Eric V Krieger¹, Peter J Leary², Alexander R Opotowsky³

Affiliations

¹ Seattle Adult Congenital Heart Service, Division of Cardiology, Department of Medicine, University of Washington School of Medicine, 1959 Northeast Pacific Street, Seattle, WA 98195, USA. Electronic address: ekrieger@u.washington.edu.
² Pulmonary Vascular Disease Program, Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington School of Medicine, 1959 Northeast Pacific Street, Seattle, WA 98195, USA.
³ Boston Adult Congenital Heart and Pulmonary Hypertension Service, Department of Cardiology, Boston Children's Hospital and Brigham and Women's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.

PMID: 26471823
DOI: 10.1016/j.ccl.2015.07.003

Abstract

Patients with adult congenital heart disease have an increased risk of developing pulmonary hypertension. There are several mechanisms of pulmonary hypertension in patients with adult congenital heart disease, and understanding them requires a systematic approach to define the patient's hemodynamics and physiology. This article reviews the updated classification of pulmonary hypertension in patients with adult congenital heart disease with a focus on pathophysiology, diagnostics, and the evaluation of pulmonary hypertension in special adult congenital heart disease populations.

Keywords: Adult congenital heart disease; Humans vasodilator agents/diagnostic use/therapeutic use; Pulmonary arterial hypertension disease management; Pulmonary hypertension.

Publication types

Review

MeSH terms

Adult
Heart Defects, Congenital / complications*
Heart Defects, Congenital / physiopathology*
Heart Defects, Congenital / surgery
Humans
Hypertension, Pulmonary / diagnosis*
Hypertension, Pulmonary / etiology
Hypertension, Pulmonary / therapy*