Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease
File(s)
Accepted version
Author(s)
Favoccia, Carla
Constantine, Andrew H
Wort, Stephen J
Dimopoulos, Konstantinos
Type
Journal Article
Abstract
Introduction: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD). It is characterised by a severe rise in pulmonary vascular resistance resulting in shunt reversal and cyanosis. Areas covered: In this paper, an overview of ES and other types of PAH related to CHD (PAH-CHD) in adults is provided. The modern management of PAH-CHD in tertiary centers, with emphasis on co-morbidities and complications is described. Expert opinion: PAH-CHD is a wide spectrum of conditions, of which ES is the archetype. The size and location of the shunt, the degree of adaptation of the right ventricle to the increased afterload and other compensatory mechanisms, such as secondary erythrocytosis, define the clinical presentation and natural history of these patients. PAH therapies have improved the quality of life and outcome of many patients with PAH-CHD, but expert multidisciplinary management remains essential in optimising the care of this rare and complex group of patients.
Date Issued
2019-01-01
Date Acceptance
2019-05-21
Citation
Expert Review of Cardiovascular Therapy, 17 (6), pp.449-459
ISSN
1477-9072
Publisher
Taylor & Francis
Start Page
449
End Page
459
Journal / Book Title
Expert Review of Cardiovascular Therapy
Volume
17
Issue
6
Copyright Statement
© 2019 Taylor & Francis. This is an Accepted Manuscript of an article published by Taylor & Francis in Expert Review of Cardiovascular Therapy on 06 Jun 2019, available online: https://doi.org/10.1080/14779072.2019.1623024.
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/31120797
Subjects
Eisenmenger syndrome
congenital heart disease
cyanosis
pulmonary arterial hypertension
secondary erythrocytosis
Publication Status
Published online
Coverage Spatial
England
Date Publish Online
2019-06-06