Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease

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Title: Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease
Authors: Favoccia, C
Constantine, AH
Wort, SJ
Dimopoulos, K
Item 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.
Issue Date: 6-Jun-2019
Date of Acceptance: 21-May-2019
URI: http://hdl.handle.net/10044/1/70517
DOI: https://doi.org/10.1080/14779072.2019.1623024
ISSN: 1477-9072
Publisher: Taylor & Francis
Journal / Book Title: Expert Review of Cardiovascular Therapy
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.
Keywords: Eisenmenger syndrome
congenital heart disease
cyanosis
pulmonary arterial hypertension
secondary erythrocytosis
Eisenmenger syndrome
congenital heart disease
cyanosis
pulmonary arterial hypertension
secondary erythrocytosis
Cardiovascular System & Hematology
1102 Cardiorespiratory Medicine and Haematology
1117 Public Health and Health Services
Publication Status: Published online
Conference Place: England
Embargo Date: 2020-05-23
Online Publication Date: 2019-06-06
Appears in Collections:National Heart and Lung Institute



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