Eisenmenger syndrome: current perspectives
File(s)
Author(s)
Type
Journal Article
Abstract
Abstract: Eisenmenger syndrome (ES) is the most severe form of pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). It results from a cardiac defect allowing significant systemic-to-pulmonary (left-to-right) shunting, which triggers the development of pulmonary vascular disease (PVD) if the defect is not repaired in a timely fashion. Once severe PVD has developed, the defect cannot be repaired. With advances in pediatric cardiology and surgery, the prevalence of ES is steadily falling in developed countries; nonetheless, there will always be patients who are unsuitable for repair at the time of diagnosis, or emigrating from countries with less advanced healthcare, who will develop ES. ES is a multisystem disorder causing chronic hypoxemia and reduced cardiac output resulting in significant morbidity and mortality. While lung (plus defect repair) or combined heart and lung transplantation is thought be the definitive treatment for ES, transplant organs are a limited resource and long-term results are still suboptimal. PAH pharmacotherapy was, until quite recently, largely directed at symptomatic relief and had no impact on morbidity and mortality. Targeted PAH therapies have recently been proven to be beneficial in various forms of PAH in terms of functional status, progression of disease, and prognosis. Data on the effect of PAH therapies in the ES cohort remain limited, but available studies demonstrate evidence of improvement in symptoms, exercise capacity, and some evidence of survival benefit. ES patients should be followed in specialized centers, by means of an interdisciplinary approach by clinicians experienced in PAH and CHD. However, local physicians working in cardiology, respiratory medicine, primary care, and emergency services are likely to encounter ES patients and need to be aware of the main issues and pitfalls in their care. The authors present an overview of the management of ES, focusing on the most common issues and complications.
Date Issued
2017-02-02
Date Acceptance
2016-10-25
Citation
Research Reports in Clinical Cardiology, 2017, 8, pp.1-12
ISSN
1179-8475
Publisher
Dove Medical Press
Start Page
1
End Page
12
Journal / Book Title
Research Reports in Clinical Cardiology
Volume
8
Copyright Statement
© 2017 Nashat et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work
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you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php).
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Subjects
Science & Technology
Life Sciences & Biomedicine
Cardiac & Cardiovascular Systems
Cardiovascular System & Cardiology
Eisenmenger syndrome
pulmonary arterial hypertension
congenital heart disease
cyanosis
pulmonary arterial hypertension therapies
hyperviscosity
thrombosis
bleeding
pregnancy
Down syndrome
CONGENITAL HEART-DISEASE
PULMONARY-ARTERIAL-HYPERTENSION
RANDOMIZED CONTROLLED-TRIAL
IMPROVES EXERCISE CAPACITY
PLACEBO-CONTROLLED TRIAL
DOUBLE-BLIND
LUNG TRANSPLANTATION
INTERNATIONAL SOCIETY
IRON-DEFICIENCY
DOWN-SYNDROME
Publication Status
Published