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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Model of chronic adaptation: right ventricular function in Eisenmenger syndrome

Gerhard-Paul Diller1,2, Konstantinos Dimopoulos1,2, Henryk Kafka3, Siew Yen Ho4 and Michael A. Gatzoulis1,2,*

1 Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK
2 National Heart and Lung Institute, Imperial College, London, UK
3 Division of Cardiology, Queen’s University Cardiovascular Laboratory, Kingston General Hospital, Kingston, ON, Canada
4 Cardiac Morphology, National Heart and Lung Institute, Imperial College London and Royal Brompton Hospital, London, UK

* Corresponding author. Tel: +44 20 73518602; fax: +44 20 73518629. E-mail address: m.gatzoulis{at}rbh.nthames.nhs.uk

Pulmonary arterial hypertension (PAH) is commonly associated with adult congenital heart disease. Eisenmenger syndrome—severe pulmonary hypertension with shunt reversal and cyanosis—represents the extreme manifestation of PAH in patients with congenital heart disease and has become the epitome of PAH in this setting. Despite representing a multi-system disorder and being associated with numerous complications, survival prospects for patients with Eisenmenger syndrome are superior compared with patients with idiopathic pulmonary hypertension. We review aetiology, clinical presentation, and prognosis of Eisenmenger syndrome and discuss potential explanations for the remarkable resilience of the right ventricle in this setting.

Key Words: Pulmonary arterial hypertension • Eisenmenger syndrome • Right ventricular function


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