European Heart Journal Advance Access originally published online on June 22, 2006
European Heart Journal 2006 27(14):1737-1742; doi:10.1093/eurheartj/ehl116
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Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case–control study
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 of Science and Medicine, London, UK
3 Department of Paediatric Cardiology and Biomedical Engineering, University Hospital of Schleswig-Holstein, Kiel, Germany
4 Department of Cardiology, Hammersmith Hospital, London, UK
Received 7 December 2005; revised 9 April 2006; accepted 1 June 2006; online publish-ahead-of-print 22 June 2006.
* Corresponding author. Tel: +44 207 351 8602; fax: +44 207 351 8629. E-mail address: m.gatzoulis{at}rbh.nthames.nhs.uk
See page 1644 for the editorial comment on this article (doi:10.1093/eurheartj/ehi823)
Aims To characterize contemporary Eisenmenger patients at a large centre for adult congenital heart disease, assess survival prospects, and identify predictors of death in this population.
Methods and results All Eisenmenger patients under follow-up at our centre since 2000 (n=171, mean age 37±12 years) were included. To identify predictors of mortality, a case–control study was performed. Data including symptoms, functional class, medication, laboratory, and electrocardiographic and echocardiographic parameters are presented. Iron deficiency was common and strongly related to phlebotomy (relative risk 4.1, P<0.0001). Haemoglobin concentration was inversely related to arterial oxygen saturations in iron-replete patients (P<0.001) but not in iron-deficient patients. During a median follow-up of 67 months, 20 patients died. Survival at 40, 50, and 60 years of age was 94, 74, and 52%, respectively. When compared with healthy individuals, median survival was reduced by 
20 years in Eisenmenger patients and was worst in those with complex lesions. Predictors of mortality included functional class, signs of heart failure, history of clinical arrhythmia, QRS duration and QTc interval, and low serum albumin and potassium levels.
Conclusion Despite good short-term prognosis, life expectancy is markedly reduced in Eisenmenger patients. Markers of heart failure and parameters associated with arrhythmia are of prognostic value in terms of mortality and may guide clinicians caring for Eisenmenger patients.
Key Words: Eisenmenger syndrome • Congenital heart disease • Pulmonary hypertension • Survival
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