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European Heart Journal Advance Access published online on May 5, 2007
European Heart Journal, doi:10.1093/eurheartj/ehm153
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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Cardiac manifestations of Anderson–Fabry disease: results from the international Fabry outcome survey

Ales Linhart1,*, Christoph Kampmann2, José L. Zamorano3, Gere Sunder-Plassmann4, Michael Beck2, Atul Mehta5, Perry M. Elliott on behalf of European FOS Investigators6

1 Second Department of Internal Medicine, First Faculty of Medicine, Charles University, Prague, Czech Republic
2 Department of Pediatrics, University of Mainz, Mainz, Germany
3 Department of Cardiology, Hospital Clínico San Carlos, Madrid, Spain
4 Division of Nephrology and Dialysis, Department of Medicine III, University of Vienna, Vienna, Austria
5 Department of Haematology, Royal Free Hospital, London, UK
6 The Heart Hospital, University College London, London, UK

Received 8 November 2006; revised 7 March 2007; accepted 6 April 2007.

* Corresponding author. Tel: +420 2 2496 2605; fax: +420 2 24912154. E-mail address: alinh{at}lf1.cuni.cz

Aims: Anderson–Fabry disease (AFD) is an uncommon X-linked disorder caused by deficient activity of the lysosomal enzyme {alpha}-galactosidase A. The Fabry Outcome Survey is a European database designed to monitor the long-term efficacy and safety of enzyme replacement therapy (ERT) with agalsidase alfa. The aim of this study was to determine the prevalence and characteristics of cardiac disease in AFD patients.

Methods and results: Clinical and laboratory data were available in 714 patients from 11 countries (mean age 35 ± 17 years, 369 women, 336 treated). The prevalence of angina was 23 vs. 22%; palpitations and arrhythmias 27 vs. 26%; exertional dyspnoea 23 vs. 23%; and syncope 2 vs. 4%, in women and men, respectively (all P = NS). The frequency of all cardiac symptoms was significantly higher in treated than in untreated patients. Gender, age, and glomerular filtration rate were independent determinants of echocardiographically assessed left ventricular hypertrophy (LVH).

Conclusion: This study confirms the high prevalence of cardiac morbidity associated with AFD. The disease burden in treated women exceeds that of untreated men, suggesting that most women selected for ERT have advanced disease. The presence of LVH is associated with higher frequency of cardiac signs and symptoms and relates independently to gender, age, and renal function.

Key Words: Anderson–Fabry disease • Cardiomyopathy • Left ventricular hypertrophy • Lysosomal storage disease • Agalsidase alfa


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