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

Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey. Research Abstract Details 

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Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey. Abstract Text:

Ales Linhart,Christoph Kampmann, Zamorano,Gere Sunder-Plassmann,Michael Beck,Atul Mehta,Perry M Elliott, ,Ales Linhart,Christoph Kampmann, Zamorano,Gere Sunder-Plassmann,Michael Beck,Atul Mehta,Perry M Elliott, ,

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.

Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey. Publishing Authors By Initials

A Linhart,C Kampmann,JL Zamorano,G Sunder-Plassmann,M Beck,A Mehta,PM Elliott, ,A Linhart,C Kampmann,JL Zamorano,G Sunder-Plassmann,M Beck,A Mehta,PM Elliott, ,

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Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: European heart journal

VOLUME: 28

Page Numbers: 1228-35

Journal Abbreviation: Eur. Heart J.

ISSN: 0195-668X

DAY: 5

MONTH: 05

YEAR: 2007

Cardiac manifestations of Anderson-Fabry disease: results from the international Fabry outcome survey. Information

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LANGUAGE: eng

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AFFILIATION: Second Department of Internal Medicine, First Faculty of Medicine, Charles University, Prague, Czech Republic. alinh@lf1.cuni.cz

Country: England

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MEDLINETA: Eur Heart J

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