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Open Access 01-12-2018 | Original Paper

Value of the CHA₂DS₂-VASc score and Fabry-specific score for predicting new-onset or recurrent stroke/TIA in Fabry disease patients without atrial fibrillation

Authors: Dan Liu, Kai Hu, Marie Schmidt, Jonas Müntze, Octavian Maniuc, Daniel Gensler, Daniel Oder, Tim Salinger, Frank Weidemann, Georg Ertl, Stefan Frantz, Christoph Wanner, Peter Nordbeck

Published in: Clinical Research in Cardiology | Issue 12/2018

Abstract

Objectives

To evaluate potential risk factors for stroke or transient ischemic attacks (TIA) and to test the feasibility and efficacy of a Fabry-specific stroke risk score in Fabry disease (FD) patients without atrial fibrillation (AF).

Background

FD patients often experience cerebrovascular events (stroke/TIA) at young age.

Methods

159 genetically confirmed FD patients without AF (aged 40 ± 14 years, 42.1% male) were included, and risk factors for stroke/TIA events were determined. All patients were followed up over a median period of 60 (quartiles 35–90) months. The pre-defined primary outcomes included new-onset or recurrent stroke/TIA and all-cause death.

Results

Prior stroke/TIA (HR 19.97, P < .001), angiokeratoma (HR 4.06, P = .010), elevated creatinine (HR 3.74, P = .011), significant left ventricular hypertrophy (HR 4.07, P = .017), and reduced global systolic strain (GLS, HR 5.19, P = .002) remained as independent risk predictors of new-onset or recurrent stroke/TIA in FD patients without AF. A Fabry-specific score was established based on above defined risk factors, proving somehow superior to the CHA₂DS₂-VASc score in predicting new-onset or recurrent stroke/TIA in this cohort (AUC 0.87 vs. 0.75, P = .199).

Conclusions

Prior stroke/TIA, angiokeratoma, renal dysfunction, left ventricular hypertrophy, and global systolic dysfunction are independent risk factors for new-onset or recurrent stroke/TIA in FD patients without AF. It is feasible to predict new or recurrent cerebral events with the Fabry-specific score based on the above defined risk factors. Future studies are warranted to test if FD patients with high risk for new-onset or recurrent stroke/TIA, as defined by the Fabry-specific score (≥ 2 points), might benefit from antithrombotic therapy. Clinical trial registration HEAL-FABRY (evaluation of HEArt invoLvement in patients with FABRY disease, NCT03362164).

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Title: Value of the CHA2DS2-VASc score and Fabry-specific score for predicting new-onset or recurrent stroke/TIA in Fabry disease patients without atrial fibrillation
Authors: Dan Liu
Kai Hu
Marie Schmidt
Jonas Müntze
Octavian Maniuc
Daniel Gensler
Daniel Oder
Tim Salinger
Frank Weidemann
Georg Ertl
Stefan Frantz
Christoph Wanner
Peter Nordbeck
Publication date: 01-12-2018
Publisher: Springer Berlin Heidelberg
Published in: Clinical Research in Cardiology / Issue 12/2018
Print ISSN: 1861-0684
Electronic ISSN: 1861-0692
DOI: https://doi.org/10.1007/s00392-018-1285-4

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Value of the CHA₂DS₂-VASc score and Fabry-specific score for predicting new-onset or recurrent stroke/TIA in Fabry disease patients without atrial fibrillation

Abstract

Objectives

Background

Methods

Results

Conclusions

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