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Clin Res Cardiol. 2018 Dec;107(12):1111-1121. doi: 10.1007/s00392-018-1285-4. Epub 2018 May 24.

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.

Author information

1
Department of Internal Medicine I, University Hospital Würzburg, Oberdürrbacher Str. 6, 97080, Würzburg, Germany.
2
Comprehensive Heart Failure Center (CHFC), University of Würzburg, Würzburg, Germany.
3
Medizinische Klinik I, Klinikum Vest, Recklinghausen, Germany.
4
Department of Internal Medicine I, University Hospital Würzburg, Oberdürrbacher Str. 6, 97080, Würzburg, Germany. nordbeck_p@ukw.de.
5
Comprehensive Heart Failure Center (CHFC), University of Würzburg, Würzburg, Germany. nordbeck_p@ukw.de.

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).

KEYWORDS:

Fabry disease; Global systolic strain; Stroke; Transient ischemic attack

PMID:
29797054
PMCID:
PMC6244978
DOI:
10.1007/s00392-018-1285-4
[Indexed for MEDLINE]
Free PMC Article

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