CHADS-VASc 评分和 Fabry 特异性评分在无房颤的 Fabry 病患者中预测新发或复发性卒中和 TIA 的价值。
Value of the CHADS-VASc score and Fabry-specific score for predicting new-onset or recurrent stroke/TIA in Fabry disease patients without atrial fibrillation.
机构信息
Department of Internal Medicine I, University Hospital Würzburg, Oberdürrbacher Str. 6, 97080, Würzburg, Germany.
Comprehensive Heart Failure Center (CHFC), University of Würzburg, Würzburg, Germany.
出版信息
Clin Res Cardiol. 2018 Dec;107(12):1111-1121. doi: 10.1007/s00392-018-1285-4. Epub 2018 May 24.
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 CHADS-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).
目的
评估中风或短暂性脑缺血发作（TIA）的潜在风险因素，并检验 Fabry 特异性中风风险评分在无房颤（AF）的 Fabry 病（FD）患者中的可行性和疗效。
背景
FD 患者常在年轻时经历脑血管事件（中风/TIA）。
方法
共纳入 159 例经基因证实无 AF 的 FD 患者（年龄 40±14 岁，42.1%为男性），并确定中风/TIA 事件的风险因素。所有患者中位随访时间为 60（35-90 四分位间距）个月。预定义的主要结局包括新发或复发性中风/TIA 和全因死亡。
结果
既往中风/TIA（HR 19.97，P<0.001）、血管角皮瘤（HR 4.06，P=0.010）、肌酐升高（HR 3.74，P=0.011）、显著左心室肥厚（HR 4.07，P=0.017）和整体收缩应变降低（GLS，HR 5.19，P=0.002）仍然是无 AF 的 FD 患者新发或复发性中风/TIA 的独立风险预测因子。根据上述定义的风险因素建立了 Fabry 特异性评分，该评分在预测本队列新发或复发性中风/TIA 方面优于 CHADS-VASc 评分（AUC 0.87 与 0.75，P=0.199）。
结论
既往中风/TIA、血管角皮瘤、肾功能不全、左心室肥厚和整体收缩功能障碍是无 AF 的 FD 患者新发或复发性中风/TIA 的独立危险因素。基于上述定义的风险因素，使用 Fabry 特异性评分预测新的或复发性脑事件是可行的。需要进一步的研究来检验 Fabry 特异性评分（≥2 分）定义的新发或复发性中风/TIA 风险较高的 FD 患者是否可能从抗血栓治疗中获益。临床试验注册号 HEAL-FABRY（评估 Fabry 病患者心脏受累情况，NCT03362164）。
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