Left Ventricular Geometry and Blood Pressure as Predictors of Adverse Progression of Fabry Cardiomyopathy.
Left Ventricular Geometry and Blood Pressure as Predictors of Adverse Progression of Fabry Cardiomyopathy.
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DOI：
10.1371/journal.pone.0140627
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发表时间：
2015
期刊：
影响因子：
3.7
通讯作者：
Weidemann F
中科院分区：
文献类型：
--
作者：
Krämer J;Bijnens B;Störk S;Ritter CO;Liu D;Ertl G;Wanner C;Weidemann F
尽管多项研究有助于描述法布里病 (FD) 中的心脏，但这种心肌病尚未完全被了解。此外，血压和几何形状改变的影响尚未得到系统评估。在 74 名 FD 患者（平均年龄 36±12 岁；45 名女性）中，使用心脏磁共振成像和晚期增强技术 (LE) 对心肌纤维化的程度及其进展进行了量化。将结果与标准超声心动图进行比较，并辅以 2D 斑点追踪、3D 球形指数 (SI) 和标准化血压测量。在基线时，没有患者接受酶替代疗法（ERT）。 51±24个月后进行随访检查。患有 LE 的患者与未患有 LE 的患者相比，收缩压 (SBP) 较高：123±17 mmHg 与 115±13 mmHg； P = 0.04。 SI 与 LE 阳性心肌数量之间存在正相关性（r = 0.51；P<0.001），表明较高的 SI 与心肌病的晚期阶段相关。基线时的 SI 与随访期间 LE 阳性心肌的增加呈正相关。最高的 SBP (125±19 mmHg) 和最高的 SI (0.32±0.05) 出现在 LE 快速增加的亚组中（即每年≥0.2%；n = 16；P = 0.04）。多变量 Logistic 回归分析（包括 SI、SBP、EF、左心室容积、壁厚度和 NT-proBNP，并根据年龄和性别进行调整）显示 SI 是检测 LE 快速进展的最有力参数（AUC = 0.785；P<0.05）。通过球形指数评估的左心室几何形状随着法布里心肌病的阶段而改变。尽管FD患者并不患有高血压，但SBP对心肌病的进展有明显的影响。
In spite of several research studies help to describe the heart in Fabry disease (FD), the cardiomyopathy is not entirely understood. In addition, the impact of blood pressure and alterations in geometry have not been systematically evaluated. In 74 FD patients (mean age 36±12 years; 45 females) the extent of myocardial fibrosis and its progression were quantified using cardiac magnetic-resonance-imaging with late enhancement technique (LE). Results were compared to standard echocardiography complemented by 2D-speckle-tracking, 3D-sphericity-index (SI) and standardized blood pressure measurement. At baseline, no patient received enzyme replacement therapy (ERT). After 51±24 months, a follow-up examination was performed. Systolic blood pressure (SBP) was higher in patients with vs. without LE: 123±17 mmHg vs. 115±13 mmHg; P = 0.04. A positive correlation was found between SI and the amount of LE-positive myocardium (r = 0.51; P<0.001) indicating an association of higher SI in more advanced stages of the cardiomyopathy. SI at baseline was positively associated with the increase of LE-positive myocardium during follow-up. The highest SBP (125±19 mmHg) and also the highest SI (0.32±0.05) was found in the subgroup with a rapidly increasing LE (ie, ≥0.2% per year; n = 16; P = 0.04). Multivariate logistic regression analysis including SI, SBP, EF, left ventricular volumes, wall thickness and NT-proBNP adjusted for age and sex showed SI as the most powerful parameter to detect rapid progression of LE (AUC = 0.785; P<0.05). LV geometry as assessed by the sphericity index is altered in relation to the stage of the Fabry cardiomyopathy. Although patients with FD are not hypertensive, the SBP has a clear impact on the progression of the cardiomyopathy.
DOI：
10.4250/jcu.2013.21.1.26
发表时间：
2013-03
期刊：
Journal of cardiovascular ultrasound
影响因子：
--
作者：
Yoon JY;Song JH;Cheon SS;Cho HJ;Bae MH;Lee JH;Yang DH;Park HS;Cho Y;Chae SC
通讯作者：
Chae SC