The diagnosis, clinical characteristics and prognostic factor of Fabry disease in the Korean population: A multicenter, prospective study

Korea University Anam Hospital0 个研究点目标入组 2,000 人开始时间: 待定最近更新: 2019年4月23日

适应症

试验速览

暂无简介。

•Inclusion criteria
•1. Conventional screening criteria: severe left ventricular hypertrophy or suspicious of HCM (Interventricular septal thickness >13mm in transthoracic echocardiography)
•2. Unexplained left ventricular hypertrophy (left ventricular mass index = 115 g/m2 in men, = 95 g/m2 in women)
•With at least one of following criteria
•- Diastolic dysfunction
•Diastolic dysfunction is diagnosed by American Society of Echocardiography recommendation.
•? At least 2 positive findings as followings:
•1) Averaged E/e’ >14
•2) Septal e’ <7cm/sec
•3) Tricuspid regurgitant peak velocity >2.9m/sec
•4) Left atrial volume index >34 ml/m2
•? Restrictive pattern of diastolic dysfunction [E/A ratio=2], which is suspected to infiltrative disease of cardiac muscle
•- Abnormal electrocardiography (ECG) findings with associated with Fabry disease, including short PR (<120ms), abnormal P-Q interval or QRS width, bradyarrhythmia or ventricular arrhythmia.
•3. Family members of index patients

•Exclusion criteria
•1. Left ventricular hypertrophy which is caused by other mechanisms, including aortic stenosis, hypertension or diabetes.
•2. Multiple myeloma
•3. Congenital heart disease
•4. Previous cardiac surgery

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