Abstract 15415: Increase Arterial Stiffness in Patients with Later-Onset Cardiac Variant Fabry Disease
Background: The novel intron mutation ( IVS4+919G→A) has been reported to be associated with later-onset cardiac variant Fabry disease (FD) which could lead to left ventricular hypertrophy without other organ involvement. Aim of this study is to investigate the vascular properties between control and cardiac variant FD.
Method: 39 controls and 23 cardiac variant FD patients without difference in age and renal function underwent echocardiography for left ventricular mass index (LVMI) and vascular function evaluation including carotid-femoral pulse wave velocity(cfPWV), carotid augmentation pressure(AP), carotid augmentation index(AIx), and carotid intima-media thickness(IMT).
Result: The LVMI of cardiac variant FD are significant higher than control (116.13±46 vs. 81.98±18 g/m P=0.002 ). The AP and IMT, cfPWV are significantly higher in cardiac variant FD patients than in control(10.06±6.4 vs. 6.66± 6.2 mmHg P=0.047, 0.62±0.13 vs. 0.53± 0.13 cm P=0.015, 7.89±2 vs. 9.78±3.2 m/s P=0.006,).
Conclusion: There are significant increase in arterial stiffness in patients with cardiac variant FD. Our findings suggest that the late onset cardiac variant may have broader involvement in cardiovascular system .
- © 2012 by American Heart Association, Inc.