Long Duration Holter ECG in Fabry Disease

Overview

The main objective is to assess the occurrence of cardiac arrhythmias and conduction disorders during a three-year follow-up using implantable Holter ECG monitoring in 40 patients with Fabry disease. The secondary objectives are to analyze the correlations of these anomalies with changes in cardiac MRI and echocardiographic parameters as biological parameters and overall severity of the disease assessed by MSSI.

The main objective of this non-blinded, monocentric non-randomized study is to assess the occurrence of cardiac arrhythmias and conduction disorders (sinus dysfunction, branch block \[BB\], atrioventricular block \[BAV\], sustained \[TVS\] or non-supported \[TVNS\] ventricular tachycardias, atrial fibrillation \[AF\] during a three-year follow-up in 40 patients with Fabry disease (half with left ventricular hypertrophy) using an implantable Holter ECG device (Medtronic Reveal-LINQ™). The secondary objectives are to analyze the correlations (at 1, 2 and 3 years) of these anomalies with the modifications of : * cardiac MRI \[Magnetic Resonance Imaging\] data at inclusion \[M0\] and at 36 months \[M36\] (left ventricular hypertrophy \[HVG\], amplitude of the T1 signal, extent of fibrosis); MRIs will be performed before implantation and after removal of the Holter to avoid the reading artifacts linked to the device. * echocardiographic measurements of the left ventricle (overall longitudinal strain systolic \[SGL\] and ejection fraction \[FE\]) * biological parameters (BNP or NT-proBNP, ultra-sensitive troponin and Lyso-Gb3) * the overall severity of the disease assessed by MSSI (Mainz Severity Score Index), DFG and proteinuria

Conditions

Interventions

Eligibility

Locations (1)

Outcomes

Central Contacts