Long Duration Holter ECG in Fabry Disease

Summary

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.

Eligibility

Inclusion Criteria:

* Male patient
* Age greater than or equal to 18 years on the day of inclusion
* Presence of a morbid mutation for MF
* Signature of the informed consent form
* Absence of significant valve disease, verified on medical file (absence stenosis or regurgitation \<2+ in color Doppler on a scale 1 to 4+ by extension of the jet)
* No history of known or documented myocardial infarction nor CAD
* No pacemaker or ICD
* no history of AF, NSVT, high-degree AV block
* Correct echogenicity
* No treatment by corticosteroid or immunosuppressive drugs
* creatinine clearance \>/= 30 Ml/mn
* LVEF ≥ 50% by ultrasound and / or MRI
* No contraindication to MRI (or claustrophobia) and gadolinium injection
* Affiliation to the French social security insurance

Conditions2

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