Long Duration Holter ECG in Fabry Disease

Institut National de la Santé Et de la Recherche Médicale, France

Status

Enrolling

Conditions

Fabry Disease

Treatments

Diagnostic Test: Implantation (subcutaneous) of a marketed miniaturized Holter ECG recording device

Study type

Interventional

Funder types

Other

Identifiers

NCT04440254

C19-57

Details and patient eligibility

About

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.

Full description

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

Enrollment

40 estimated patients

Sex

Male

Ages

18+ years old

Volunteers

No Healthy Volunteers

Inclusion and exclusion criteria

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