Long Duration Holter ECG in Fabry Disease

Not Applicable

Recruiting

• Conditions: Fabry Disease
• Interventions: Diagnostic Test: Implantation (subcutaneous) of a marketed miniaturized Holter ECG recording device

• Registration Number: NCT04440254
• Lead Sponsor: Institut National de la Santé Et de la Recherche Médicale, France

Brief 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.

Detailed 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

Study Timeline

• Study First Submitted: 2020-06-12
• Study First Submitted QC: 2020-06-18
• Study First Posted: 2020-06-19
• Study Start: 2021-05-05
• Status Verified: 2025-01
• Last Update Submitted: 2025-01-21
• Last Update Posted: 2025-01-23
• Primary Completion: 2029-04-30
• Study Completion: 2029-04-30

Recruitment & Eligibility

• Status: RECRUITING
• Sex: Male
• Target Recruitment: 40

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

Exclusion Criteria

Not provided

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
ONE	Implantation (subcutaneous) of a marketed miniaturized Holter ECG recording device	One single group of patients

Primary Outcome Measures

Name	Time	Method
Occurrence of cardiac arrhythmias and conduction disorders	3 years

Secondary Outcome Measures

Name	Time	Method
Correlations between electrical disorders and changes in MRI/ultrasonic data (LV mass, T1 signal, fibrosis extent , systolic strain, ejection fraction), concentrations of BNP, troponin, Lyso-Gb3, MSSI score and renal function	3 years

Trial Locations

Locations (1)

20 Rue Leblanc, HEGP/PARCC, 75015 Paris; 🇫🇷 Paris, France