Long Duration Holter ECG in Fabry Disease
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Implantation (subcutaneous) of a marketed miniaturized Holter ECG recording device.
- Who it may be relevant to
- Registry conditions: Fabry Disease. Basic parameters: from 18 years · Male.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- France
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Official title
Natural History of Cardiac Rhythm and Conduction Disorders in Patients With Fabry Disease Evaluated by Long-Term Implantable Holter ECG
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.
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
Interventions
- Diagnostic test Implantation (subcutaneous) of a marketed miniaturized Holter ECG recording device
Subcutaneously implanted under local anesthesia of the device on the presternal or subclavicular region
Primary outcome measures
- Occurrence of cardiac arrhythmias and conduction disorders [Time frame: 3 years]
Secondary outcome measures (1)
- 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 [Time frame: 3 years]
Eligibility 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
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Allocation
- N/A
- Model
- Single group
- Masking
- Open label
- Primary purpose
- Diagnostic
Study locations
France · 1 center
- 20 Rue Leblanc, HEGP/PARCC, 75015 Paris — Paris
Identifiers
NCT: NCT04440254 · C19-57