Long Duration Holter ECG in Fabry Disease

Fabry Disease Clinical Trial

Official title:

Natural History of Cardiac Rhythm and Conduction Disorders in Patients With Fabry Disease Evaluated by Long-Term Implantable Holter ECG

Clinical Trial Details — Status: Not yet recruiting

Administrative data

• NCT number: NCT04440254
• Other study ID #: C19-57
• Status: Not yet recruiting
• Phase: N/A
• Start date: September 1, 2020
• Est. completion date: September 1, 2025

Study information

• Verified date: May 2020
• Source: Institut National de la Santé Et de la Recherche Médicale, France
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

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

Recruitment information / eligibility

• Status: Not yet recruiting
• Enrollment: 40
• Est. completion date: September 1, 2025
• Est. primary completion date: September 1, 2025
• Accepts healthy volunteers: No
• Gender: Male
• Age group: 18 Years and older

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

Related Conditions & MeSH terms

• Fabry Disease

Intervention

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

Locations

Country	Name	City	State
n/a

Sponsors (1)

Lead Sponsor	Collaborator
Institut National de la Santé Et de la Recherche Médicale, France

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Occurrence of cardiac arrhythmias and conduction disorders		3 years
Secondary	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