An observational study in Hereditary Heart Diseases, sponsored by Nantes University Hospital. Recruiting at 15 sites in 3 countries. Open to participants aged 1 Year to 100 Years. Per ClinicalTrials.gov, last updated 2026-10-27.
Sponsored by Nantes University Hospital · Observational
Sudden cardiac death (SCD) is one of the leading causes of death in developed countries. These deaths (more than 5,000 per year in France) are due to hereditary arrhythmias or cardiomyopathies. Early diagnosis of SCD is often achieved through family screening, but the main challenge is to stratify the risk of SCD in these patients. Indeed, prevention of SCD relies mainly on the implantation of an automatic defibrillator. The challenge is to identify patients who will develop SCD and avoid implanting an implantable cardioverter defibrillator (ICD) in patients who will never develop arrhythmias but who will face complications related to the ICD (inappropriate shocks, infection, lead failure), leading to a reduced quality of life and significant costs for the healthcare system. However, there is a lack of relevant clinical and biological markers for risk stratification, which rules out any possibility of preventive screening. Most of the clinical and ECG (electrocardiogram) parameters identifying an increased risk of SCD have not been reproduced in replication studies.
In this project, the investigator will develop a data processing and analysis pipeline using artificial intelligence methods to assess the individual risk of serious arrhythmic events or heart failure in patients with hereditary arrhythmic diseases or cardiomyopathies through the automated processing of multimodal data (clinical data, electrocardiogram (ECG), imaging (echocardiography, MRI magnetic resonance imaging), genetic data, biomarkers).
The eligibility of patients (index cases and related cases) for the study will be determined during a cardiology consultation or day hospitalisation, carried out as part of routine care.
The investigator undertakes to obtain the person's free, informed and express consent, collected in writing, after providing them with oral and written information on the protocol and allowing them sufficient time to reflect. In the case of a minor patient, the investigator undertakes to inform the minor patient and their legal representatives orally and in writing and to obtain the minor's assent, i.e. their oral or written agreement depending on their age, and the written consent of their legal representatives.
Specific acts for research:
Clinical data will be collected in a parameterised and secure eCRF (electronic Case Report Form).
Patients will be followed in the routine care for maximum 10 years. Arrhythmias, heart failure and sudden cardiac death will be collected during follow-up.
A data processing and analysis pipeline using artificial intelligence methods to assess the individual risk of serious arrhythmic events or heart failure in patients with hereditary arrhythmic diseases or cardiomyopathies through the automated processing of multimodal data (clinical data, electrocardiogram (ECG), imaging (echocardiography, MRI magnetic resonance imaging), genetic data, biomarkers) will be developped.
Nantes University Hospital is the lead sponsor of 839 studies on the registry; 195 are open to participants now.
Counted across the registry records on this site, refreshed daily.
All adult and minor patients with hereditary heart disease or cardiomyopathies and their relatives.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
I. hereditary heart disease II. All relatives of patients III. Patients referred to the reference centre for suspected hereditary rhythm disorders or cardiomyopathies IV. Written consent V. social security scheme
Exclusion Criteria :
I. Patients participating in a therapeutic trial that may interfere with the research results II. Patients under guardianship or curatorship.
Collection of two additional EDTA tubes (2 x 5 ml) blood sample
Arrhythmic and heart failure risk stratification
to assess the arrhythmic risk and/or risk of heart failure in patients with hereditary heart disease at 5, 8 and 10 years, using a model combining clinical, electrocardiographic, imaging, genetic and biomarker data.
Time frame: 5, 8 and 10 years
Demographics data
Determine the risk of arrhythmia and/or heart failure based on demographics data
Time frame: 5, 8 and 10 years
Diagnosis of Brugada syndrome
Evaluate the diagnostic performance of the model combining clinical, electrocardiographic, genetic and biomarker data for the identification of Brugada syndrome.
Time frame: 5 years
Started Feb 2026 (actual). Primary completion is estimated for Feb 2036, 120 months after the start.
How these figures are worked out, and which studies are left out of them.
From the registry record's own update history. This site started tracking changes on Sep 25, 2026; for anything earlier, see the record history on ClinicalTrials.gov ↗
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