How to optimize decision making for defibrillator implantation in hypertrophic cardiomyopathy?
Hypertrophic cardiomyopathy (HCM) is a genetic heart disease affecting around 120,000 people in France. It is a major cause of sudden cardiac death, particularly in athletes under 35.
Today, the only effective prevention of sudden cardiac death, apart from exercise restriction, is the implantable automatic defibrillator (ICD), but the indications for its implantation remain controversial.
To address this issue, the Pr Philippe Charron launches the project OPTIM-HCM. Its main objective is to improve decision-making in the field of ICDs by developing a new prediction model of sudden cardiac death in hypertrophic cardiomyopathy based on imaging and genetic biomarkers.
The context: how to improve the decision to implant an automatic defibrillator?
What is hypertrophic cardiomyopathy (HCM)? This disease is characterized by abnormal thickening of the heart muscle (myocardium), which stiffens the left ventricular muscle and thus impedes the heart’s pumping function.
- Symptoms: loss of consciousness, chest pain, shortness of breath, heart failure, etc.
- Possible complications: atrial arrhythmia (with risk of stroke) or ventricular arrhythmia (with risk of sudden death).
In view of the possible dramatic evolution of HCM, the identification of patients who should receive an implantable automatic defibrillator (ICD) is of paramount importance.
However, the indications for ICD implantation remain controversial. Coming from the American Society of Cardiology, the French National Authority for Health (HAS) and the European Society of Cardiology, the various recommendations are based on retrospective studies with significant limitations:
- Lack of predictive power of the features on which it is based,
- Failure to take into account recently identified MRI and genetic risk markers,
- Failure to take into account recently identified MRI and genetic risk markers,
- Failure to take into account the adverse effects of defibrillators,
- No medico-economic evaluation.
As a result, improvements in HCM management are urgently needed.
The OPTIM-HCM project: a prospective study to propose new recommendations
The main aim of the OPTIM-HCM research project is to improve decision-making in the field of automatic implantable defibrillators, by developing a new model for predicting sudden cardiac death in hypertrophic cardiomyopathy. This will reduce mortality and improve patients’ quality of life.
- The 1st prospective study to take into account recent risk markers such as magnetic resonance imaging (MRI) and genetics in a multivariate model, and to integrate potential adverse effects of ICDs into the overall decision-making strategy.
- Designed to propose a revision of international recommendations for defibrillator implantation in patients with HCM.
Progress of the OPTIM-HCM research project
Our hypothesis is that the data available and measurable non-invasively in vivo in MRI, are very under-exploited in this pathology, despite their richness and accuracy.
The OPTIM-HCM study therefore aims to generate detailed, innovative and multimodal complementary data using the latest imaging and genetic techniques, in order to evolve the sudden death risk score currently used for the decision to implant an automatic implantable defibrillator.
The project draws on the joint expertise of our ICAN Imaging Core Lab, part of the Pitié Salpêtrière cardiovascular imaging team (ICT) and the cardiovascular imaging team at the Laboratoire d’Imagerie Biomédicale (LIB, Sorbonne University, INSERM, CNRS), under the supervision of Pr Alban Redheuil, de Nadjia Kachenoura (DR INSERM) et Khaoula Bouazizi (IR INSERM).
By mid-2023, 600 cardiac MRI scans from some 30 centers in France have already been analyzed to study biomarkers of cardiac function, structure and morphology.
- Studied data: clinical data, MRI, echocardiography, genetic data, biological data
- Total study duration: 72 months
- Total budget: €1,063,698
- Project sponsor: Pr Philippe Charron, PU-PH, Head of the Genomics and Pathophysiology of Myocardial Diseases research team (UMR 1166/IHU ICAN) and Coordinator of the Reference Center for Hereditary and Rare Heart Diseases (Hôpital de la Pitié-Salpêtrière, AP-HP).
The essential role of our patrons
Le Triomphe du cœur, a private endowment fund for research into sudden death in adults, has joined forces with the ICAN IHU teams to support the OPTIM-HCM project.
We warmly thank this new patron for his commitment and confidence!
This project still needs support!
Contact Francine Trocmé (Director of Patronage and Communication IHU ICAN) to find out more about our research into hypertrophic cardiomyopathy, and how you can support us.
