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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
Published on: August 8, 2022
[Risk stratification of sudden death in hypertrophic cardiomyopathy in 2016]
Olivier Dubourg1, Philippe Charron1, Marc Sirol1
1Assistance publique-Hôpitaux de Paris (AP-HP), hôpital Ambroise-Paré, université de Versailles-Saint Quentin (UVSQ), centre de référence pour les maladies cardiaques héréditaires, pôle V thorax vasculaire digestif et néphrologie, 92100 Boulogne, France; Inserm U-1018, CESP équipe 5 (EpReC, épidémiologie rénale et cardiovasculaire), UVSQ, 94807 Villejuif, France.
Abstract:
Patients with hypertrophic cardiomyopathy (HCM) are at an increased risk of death from many causes and sudden cardiac death is one of them. The study of the sudden cardiac death of patients with HCM has allowed the identification of risk factors and among them major risk factor are: family history of sudden cardiac death, the occurrence of syncope/dizziness, the existence of non-sustained ventricular tachycardia an abnormal blood pressure response during stress test, presence of severe left ventricular hypertrophy≥30mm. Risk stratification for sudden cardiac death is essential, for symptomatic or asymptomatic HCM patients. Two approaches are possible: the classical approach or risk stratification methods with major risk factors and the new approach using the risk-calculator recommended by the ESC. Both methods are not in opposition but complementary. The risk stratification in hypertrophic cardiomyopathy should be still improved to be sure that only the most high-risk patients receive an implantable cardiac defibrillator.
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