S.I.3 Prevention of cardiac sudden death in patients with muscle diseases

Abstract Cardiac sudden death is one of the main complications accounting for reduced life expectancy in patients with myopathies. Sudden death can occur either in patients presenting cardiomyopathy with severe myocardial dysfunction, which are at risk for severe ventricular tachyarrhythmias, or in patients with muscle diseases associated with primary involvement of the cardiac conduction system leading to severe conduction defects or ventricular tachyarrhythmias, such as myotonic dystrophy type 1, laminopathies, desminopathies or mitochondrial diseases. Regarding the major impact of sudden death on patient prognosis, preventive strategies including prophylactic implantations of pacemaker or cardiac defibrillator or use of antiarrhythmic drugs should be discussed in patients with a high risk for severe cardiac arrhythmias. Since the natural history of cardiac involvement and mechanisms underlying sudden death differ from one disease to another, these strategies have to be specific for each muscle disease. However, regarding the low prevalence of muscle diseases, the identification of risk factors for sudden death is complex and, moreover, the benefit associated with preventive measures is particularly difficult to demonstrate and can hardly be based on randomized trials. I will discuss several disease-specific approaches for the prevention of sudden death.