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Fayssoil A et al. – Steinert’s disease is an autosomal dominant neuromuscular disorder. Heart involvement is characterized by conduction system abnormalities, supraventricular and ventricular arrhythmias. Patients with Steinert’s disease are reported to have a higher incidence of sudden death. Preventing sudden death is crucial in this disease. It relies on non–invasive and/or invasive approach for risk stratification.


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