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What is the best way to detect infra-Hisian conduction abnormalities and prevent sudden cardiac death in myotonic dystrophy?
  1. Denis Duboc1,
  2. Karim Wahbi2
  1. 1Department of Cardiology, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France
  2. 2Myology Institute, Hôpital Pitié-Salpêtrière, APHP, Université Pierre et Marie Curie, Paris, France
  1. Correspondence to Professor Denis Duboc, Department of Cardiology, Hôpital Cochin, APHP, Université Paris Descartes, Paris 75014, France; denis.duboc{at}cch.aphp.fr

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Myotonic dystrophy type 1 (DM1), also called Steinert's disease, is a multisystemic disorder for which the cardiologist is often consulted. Clinical manifestations of this disease, which involves multiple systems, may include muscle weakness, myotonia, multiple endocrinal disorders, cataract, respiratory insufficiency, conduction system disease, supraventricular arrhythmias, ventricular tachyarrhythmias and dilated cardiomyopathy.1–3

Sudden cardiac death is the second most common mode of death after respiratory failure in this population and occurs in up to one-third of patients.4 As DM1 becomes apparent in most cases by extracardiac manifestations, neurologists usually refer patients to cardiologists in order to evaluate their risk of cardiac sudden death and to suggest the best measures to prevent it. In this case, interventional measures are part of a primary prevention strategy in patients, often with no personal history of cardiac disease, such …

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