Background: There is limited data on the causes and predictors of mortality in patients (pts)
with myotonic dystrophy type 1 (DM1) evaluated and treated with modern medical therapy in …

Myotonic Dystrophy type 1 (DM1) is an autosomal dominant neuromuscular disorder
characterized by progressive muscle weakness and cardiac involvement. Routine cardiac …

Abstract Myotonic dystrophy type 1 (DM1) is the commonest muscular dystrophy in adults,
affecting multiple organs in addition to skeletal muscles. Cardiac conduction system …

Methods| The source cohort is a Johns Hopkins Hospital institutional review board–
approved prospective open cohort of consecutive patients with MMD referred to the …

Background Myotonic dystrophy type 1 (MD1) is a neuromuscular disorder with potential
involvement of the heart and increased risk of sudden death. Considering the importance of …

So far, myotonic dystrophy (DM) has been linked to two distinct loci: DM1, associated with an
abnormal CTG triplet expansion in the 3'-untranslated region of the myotonin-protein kinase …

Background Type 1 myotonic dystrophy (DM1) patients' prognosis is very poor. Up until now,
only a few prognostic factors for cardiovascular events have been identified, and they are …

Objective To study the prevalence and natural evolution of arrhythmic events and
conduction disturbances in myotonic dystrophy; to correlate the genetic defect with …

Abstract Myotonic Dystrophy type 1 (DM1) is a multisystemic disease, autosomal dominant,
caused by a CTG repeat expansion in DMPK gene. We assessed the appropriateness of …

Background: Pacemakers (PM) and implantable cardioverter defibrillators (ICD) may be
indicated for sudden death prevention in myotonic dystrophy type 1 (DM1), however the risk …