Myotonic dystrophy is frequently associated with functional and anatomic derangements in
the myocardium. Ten myotonic dystrophy patients (seven men and three women, ages …

Myotonic dystrophy is an inherited systemic disorder affecting skeletal muscle and the heart.
Genetic testing for myotonic dystrophy is diagnostic and identifies those at risk for cardiac …

Muscular dystrophies are inherited disorders sharing similar clinical features and dystrophic
changes on muscle biopsy. Duchenne muscular dystrophy is the most common inherited …

Cardiac involvement is recorded in about 80% of patients affected by myotonic dystrophy
type 1 (DM1). The prevalence of cardiac conduction abnormalities is well described. Data …

We evaluated the progression of conduction system and myocardial disease in 17
asymptomatic myotonic dystrophy patients by clinical evaluation, electrocardiography, vector …

Objectives: Our aim was to determine whether myocardial fibrosis, detected by
cardiovascular magnetic resonance (CMR), represents an arrhythmogenic substrate in …

BACKGROUND: Myotonic dystrophy is associated with arrhythmias and risk of sudden
death but also with symptoms of heart failure. Our study aimed to identify early biventricular …

We sought to determine whether and to what degree myocardial myotonia might occur in
myotonic muscular dystrophy. Cardiac involvement manifests itself chiefly as abnormalities …

Objective We hypothesised that, in hypertrophic cardiomyopathy (HCM), late gadolinium
enhancement (LGE) on cardiac magnetic resonance (CMR) is progressive and can be …

Cardiac involvement in myotonic dystrophy type 1 (DM1) is well known. In contrast, the
severity and frequency of cardiac abnormalities in proximal myotonic myopathy (PROMM) …