Myotonic dystrophies (dystrophia myotonica, or DM) are inherited disorders characterized by
myotonia and progressive muscle degeneration, which are variably associated with a …

Myotonic dystrophy type 1 (DM1), the most common muscular dystrophy affecting adults and
children, is a multi-systemic disorder affecting skeletal, cardiac, and smooth muscles as well …

Background—Myotonic dystrophy type 2 (DM2) is a genetic disorder characterized by
skeletal muscle symptoms, metabolic changes, and cardiac involvement. Histopathologic …

Background Cardiac involvement is a major determinant of prognosis in type 1 myotonic
dystrophy (DM1), but limited information is available about myocardial remodeling and …

Myotonic dystrophy type 1 (DM1) is the most common adult form of muscular dystrophy,
presenting with a constellation of systemic findings secondary to a CTG triplet expansion of …

A variety of cardiomyopathies are due to familial disease. Most are primarily associated with
cardiac involvement and can lead to hypertrophic, dilated, or restrictive cardiomyopathy …

Conduction system disease and ventricular tachycardia (VT) are known to occur in myotonic
dystrophy (MD) patients. The most commonly reported VT is bundle branch reentry; 1 …

Bundle branch re-entry (BBR)-ventricular tachycardia (VT) is relatively rare with an
incidence of about 6% in sustained monomorphic VT series. However, physicians …

JUSTIFICATIVA E OBJETIVOS: A doença de Steinert é a forma de distrofia muscular mais
comum no adulto. Devido ao seu caráter multissistêmico o manuseio perioperatório é um …

Discussion Cardiac involvement in MD1 includes impairment of impulse generation,
including sinus bradycardia, atrial and ventricular ectopic beats, atrial fibrillation, atrial flutter …