Focal right atrial dysplasia may serve as an arrhythmogenic substrate for atrial flutter and sudden death in patients with myotonic dystrophy.
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May flag atrial dysplasia in myotonic dystrophy flutter; extends histopathology and leaves open screening or risk-stratification studies.
Focal dysplasia of the right atrium was identified postmortem in a 22-year-old man with myotonic dystrophy and sudden death. Antemortem cardiac abnormalities included a single syncopal episode associated with atrial flutter with exercise-induced 1:1 atrioventricular conduction, sinus node dysfunction, and mild mitral valve prolapse. Pathologically there was only mild conduction system disease and the ventricular myocardium was normal. Right atrial dysplasia, previously unreported in myotonic dystrophy, appears to have been an arrhythmogenic lesion in this patient, serving as a morphological substrate for reentry.
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Pickering et al. (1989) studied this question.
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