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Intermittent Pre-Excitation-Syndrome in Facio-Scapulo-Humeral Muscular Dystrophy
Josef Finsterer,Claudia Stöllberger,Edmund Gatterer,Sibylle Jakubiczka 대한심장학회 2014 Korean Circulation Journal Vol.44 No.5
Pre-excitation-syndrome has not been reported as a phenotypic feature of facio-scapulo-humeral muscular dystrophy (FSH-MD). In a39-year-old male with FSH-MD due to a reduced tandem repeat size in the D4Z4-locus on chromosome 4q35, cardiac involvement, manifestingas an incomplete right bundle-branch-block, tall T-waves in V 3-5, ST-elevation in V 2-4, and mild thickening of the left ventricularmyocardium, was first recognised 10 years earlier. Follow-up at age 39 years revealed mild myocardial thickening, two intra-ventricularaberrant bands, and, surprisingly, intermittent pre-excitation on a routine electrocardiography. Cardiac involvement in FSH-MD maymanifest as hypertrophic cardiomyopathy or various arrhythmias, of which one may be pre-excitation-syndrome.