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KMID : 0368120140440050348
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Korean Circulation Journal
2014 Volume.44 No. 5 p.348 ~ p.350
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Intermittent Pre-Excitation-Syndrome in Facio-Scapulo-Humeral Muscular Dystrophy
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Finsterer Josef
Stollberger Claudia
Gatterer Edmund
Jakubiczka Sibylle
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Abstract
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Pre-excitation-syndrome has not been reported as a phenotypic feature of facio-scapulo-humeral muscular dystrophy (FSH-MD). In a 39-year-old male with FSH-MD due to a reduced tandem repeat size in the D4Z4-locus on chromosome 4q35, cardiac involvement, manifesting as 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 ventricular myocardium, was first recognised 10 years earlier. Follow-up at age 39 years revealed mild myocardial thickening, two intra-ventricular aberrant bands, and, surprisingly, intermittent pre-excitation on a routine electrocardiography. Cardiac involvement in FSH-MD may manifest as hypertrophic cardiomyopathy or various arrhythmias, of which one may be pre-excitation-syndrome.
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KEYWORD
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Heart, Muscular dystrophy, Fasuoscapulohumerales, Arrhythmia, Pre-excitation syndromes, Cardiomyopathies
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