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Finsterer J, Stöllberger C, Gatterer E, et al. Intermittent pre-excitation-syndrome in facio-scapulo-humeral muscular dystrophy. Korean Circ J. 2014;44(5):348-50doi: 10.4070/kcj.2014.44.5.348.
Finsterer, J., Stöllberger, C., Gatterer, E., & Jakubiczka, S. (2014). Intermittent pre-excitation-syndrome in facio-scapulo-humeral muscular dystrophy. Korean circulation journal, 44(5), 348-50. https://doi.org/10.4070/kcj.2014.44.5.348
Finsterer, Josef, et al. "Intermittent pre-excitation-syndrome in facio-scapulo-humeral muscular dystrophy." Korean circulation journal vol. 44,5 (2014): 348-50. doi: https://doi.org/10.4070/kcj.2014.44.5.348
Finsterer J, Stöllberger C, Gatterer E, Jakubiczka S. Intermittent pre-excitation-syndrome in facio-scapulo-humeral muscular dystrophy. Korean Circ J. 2014 Sep;44(5):348-50. doi: 10.4070/kcj.2014.44.5.348. Epub 2014 Sep 25. PMID: 25278989; PMCID: PMC4180613.
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Korean Circ J. 2014 Sep;44(5):348-50. doi: 10.4070/kcj.2014.44.5.348. Epub 2014 Sep 25.

Intermittent pre-excitation-syndrome in facio-scapulo-humeral muscular dystrophy.

Korean circulation journal

Josef Finsterer, Claudia Stöllberger, Edmund Gatterer, Sibylle Jakubiczka

Affiliations

  1. Krankenanstalt Rudolfstiftung, Vienna, Austria.
  2. Second Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
  3. Institute of Human Genetics, University Hospital Magdeburg, Magdeburg, Germany.

PMID: 25278989 PMCID: PMC4180613 DOI: 10.4070/kcj.2014.44.5.348

Abstract

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.

Keywords: Arrhythmia; Cardiomyopathies; Heart; Muscular dystrophy, Fasuoscapulohumerales; Pre-excitation syndromes

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