title: Intermittent pre-excitation-syndrome in facio-scapulo-humeral muscular dystrophy.
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.
authors.affiliation: Krankenanstalt Rudolfstiftung, Vienna, Austria.
authors.forename: Josef
authors.initials: J
authors.lastname: Finsterer
authors.affiliation: Second Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
authors.forename: Claudia
authors.initials: C
authors.lastname: Stöllberger
authors.affiliation: Second Medical Department, Krankenanstalt Rudolfstiftung, Vienna, Austria.
authors.forename: Edmund
authors.initials: E
authors.lastname: Gatterer
authors.affiliation: Institute of Human Genetics, University Hospital Magdeburg, Magdeburg, Germany.
authors.forename: Sibylle
authors.initials: S
authors.lastname: Jakubiczka
keywords: Arrhythmia; Cardiomyopathies; Heart; Muscular dystrophy, Fasuoscapulohumerales; Pre-excitation syndromes