Intermittent Pre-Excitation-Syndrome in Facio-Scapulo-Humeral Muscular Dystrophy.
10.4070/kcj.2014.44.5.348
- Author:
Josef FINSTERER
1
;
Claudia STOLLBERGER
;
Edmund GATTERER
;
Sibylle JAKUBICZKA
Author Information
1. Krankenanstalt Rudolfstiftung, Vienna, Austria. fifigs1@yahoo.de
- Publication Type:Case Report
- Keywords:
Heart;
Muscular dystrophy, Fasuoscapulohumerales;
Arrhythmia;
Pre-excitation syndromes;
Cardiomyopathies
- MeSH:
Adult;
Arrhythmias, Cardiac;
Cardiomyopathies;
Cardiomyopathy, Hypertrophic;
Electrocardiography;
Follow-Up Studies;
Heart;
Humans;
Male;
Muscular Dystrophies*;
Myocardium;
Pre-Excitation Syndromes;
Tandem Repeat Sequences
- From:Korean Circulation Journal
2014;44(5):348-350
- CountryRepublic of Korea
- Language:English
-
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.
