Key result
A 39-year-old male with facio-scapulo-humeral muscular dystrophy exhibited cardiac involvement manifesting as intermittent pre-excitation on routine electrocardiography.
Case Report (n=1)
This case report provides the first evidence that cardiac involvement in facio-scapulo-humeral muscular dystrophy may manifest as pre-excitation syndrome.
Suggests pre-excitation as possible cardiac feature in FSHD; hypothesis-generating and leaves open need for larger studies.
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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Finsterer et al. (2014) conducted a case report in Facio-scapulo-humeral muscular dystrophy (FSH-MD) (n=1). Facio-scapulo-humeral muscular dystrophy was evaluated on Cardiac involvement (intermittent pre-excitation). A 39-year-old male with facio-scapulo-humeral muscular dystrophy exhibited cardiac involvement manifesting as intermittent pre-excitation on routine electrocardiography.