September 1, 2008Archives of Neurology
Cardiac Involvement in Patients With Limb-Girdle Muscular Dystrophy Type 2 and Becker Muscular Dystrophy
Key result
Becker and LGMD patients exhibit up to ~67% cardiac involvement regardless of age or strength.
- n=130
Why the study?
The extent of cardiac involvement in patients with recessively inherited limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy was not well characterized.
Comparison
Cardiac involvement across LGMD2 subtypes and BMD
Design
Prospective screening at neuromuscular and cardiology clinics
Authors
Discussion
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Implication
Supports screening consideration in these dystrophies; cross-sectional data leaves open outcome impact and prospective validation.
Study Design
Type
Observational (n=130)
Multicenter
No
Structured PICO
P
Population
130 patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy undergoing prospective cardiac screening.
E
Exposure
Prospective cardiac screening (clinical investigation, echocardiography, and electrocardiography)
O
Outcome
Cardiac involvement based on clinical investigation, echocardiography, and electrocardiographic findingssurrogate
There is a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD, highlighting the need for routine cardiac screening in these specific muscular dystrophy subtypes.
Cite This Study
Sveen et al. (2008) conducted an observational in Limb-Girdle Muscular Dystrophy Type 2 and Becker Muscular Dystrophy (n=130). Limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy was evaluated on Cardiac involvement based on clinical investigation, echocardiography, and electrocardiographic findings. Cardiac involvement was highly prevalent in patients with Becker muscular dystrophy (47%), LGMD2E (67%), and LGMD2I (29%), and was not correlated with age or muscle strength.