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Marie-Louise Sveen, MD; Jens Jakob Thune, MD; Lars Køber, MD, DMSci; John Vissing, MD, DMSci

Arch Neurol. 2008;65(9):1196-1201.

Objective  To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD).

Design  Prospective screening.

Setting  Neuromuscular Clinic and Department of Cardiology at Rigshospitalet.

Patients  One hundred one patients with LGMD2A-I and BMD and 29 patients with LGMD2 and no molecular diagnosis.

Main Outcome Measures  Clinical investigation, echocardiography, and electrocardiographic findings.

Results  Cardiac involvement was present in 24 of 100 patients (24%) with LGMD2A-I and in 14 of 30 patients (47%) with BMD. Only a few patients with LGMD2A and unclassified LGMD2 had mild cardiac involvement, whereas 29% and 67% of patients with LGMD2I and LGMD2E, respectively, had cardiac involvement. Cardiac involvement was not correlated with age, muscle strength, or the level of dystrophic changes on muscle biopsy.

Conclusions  This study demonstrates a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD. Patients with LGMD2A, LGMD2D, and unclassified LGMD2 have a much lower and milder prevalence of cardiac involvement.

Author Affiliations: Neuromuscular Research Unit, Department of Neurology, Copenhagen Muscle Research Center (Drs Sveen and Vissing), and Department of Cardiology, University of Copenhagen, Rigshospitalet (Drs Thune and Køber), Copenhagen, Denmark.

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