Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy.

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.