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).


METHODS
Prospective screening.


METHODS
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.


METHODS
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.
Sveen
Marie-Louise
ML
Neuromuscular Research Unit 3342, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark. RH18329@rh.hosp.dk
Thune
Jens Jakob
JJ
Køber
Lars
L
Vissing
John
J
D000293:Adolescent; D000328:Adult; D000368:Aged; D002318:Cardiovascular Diseases / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D049288:Muscular Dystrophies, Limb-Girdle / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D011446:Prospective Studies