Subclinical cardiological involvement in Sicilian patients with pure congenital muscular dystrophy.
OBJECTIVE
Congenital muscular dystrophies (CMD) are a heterogeneous group of diseases characterized by a marked weakness, generalized hypotonia, joint contractures and a muscle biopsy suggestive of dystrophic changes. The aim of this study has been to assess the prevalence of heart involvement in merosin-positive CMD patients examined by echocardiography and Doppler.


METHODS
A total of 10 merosin-positive CMD patients and 100 healthy children were evaluated by echocardiography and Doppler examinations and the results were compared.


RESULTS
Three out of 10 patients showed a reduction of the ejection fraction and of the shortening fraction; in 1 case, a dilatation of left ventricular diastolic diameter was observed.


CONCLUSIONS
In the 10 patients examined by echocardiography and Doppler, a heart involvement has been observed in 25% of cases.
Falsaperla
R
R
Department of Pediatrics, University of Catania, Catania, Italy. raffaelefalsaperla@hotmail.com
Romeo
G
G
Mattia
C
C
Pavone
P
P
Quattrocchi
S
S
D002648:Child; D002675:Child, Preschool; D003627:Data Interpretation, Statistical; D003971:Diastole; D015150:Echocardiography, Doppler; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D006801:Humans; D007223:Infant; D007558:Italy; D008297:Male; D009136:Muscular Dystrophies / Q000150:complications* / Q000151:congenital*; D015995:Prevalence; D013318:Stroke Volume; D018615:Ultrasonography, Doppler, Color