title: Subclinical cardiological involvement in Sicilian patients with pure congenital muscular dystrophy.
abstract: 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.
authors.affiliation: Department of Pediatrics, University of Catania, Catania, Italy. raffaelefalsaperla@hotmail.com
authors.forename: R
authors.initials: R
authors.lastname: Falsaperla
authors.forename: G
authors.initials: G
authors.lastname: Romeo
authors.forename: C
authors.initials: C
authors.lastname: Mattia
authors.forename: P
authors.initials: P
authors.lastname: Pavone
authors.forename: S
authors.initials: S
authors.lastname: Quattrocchi
mesh_terms: 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