title: Assessment of left ventricular systolic and diastolic functions in children with merosin-positive congenital muscular dystrophy.
abstract: Cardiopathy is an expected finding in X-linked Duchenne and Becker muscular dystrophies. This holds true for some other forms such as autosomal recessive limb-girdle dystrophies. However, data on early-onset and usually severe congenital muscular dystrophies are limited. The purpose of this study was to investigate the presence of cardiac involvement in children with merosin-positive congenital muscular dystrophy. A total of 42 patients and 22 healthy subjects were evaluated by M-mode, 2D, and Doppler echocardiography. Cardiac anatomy, left ventricular dimensions, wall thickness and systolic and diastolic functions were investigated in patients and compared with those of healthy control subjects. Mean left ventricular ejection fraction and shortening fraction were significantly lower in the patient group (P<0.05 and P<0.001, respectively) and in three patients ejection fraction was below 55%. Although some impairments in left ventricular inflow indexes which were suggestive of left ventricular diastolic dysfunction were detected in patients with merosin-positive congenital muscular dystrophy they were not statistically significant. Our results suggest that left ventricular systolic abnormalities may occur in children with merosin-positive congenital muscular dystrophy.
authors.affiliation: Cardiology Unit, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey. nceviz@atauni.edu.tr
authors.forename: Naci
authors.initials: N
authors.lastname: Ceviz
authors.forename: Füsun
authors.initials: F
authors.lastname: Alehan
authors.forename: Dursun
authors.initials: D
authors.lastname: Alehan
authors.forename: Sencan
authors.initials: S
authors.lastname: Ozme
authors.forename: Zuhal
authors.initials: Z
authors.lastname: Akçören
authors.forename: Gülsev
authors.initials: G
authors.lastname: Kale
authors.forename: Haluk
authors.initials: H
authors.lastname: Topaloglu
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003971:Diastole / Q000502:physiology; D004452:Echocardiography; D005260:Female; D006334:Heart Function Tests; D006801:Humans; D007223:Infant; D007797:Laminin; D008279:Magnetic Resonance Imaging; D008297:Male; D009136:Muscular Dystrophies / Q000151:congenital* / Q000175:diagnosis*; D011336:Probability; D011379:Prognosis; D012016:Reference Values; D018570:Risk Assessment; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D013599:Systole / Q000502:physiology; D014057:Tomography, X-Ray Computed; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis*; D016277:Ventricular Function, Left / Q000502:physiology*
chemical_list: D007797:Laminin