engleski

Childhood cardiomyopathies - retrospective study (1988-1999)

Cardiomyopathies are a heterogeneous group defined as a disease of the myocardium associated with cardiac dysfuntion A retrospective study was carried out at the Department of Paediatric Cardiology in University Hospital Centre Rebro, Zagreb, between 1988-1999 to obtain information on the epidemiology of childhood cardiomyopathies (CMP). The patients were categorized mainly according to the guidelines of the WHO/ISFC. A 135 infants, children and adolescents were identified as having cardiomyopathy. During these follow-up study 10 pts died. The average occurrence of all CMP regarding to other cardiac diseases (31167 pts) was 0.21%. Fifty-five patients were female (40%) and 80 were male (60%). Dilated cardiomyopathy (DCM) was diagnosed in 52 pts (38, 5%), 43 pts (31.9%) had hypertrophic cardiomyopathy (HCM) and 6 pts (4.4%) was identifed as a restrictive cardiomyopathy (RCM). There were no patients in our study with arrhythmogenic right ventricular dysplasia. In nine patients (6, 6%) we couldn’t classified CMPs. They were in a group of unclassified CMPs. Eleven patients (8, 2%) were in a group of specific cardiomyopathies and 14 patients (10, 4%) were diagnosed like myocarditis. In seven pts analysis of biopsied material was performed with light microscopy according to Dallas criteria, following with immunohystology and immunohystochemistry and electron microscopy. There were 12 (9%) patients who besides cardiomypathies also suffer from neuromuscular disorders. Most common cardiomyopathies, being a feature of myogenic and neurogenic muscle disorders are dilated, appearing in muscular dystrophies and neurogenic muscular atrophies. Mitochondrial disorders are linked to HCM. According to age distribution DCM was in most cases diagnosed in infants, and HCM in age from 10 to 15 yrs. In recent years 7-8 new cases of cardiomypathy were diagnosed in our department each year. The great number of CMP were diagnosed between 1990-1992, but mostly diagnosed pts belong older age group (10-15 yrs) and we assumed that in these cases CMP had developed earlier., while today owing increasing use and accurancy echocardiographic techniques and by higer index of suspicion among clinicals, the median age for diagnosis is 2 year. Today many clinical and molecular genetic studies show that genetic alterations have a major role in the pathogenesis of CMP. In this study we especially selected HCM. Now we sistematically make genetic analysis in Ruđer Bošković Institute, Division of Molecular Medicine, 24-hour ambulatory Holter moonitoring, scintigraphy of myocardium, cardiac catheterization and electrophysilogic protocol of each patient whose having asymmetric form HCM, and with this five steps we try to prevent the main risk of HCM- sudden death.

cardiomyopathy; epidemiology; children

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