engleski

Epidemiology of cardiomyopathies in children and adolescents - retrospective study in the last ten years.

Purpose: 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). Patients and methodes: The patients were categorized mainly according to the guidelines of the World Health Organization/International Society and Federation of Cardiology Task Force on Cardiomyopathies (WHO/ISFC) All patients aged birth to 20 years diagnosed in 1988-1998 as having cardiomyopathy according strict inclusion and exclusion criteria for CMP. The evaluation of patients had included thorough medical history, physical examination, standard blood chemical analysis, hematologic measurements, protein electrophoresis, ECG, X-ray, ECHO, heart catheterization and endomyocardial biopsy. In physical examination we followed subjective difficulties according New York Heart Association (NYHA) guidelines, congestive lung disease, positive venous puls, apical and parasternal cardiac impulses, systolic murmur, heart frequention, hepatomegaly, splenomegaly and sympthoms of protein losing enteropathy. Initial haemodinamic diagnosis was based on echocardiographic findings. Results: A 135 infants, children and adolescents were identified as having cardiomyopathy. The average occurrence of all CMP regarding to other cardiac diseases (31167 pts) was 0.21%. Fifty-three patients were female (40%) and 80 were male (60%). Dilated cardiomyopathy (DCM) was diagnosed in 52 pts (38, 5%), 43pts (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 (4 had metabolic, 1 ischemic, 2 hypertensive, 3 valvular form and 1 had toxic reactions) and 14 patients (10, 4%) were diagnosed like myocarditis. In a group of HCM 15 pts (35%) had asymmetric and 28 pts (65%) had concentric form. In a group of asymmetric HCM in 8 patients dominant inheritance was strongly suggested on the base of family history. Among these patients we found brother and sister in whom a gradual passing from RCM to HCM had been seen. They were finally classified in a group of HCM. During these follow-up study 10 patients died (4 pts with DCM, 4 pts with HCM, 1 pts with RCM and 1 with specific CMP). According to age distribution DCM was in most cases diagnosed in infants, and HCM in age from 10 to 15 yrs. There were 12 (9%) patients who besides cardiomypathies also suffer from neuromuscular disorders. Most common cardiomyopathies, being a feture of myogenic and neurogenic muscle disorders are dilated, appearing in muscular dystrophies and neurogenic muscular atrophies. Mitochondrial disorders are linked to HCM. Conclusion: In recent years 7-8 new cases of cardiomypathy were diagnosed in our department each year. The great number of cardiomyopathies 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 1 year. Today the cardiomyopathies represent the great field of interest in cardiology. Many clinical and molecular genetic studies show that genetic alterations have a major role in the pathogenesis of cardiomyopathies. Unfortunaltely, the significant advances in the cause and disease mechanism have not been matched in the therapeutic. In this study we especially selected HCM. Cardiomyopathies, and more specifically hypertrophic cardiomyopathy, have opened the route what is now called genetic cardiology. Now we sistematically make genetic analysis, 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.

Cardiomyopathies; Childhood; Epidemiology

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