Cardiomyopathies in children – inherited heart muscle disease: Overview of hypertrophic, dilated, restrictive and non-compaction phenotypes

摘要: Abstract Pediatric cardiomyopathies of genetic origin directly involving the heart muscle – i.e. not related to metabolic causes - are rare, yet serious diseases with an annual incidence 1.1 1.5 per 100,000 children. Cardiomyopathies a prevalent cause failure and most common transplantation in children older than 1 year age. Dilated hypertrophic cardiomyopathy (HCM) have largest prevalence, whereas restrictive left ventricular non-compaction very rare. The epidemiology, natural history prognostic indicators pediatric were poorly understood before 1990s, many gaps knowledge remain this day. Advances cardiac imaging genomic characterization, including genome-wide analysis, providing fresh insights into etiology outcome cardiomyopathy. While morphological clinical manifestations similar those adult patients, tend more severe outcomes may respond less well pharmacological treatment. present review aims examine familial children, focusing particularly on risk predictors HCM, probably field impressive advances recent years.