Metabolic Cardiomyopathy in Paediatric Practice

Aim. To review Russian and foreign literature of the recent years pertaining to the modern methods of diagnosis and management of children and adolescents suffering from cardiomyopathies.

Materials and methods. Russian and foreign literature was analysed using the following databases: RSCI, Scopus, Web of Science, Pubmed.

Results. There has been an increase in the proportion of non-specifi c myocardial lesions of non-infl ammatory origin in the structure of cardiovascular diseases in children and adolescents. Cardiomyopathies (CMP) constitute a group of myocardial diseases, which have been defi ned relatively recently and are increasingly attracting much research attention of cardiologists, morphologists, geneticists and cardiac surgeons. CMPs in children constitute a severe pathology and are characterised by a progressive course, resistance to therapy, as well as by an unfavourable prognosis. In this connection, a fundamentally new concept of CMP diagnosis, as well as its role in the structure of cardiovascular diseases, has been introduced, which is associated with the achievements in the spheres of medical genetics, immunology and molecular endocrinology. The article presents data from the Russian and foreign publications on paediatric aspects of metabolic CMP. In ths paper, we consider clinical, paraclinic and instrumental criteria for the diagnostics of non-coronary myocardial pathologies and associated pharmacotherapy, as well as some terminological issues.

Conclusion. The problem of effi cient CMP diagnostics remains to be solved; its complexity is explained by the absence of specifi c clinical disease markers and a long asymptomatic course. CMPs occupy a special place among myocardial diseases of various origins, thus requiring a common diagnostic algorithm, as well as a complex of preventive, therapeutic and rehabilitation measures to be used in the paediatric practice.