Noncompaction cardiomyopathy (NCMP) is characterized by the anomalous myocardium structure and various types of cardiac remodeling, in some cases it is accompanied by thrombotic complications. Preconditions for thrombosis in the disease are unknown, as also there are differences in thrombosis rates between NCMP and other cardiomyopathies, similarly accompanied by the chronic heart failure and analogous remodeling phenotypes. Aim of study is to reveal the difference in the rate of thrombosis in NCMP and dilated cardiomyopathies (DCMP) in children, and to define differences in the frequency of different genotypes of polymorphic markers in an array of hemostasis genes in the two cardiomyopathies. Methods. There was executed a prospective-retrospective cohort study, included patients from the Cardiac Department of the National Scientific and Practical Center of Children's Health from October 2011 to May 2015. The presence of NCMP was established by echocardiography, alleles and genotypes of polymorphic markers of hemostasis and folate cycle genes were determined by polymerase chain reaction in real-time mode. Results. Thrombotic complications in NCMP children were observed more often than in DCMP cases. There were no differences between NCMP and DCMC patients in the frequency of the polymorphic markers c.1691G>A of the F5 gene (p=0.61) , c.20210G>A of the F2 gene (p=1.0) , c.1565T> C of the ITGB3 gene (p=0.32) , 5G(-675)4G of PLANH1 gene (p=0,52) , G(-455)A of FGB gene (p=0.82) , c.677C>T of MTHFR gene (p=0.11). Conclusion Thrombotic complications in NCMP children occur rather more often than in DCMP cases, studied polymorphic markers of the hemostasis and folate cycle genes do not cause this difference, and this requires continuation of the study.

Некомпактный миокард является редкой врожденной кардиомиопатией. В настоящее время диагностика данной патологии затруднена в связи с отсутствием единых критериев, что приводит к частой гипердиагностике. Натрийуретического гормона N-концевой полипептид (NT-proBNP) является маркером хронической сердечной недостаточности у детей. В статье представлены собственные результаты изучения роли NT-proBNP в диагностике некомпактного миокарда. Выявлено, что содержание NT-proBNP в сыворотке крови детей с некомпактным миокардом выше, чем у детей с хронической сердечной недостаточностью без данной патологии (р < 0,01). Полученные результаты могут быть полезны врачу в клинической практике.

Left ventricular noncompaction (LVNC) is a disease of myocardium presumably caused by developmental arrest during embryogenesis. LVNC is characterized by the presence of pathological trabeculae in left ventricle. LVNC can be associated with different congenital heart diseases. Different types of myocardium remodeling are follows: dilated, hypertrophic, restrictive and mixed phenotypes. It is unclear whether LVNC is a distinct disease or a morphological feature of different cardiomyopathies. Distinction between pathological trabeculae constituting LVNC and normal phenotype is often difficult. For the time present the considerable attention is paid to the study of disease. Despite the progress in the study of the disease significant controversy remains around understanding of etiology, pathogenesis, clinical characteristics and prognosis of LVNC. Current literature review is aimed to discuss mentioned aspects with a focus on pediatric population.