Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya3375110.26442/00403660.2019.12.000142Editorial articleПередовая статьяReview ArticleLeft ventricular non - compaction: contemporary view of genetic background, clinical course, diagnostic and treatmentНекомпактный миокард: современные представления о генетических основах, клинической картине, диагностике и леченииStreltsovaA AСтрельцоваА АGudkovaA YaГудковаА ЯKostarevaA AКостареваА АAlmazov Federal Medical Research CentreФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Минздрава РоссииAlmazov Federal Medical Research CentreФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Минздрава РоссииPavlov First St. Petersburg State Medical UniversityФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Минздрава России151220199112VOL 91, NO12 (2019)ТОМ 91, №12 (2019)909716042020Copyright ©; 2019, Consilium MedicumCopyright ©; 2019, ООО "Консилиум Медикум"2019Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/33751

This review highlights and discusses recent advances in understanding left ventricular non - compaction (LVNC). Clinical profile, prognosis and even diagnosis are still a great challenge faced by the world. The population prevalence of left ventricular non - compaction remains unknown. High variability of clinical manifestations, genetic heterogenity with overlap of different phenotypes, variability of hereditary patterns suggests that LVNC seems to be rather an isolated trait or a part of phenotypic expression of different cardiac diseases or complex genetic syndromes.

Некомпактный миокард: современные представления о генетических основах, клинической картине, диагностике и лечении Представлен обзор литературы по проблеме некомпактного миокарда. Особенности клинического течения заболевания, прогноз и даже определение некомпактного миокарда левого желудочка (ЛЖ) до сих пор являются предметом дискуссий. Истинная распространенность некомпактного миокарда ЛЖ на сегодняшний день остается неизвестной. Малая специфичность клинических проявлений, генетическая гетерогенность с наслаиванием друг на друга различных фенотипов, вариабельность наследственных паттернов - все это ставит под сомнение гипотезу о том, что некомпактный миокард ЛЖ представляет собой отдельный вид кардиомиопатии. Существует мнение, что эта патология служит фенотипическим, морфологическим отражением широкого спектра генетических заболеваний миокарда.

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