perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2016-61-3-22-30perinatology-321Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWSМитохондриальные кардиомиопатииMitochondrial cardiomyopathiesЛеонтьеваИ. В.LeontyevaI. V.

д.м.н., проф., гл. н. сотр. отдела детской кардиологии и аритмологии,

125412 Москва, ул. Талдомская, д.2

Moscow

НиколаеваЕ. А.NikolaevaE. A.

д.м.н., и.о. рук. отдела психоневрологии и наследственных заболеваний,

125412 Москва, ул. Талдомская, д.2

Moscow

ОСП «Научно-исследовательский клинический институт педиатрии им. Ю.Е. Вельтищева» ГБОУ ВПО РНИМУ им. Н.И. ПироговаРоссияAcademician Yu.E. Veltishchev Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical UniversityRussian Federation2016010720166132230Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20162016Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/321

Рассмотрена проблема диагностики гетерогенных форм митохондриальных кардиомиопатий, связанных с нарушением окислительного фосфорилирования и снижением активности митохондриальной электронно-транспортной системы вследствие мутации генов митохондриальной или ядерной ДНК. Подчеркнуто, что митохондриальные кардиомиопатии редко представляют изолированное поражение миокарда, чаще служат проявлением мультисистемного заболевания. Дана характеристика кардиомиопатии при отдельных митохондриальных синдромах, отражены возможности лечения патологии.

The paper considers the problem of diagnosing the heterogeneous forms of mitochondrial cardiomyopathies associated with impaired oxidative phosphorylation and decreased activity of the mitochondrial electron transport system due to mutations in mitochondrial or nuclear DNA genes. It is emphasized that mitochondrial cardiomyopathies rarely present as an isolated myocardial lesion and more frequently serve as a manifestation of multisystem disease. The paper provides the characteristics of cardiomyopathies in some mitochondrial syndromes and shows the possibilities of treating this disease.

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The authors declare that there are no conflicts of interest present.