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”10.21508/1027-4065-2016-61-1-64-70perinatology-22Research ArticleКАРДИОЛОГИЯCARDIOLOGYПоражение сердца при синдроме БартаHeart injury in Barth syndromeЛеонтьеваИ. В.LeontyevaI. V.

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

НиколаеваЕ. А.NikolaevaE. A.

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

КалачановаЕ. П.KalachanovaE. P.

к.м.н., врач отделения кардиоревматологии

ОСП Научно-исследовательский клинический институт педиатрии им. акад. Ю.Е. Вельтищева ГБОУ ВПО РНИМУ им. Н.И. Пирогова Минздрава России, МоскваРоссияAcademician Yu.E. Veltishchev Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia, MoscowRussian Federation2016010320166116470Copyright © 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/22

Приведенное клиническое наблюдение ребенка с синдромом Барта демонстрирует тяжелое поражение сердца – сочетание дилатационной кардиомиопатии, синдрома некомпактного миокарда, признаков фиброэластоза эндокарда. Заболевание манифестировало с первых дней жизни, однако длительно протекало под маской врожденного миокардита. Нарушений сердечного ритма, электрической нестабильности миокарда, удлинения интервала Q–T в данном наблюдении не выявлено. Течение заболевания нестабильное с частыми периодами декомпенсации на фоне интеркуррентных заболеваний. Представлен современный взгляд на проблему поражения сердца при синдроме Барта, обсуждаются современные подходы к лечению, прогноз, показания к трансплантации сердца, возможности метаболической терапии.

The given clinical case report of a child with Barth syndrome demonstrates the severe heart injury – a concurrence of dilated cardiomyopathy, myocardial non-compaction, and signs of endocardial fibroelastosis. The disease was manifested in the first days of life; however, it was long masked by congenital myocarditis. No cardiac arrhythmias, myocardial electrical instability, or long Q–T syndrome were detected in this case. The disease was unstable with frequent episodes of decompensation in the presence of intercurrent diseases. The paper gives a modern view on the problem of cardiac injury in Barth syndrome and discusses current treatment approaches, prognosis, indications for heart transplantation, and possibilities of metabolic therapy.

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