medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/2079-701X-2022-16-17-130-135medsovet-7140Research ArticleПРАКТИКАPRACTICEКлинический случай некомпактного миокарда левого желудочка в сочетании с рестриктивной кардиомиопатиейThe clinical case of non-compact left ventricular myocardium in combination with restrictive cardiomyopathyhttps://orcid.org/0000-0002-8965-7628БарановС. А.BaranovS. A.

к.м.н., доцент кафедры пропедевтики внутренних болезней, 

119991, Москва, ул. Трубецкая, д. 8, стр. 2

Cand. Sci. (Med.), Associate Professor Department of Internal Medicine Propaedeutics, 

 8, Bldg. 2, Trubetskaya St., Moscow, 119991

BaranovSA1975@yandex.ruhttps://orcid.org/0000-0003-0881-9064НечаевВ. М.NechaevV. M.

к.м.н., доцент кафедры пропедевтики внутренних болезней, 

119991, Москва, ул. Трубецкая, д. 8, стр. 2

Cand. Sci. (Med.), Associate Professor Department of Internal Medicine Propaedeutics, 

 8, Bldg. 2, Trubetskaya St., Moscow, 119991

brunhild1958@mail.ruhttps://orcid.org/0000-0002-5467-2625ФадееваМ. В.FadeevaM. V.

врач отделения кардиологии Клиники пропедевтики внутренних болезней, гастроэнтерологии и гепатологии имени В.Х. Василенко,

119435, Москва, ул. Погодинская, д. 1

Physician of the Cardiology Department, Clinic of Internal Diseases Propaedeutics, Gastroenterology and Hepatology named after V.Kh.Vasilenko,

1, Pogodinskaya St., Moscow, 119435

fadeeva_m_v@staff.sechenov.ruПервый Московский государственный медицинский университет имени И.М. Сеченова (Сеченовский Университет)РоссияSechenov First Moscow State Medical University (Sechenov University)Russian Federation202216102022017130135Copyright © Баранов С.А., Нечаев В.М., Фадеева М.В., 20222022Баранов С.А., Нечаев В.М., Фадеева М.В.Baranov S.A., Nechaev V.M., Fadeeva M.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-sovet.pro/jour/article/view/7140

В статье представлена информация о сравнительно редкой форме кардиомиопатии – некомпактном миокарде левого желудочка. В результате генетических изменений на ранних стадиях эмбриогенеза происходит нарушение уплотнения миокардиальных волокон и формируется двуслойная структура миокарда: тонкий компактный слой и сохраняющийся более объемный некомпактный слой с  выраженной трабекуляцией и  глубокими межтрабекулярными полостями, сообщающимися с полостью желудочка. Одной из основных причин некомпактного миокарда считается наличие мутации в генах, кодирующих саркомерные, структурные и  регуляторные белки, а  также белки, ответственные за  функционирование ионных каналов. Существует теория, которая рассматривает некомпактный миокард и  как следствие воздействия на  организм в  течение жизни различных факторов, т.  н. неэмбриональный  (приобретенный) некомпактный миокард. Неэмбриональная гипотеза рассматривает некомпактный миокард как проявление функциональной дезадаптации, возможный этап в развитии кардиомиопатии. В  качестве иллюстрации в  статье представлен клинический случай пациентки 32  лет, у  которой впервые был диагностирован некомпактный миокард левого желудочка в сочетании с рестриктивной кардиомиопатией. Диагноз подтвержден основными методами диагностики этой патологии – ЭхоКГ и МРТ сердца. Данное клиническое наблюдение интересно тем, что картина заболевания определяется двумя редко сочетающимися патологическими состояниями: синдромом некомпактного миокарда и  рестриктивной кардиомиопатией. Изменения сердечной гемодинамики происходят при их взаимодействии. Вследствие рестриктивной кардиомиопатии снижается диастолическое наполнение левого желудочка, что уменьшает нагрузку на  его миокард, поэтому систолическая дисфункция, характерная для некомпактного миокарда, не возникает. В целом прогноз, учитывая наличие двух серьезных патологий, у данной больной неблагоприятный. 

In this paper the information about a comparatively rare form of cardiomyopathy – noncompaction of the left ventricular myocardium is showed. As a result of genetical changes on the early stages of embryogenesis a disability of myocardial fibres develops, and two-layer myocardial structure is formed: thin compact layer and remaining more voluminous non-compact layer with significant trabeculation and deep intratrabecular cavities communicated with ventricular cavity. Mutations in  genes which encode sarcomeric, structural and regulatory proteins and proteins, which are responsible for ion channels functioning, are considered to be one of the main reasons of non-compact myocardium. There is a theory that considers a non-compact myocardium as a result of an exposure of various factors during lifetime – so-called non-embryonal (acquired) noncompaction myocardium. “Non-embryonal” hypothesis views non-compact myocardium as a sign of functional maladaptation, possible stage in cardiomyopathy development. By way of illustration the clinical case of 32-year-old female patient is presented in the article. The noncompaction of  the left ventricular myocardium in  conjunction with restrictive cardiomyopathy was first diagnosed in  her. The  diagnosis was confirmed by main diagnostic methods for  this pathology such as echocardiography and MRI of  a  heart. This clinical observation is interesting due to formation of disease patterns by two rare combined pathological conditions: noncompaction of the myocardium syndrome and restrictive cardiomyopathy. Changes of heart hemodynamics occurs in interaction between these pathologies. Diastolic filling of left ventricular decreases as a result of restrictive cardiomyopathy, which leads to decreasing of its myocardium load. Therefore, systolic disfunction, which is specific to non-compact myocardium, doesn’t occur. Generally, prognosis for this patient is poor due to presence of two serious pathologies. 

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