cardioКардиологияKardiologiia0022-90402412-5660Kardiomag10.18087/cardio.2024.6.n2502cardio-2502Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИRESEARCH ARTICLESУгрожающие жизни осложнения синдрома некомпактного миокарда левого желудочка у взрослых: стратификация риска и подходы к лечениюAdverse Events in Patients With Left Ventricular Noncompaction: Risk Stratification and Treatment Optionshttps://orcid.org/0000-0002-8868-0623ВариончикН. В.VarionchikN. V.

кафедра факультетской терапии №1, старший лаборант

Москва, Россия

post-graduate student of the Department of Faculty Therapy No. 1 of  Sechenov First Moscow State Medical University (Sechenov University)

Moscow, Russia

vanadya@gmail.comhttps://orcid.org/0000-0002-5253-793XБлаговаО. В.BlagovaO. V.

кафедра факультетской терапии №1, профессор, доктор медицинских наук

Москва, Россия

Doctor of Sciences in Medicine, Professor  at the Department of Faculty Therapy № 1 of  Sechenov First Moscow State Medical University (Sechenov University)

Moscow, Russia

blagovao@mail.ruhttps://orcid.org/0000-0002-4510-7763ПавленкоЕ. В.PavlenkoE. V.

кафедра факультетской терапии №1, ассистент, кандидат медицинских наук

Москва, Россия

Candidate of Sciences in Medicine, Assistant Professor at the Department of Faculty Therapy № 1 of  Sechenov First Moscow State Medical University (Sechenov University)

Moscow, Russia

evd88@mail.ruhttps://orcid.org/0000-0001-9587-6707НедоступА. В.NedostupA. V.

доктор медицинских наук, научный сотрудник НИО кардиологии

Москва, Россия

Doctor of Sciences in Medicine, Professor, Researcher at the Science and Technology Park of Biomedicine of Sechenov First Moscow State Medical University (Sechenov University)

Moscow, Russia

evd88@mail.ruhttps://orcid.org/0000-0003-2326-9347СедовВ. П.SedovV. P.

доктор медицинских наук, профессор кафедры лучевой диагностики 

Москва, Россия

Doctor of Sciences in Medicine, professor of the Department of Cardiology, Functional and Ultrasound Diagnostics of Sechenov First Moscow State Medical University (Sechenov University)

Moscow, Russia

vps52@mail.ruhttps://orcid.org/0000-0003-4575-6058ГагаринаН. В.GagarinaN. V.

врач-рентгенолог отделения лучевой диагностики, кандидат медицинских наук

Москва, Россия

Candidate of Sciences in Medicine, radiologist, Department of Radiation Diagnostics, University Clinical Hospital No. 1 of Sechenov First Moscow State Medical University (Sechenov University)

Moscow, Russia

gagarina_n_v@staff.sechenov.ruhttps://orcid.org/0000-0002-1266-4926МершинаЕ. А.MershinaE. A.

Москва, Россия

Candidate of Sciences in Medicine, radiologist, Head of the Department of X-ray Diagnostics, CT and MRI of the Medical Research and Education Center of Lomonosov Moscow State University 

Moscow, Russia

elena_mershina@mail.ruhttps://orcid.org/0000-0002-5649-2193СиницынВ. Е.SinitsynV. E.

доктор медицинских наук, профессор, заведующий кафедрой лучевой диагностики и терапии факультета фундаментальной медицины МГУ имени М.В. Ломоносова

Москва, Россия

Doctor of Sciences in Medicine, radiologist, Professor, Head of the Radiology Department and Chair of Radiology at the University Hospital of Moscow Lomonosov State University 

Moscow, Russia

elena_mershina@mail.ruhttps://orcid.org/0000-0003-4923-1945ПолякМ. Е.PolyakM. E.

кандидат медицинских наук, врач- генетик Российского научного центра хирургии им. акад. Б. В. Петровского

Москва, Россия

Candidate of Sciences in Medicine, geneticist, Petrovsky National Research Center of Surgery

Moscow, Russia

margaritapolyak@gmail.comhttps://orcid.org/0000-0002-6244-9546ЗаклязьминскаяЕ. В.ZaklyazminskayaE. V.

доктор медицинских наук, профессор, заведующий лабораторией медицинской генетики

Москва, Россия

Doctor of Sciences in Medicine, Head of the Laboratory (Medical Genetics Laboratory) Petrovsky National Research Center of Surgery 

Moscow, Russia

helenezak@gmail.comФГАОУ ВО «Первый МГМУ имени И. М. Сеченова» Минздрава РФ (Сеченовский Университет)РоссияSechenov First Moscow State Medical UniversityRussian FederationМедицинский научно-образовательный центр МГУ имени М. В. ЛомоносоваРоссияMedical Research and Educational Center, Lomonosov Moscow State UniversityRussian FederationГосударственный научный центр Российской Федерации ФГБНУ «Российский научный центр хирургии им. академика Б. В. Петровского»РоссияState Medical Center of the Russian Federation, Petrovsky National Scientific Center of SurgeryRussian Federation202430062024646311Copyright © Kardiomag, 20242024KardiomagKardiomaghttps://cardio.elpub.ru/jour/about/submissions#copyrightNoticehttps://cardio.elpub.ru/jour/article/view/2502Цель

Цель. Изучить клинические проявления, частоту угрожающих жизни осложнений некомпактного миокарда левого желудочка (НКМ ЛЖ) у взрослых, их возможные механизмы и исходы.Материал и методы. В исследование включено 125 взрослых пациентов с НКМ ЛЖ, 74 мужчины (59,2 %), 51 женщина (40,8 %). Средний возраст 46,4±15,1 лет. Выполнялись эхокардиография (ЭхоКГ) (n=125), Холтеровское мониторирование (n=125), магнитно-резонансная томография (МРТ) сердца (n=60), мультиспиральная компьютерная томография (МСКТ) сердца с контрастированием (n=90); по показаниям – коронароангиография (КАГ) (n=33), сцинтиграфия миокарда (n=27). Диагноз НКМ в 74 случаях подтвержден при помощи двух методов, в 21 случае – трех визуализирующих методов. Большинству больных выполнена ДНК-диагностика. Большинству пациентов определялись уровень антикардиальных антител и геном кардиотропных вирусов в крови. Средняя фракция выброса (ФВ) левого желудочка (ЛЖ) 38,6±14,0 %; конечно-диастолический объем (КДО) ЛЖ 158,1±67,8 мл; конечно-диастолический размер (КДР) ЛЖ 6,1±0,9 см; объем левого предсердия (ЛП) 97,1±38,1 мл. Средний срок наблюдения составил 14 месяцев [4,0; 41,0]; от 1 месяца до 10 лет.Результаты. Летальность составила 14,4 %, трансплантация сердца выполнена в 5,6 % случаев. Неустойчивая желудочковая тахикардия выявлена в 45,6 % случаев, устойчивая ЖТ – в 13,6 %. Наличие ЖТ ассоциировалось с недостаточным нарастанием зубца R в грудных отведениях ЭКГ; низким вольтажом QRS, продолжительностью QRS >105 мс, функциональным классом хронической сердечной недостаточности (ФК ХСН) ≥2–3 по NYHA, ФВЛЖ <40 %; КДР ЛЖ >6,1 см, наличием миокардита, большей летальностью. Кардиовертеры-дефибрилляторы, в том числе ресинхронизирующие (cardiac resynchronization therapy defibrillator, CRTD), имплантированы 38 пациентам. Оправданные шоки дефибрилляторов ассоциировались с частой желудочковой экстрасистолией (ЖЭ). Частота тромбозов и эмболий составила 22,4 %. Предикторами их развития были: ФК ХСН ≥2–3, переднезадний размер ПЖ >3,1 см, ЛП >98 мл, Е / А >1,65, КДР ЛЖ >6,3 см, КДО ЛЖ >153 мл, ФВЛЖ <35 %, некроз миокарда неустановленного генеза (у больных без атеросклероза коронарных артерий). Частота некроза миокарда при НКМ составила 16,0 %. В качестве механизмов выделены, помимо коронарного атеросклероза, эмболии в неизмененные коронарные артерии, присоединение миокардита, наличие генетически обусловленной тромбофилии.Заключение. НКМ ЛЖ ассоциируется с высоким риском угрожающих жизни состояний: желудочковые аритмии, тромбозы и эмболии, некроз миокарда являются типичным осложнением НКМ у взрослых. Необходима переоценка предикторов риска тромбоэмболических и аритмических событий, уточнение показаний к имплантации кардиовертера-дефибриллятора и назначению антикоагулянтов, активное выявление и лечение сопутствующего миокардита.

 

 

Aim. To study the clinical manifestations, incidence of life-threatening complications, and their possible mechanisms and outcomes of left ventricular non-compaction (LVNC) in adults.Material and methods. This study included 125 adult patients with LVNC, 74 men (59.2%) and 51 women (40.8%) aged 46.4±15.1 years. Echocardiography (EchoCG) (n=125), Holter monitoring (n=125), cardiac magnetic resonance imaging (MRI) (n=60), and contrast-enhanced multislice computed tomography (MSCT) of the heart (n=90), and, if indicated, coronary angiography (CAG) (n=33) and myocardial scintigraphy (n=27) were performed. The diagnosis of LVNC was confirmed in 74 cases using two methods, and in 21 cases, using three imaging methods. DNA diagnostics was performed in most patients. For most patients, the level of anticardiac antibodies and the genome of cardiotropic viruses were determined in the blood. Mean left ventricular (LV) ejection fraction (EF) was 38.6±14.0%; LV end-diastolic volume (EDV) was 158.1±67.8 ml; LV end-diastolic dimension (EDD) was 6.1±0.9 cm; and left atrial (LA) volume was 97.1±38.1 ml. The mean follow-up period was 14 months [4.0; 41.0]; from 1 month to 10 years.Results. Death rate was 14.4%; heart transplantation was performed in 5.6% of cases. Nonsustained ventricular tachycardia (VT) was detected in 45.6% of cases and sustained VT in 13.6%. The presence of VT was associated with poor R-wave progression in the precordial ECG leads, low QRS voltage, QRS duration >105 ms, NYHA chronic heart failure functional class (CHF FC) ≥2-3, LV EF <40%; LV EDD >6.1 cm, the presence of myocarditis, and higher death rate. Cardioverter defibrillators, including cardiac resynchronization therapy defibrillators (CRTD), were implanted in 38 patients. Appropriate defibrillator shocks were associated with frequent premature ventricular contractions (PVCs). The incidence of thrombosis and embolism was 22.4%. Their predictors included CHF FC ≥2-3, RV anteroposterior dimension >3.1 cm, LA volume >98 ml, E/A >1.65, LV EDD >6.3 cm, LV EDV >153 ml, LV EF <35%, and myocardial necrosis of unknown origin (in patients without coronary atherosclerosis). The incidence of myocardial necrosis in LVNC was 16.0%. The mechanisms identified, in addition to coronary atherosclerosis, were embolism in unchanged coronary arteries, secondary myocarditis, and the presence of genetically determined thrombophilia.Conclusion. LVNC is associated with a high risk of life-threatening conditions, such as ventricular arrhythmias, thrombosis and embolism, and myocardial necrosis, that are typical complications of LVNC in adults. Reassessing the predictors for the risk of thromboembolic and arrhythmic events, specifying the indications for implantable cardioverter defibrillator and anticoagulants, and actively identifying and treating concomitant myocarditis are essential.

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