evrazkarЕвразийский Кардиологический ЖурналEurasian heart journal2225-16852305-0748Евразийская ассоциация кардиологов10.38109/2225-1685-2022-2-110-117evrazkar-6328Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИORIGINAL PAPERSНекомпактная кардиомиопатия: клинические формы, исходы и выживаемость в когорте белорусских пациентовNon-compaction cardiomyopathy: clinical forms, outcomes and survival in a cohort of belarusian patientshttps://orcid.org/0000-0001-9917-5932КомиссароваС. М.KomissarovaS. M.

Комиссарова Светлана Михайловна, ведущий научный сотрудник лаборатории хронической сердечной недостаточности, доктор мед. наук, доцент

ул. Розы Люксембург, д.110 Б, г. Минск 220036

Svetlana M. Komissarova, Dr. of Sci. (Med.), associate professor, leading researcher, the laboratory of chronic heart failure

R. Luxemburg 110, Minsk 220036

kom_svet@mail.ruhttps://orcid.org/0000-0002-4150-282XКраськоО. В.KraskoO. V.

Красько Ольга Владимировна, ведущий научный сотрудник лаборатории биоинформатики, кандидат математических наук, доцент

ул. Сурганова, д. 6, г. Минск 220012

Olga V. Krasko, Cand of Sci. (Mathematical), leading researcher, the State scientific institution “United Institute of Informatics Problems, candidate of mathematical sciences, associate professor

Surganova 6, Minsk 220012

olga.krasko.ok@gmail.comhttps://orcid.org/0000-0002-1986-1367РинейскаяН. М.RinejskayaN. M.

Ринейская Надежда Михайловна, младший научный сотрудник лаборатории хронической сердечной недостаточности

ул. Розы Люксембург, д.110 Б, г. Минск 220036

Nadezhda M. Rinejskaya, junior researcher, the laboratory of chronic heart failure

R. Luxemburg 110, Minsk 220036

nadya.rin@gmail.comhttps://orcid.org/0000-0003-3634-0616СеврукТ. В.SevrukT. V.

Севрук Татьяна Васильевна, заведующая отделением ультразвуковой диагностики

ул. Розы Люксембург, д.110 Б, г. Минск 220036

Tatyana V. Sevruk, head of the department of ultrasound diagnostics

R. Luxemburg 110, Minsk 220036

https://orcid.org/0000-0003-2424-6104ЕфимоваА. А.EfimovaA. A.

Ефимова Анастасия Александровна, врач лучевой диагностики рентгеновского отделения

ул. Розы Люксембург, д.110 Б, г. Минск 220036

Anastasiya A. Efimova, doctor of radiology diagnostics of the X-ray department

R. Luxemburg 110, Minsk 220036

anst.efimova@gmail.comГУ Республиканский научно-практический центр «Кардиология»БеларусьState Institution Republican Scientific and Practical Centre «Cardiology»BelarusГНУ «Объединенный институт проблем информатики НАН Беларуси»БеларусьState scientific institution “United Institute of Informatics Problems of the National Academy of Sciences of Belarus”Belarus20222805202202110117Copyright © Комиссарова С.М., Красько О.В., Ринейская Н.М., Севрук Т.В., Ефимова А.А., 20222022Комиссарова С.М., Красько О.В., Ринейская Н.М., Севрук Т.В., Ефимова А.А.Komissarova S.M., Krasko O.V., Rinejskaya N.M., Sevruk T.V., Efimova A.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.heartj.asia/jour/article/view/6328

Цель – оценить результаты длительного наблюдения за пациентами с различными клиническими формами НКМП, связанных с ними клиническими исходами, а также 3-летнюю выживаемость.Материал и методы. Обследовано 211 пациентов с НКМП (медиана возраста 39 [18; 72] лет), из них: у 94 (44,5 %) диагностирован изолированный фенотип и у 117 (55,5 %) – сочетание с другими кардиомиопатиями, в том числе у 103 (48,8 %) пациентов – сочетание с ДКМП; у 14 (6,6 %) – с ГКМП, которым помимо традиционных клинических методов исследования выполняли МРТ сердца с отсроченным контрастированием гадолинием. Конечные точки исследования включали ВСС, ВСС с успешной реанимацией и имплантацией КД и выполнение ОТС. Результаты. За период наблюдения (медиана наблюдения 36 [6-211] мес.) из 211 пациентов с НКМП у 24 (11,4 %) были зарегистрированы неблагоприятные исходы, в том числе у 1 (0,5 %) – ВСС, у 10 (4,7 %) – ВСС с успешной реанимацией и установкой ИКД, у 13 (6,2 %) выполнена ОТС. ВСС наступила у 11 (5,2 %) пациентов с ФК I-II NYHA, в том числе у 10 пациентов с успешной реанимацией и имплантацией ИКД через 23 (от 5 до 152) месяцев после установления диагноза. ОТС выполнена 13 пациентам с ФК III NYHA в сроки 30 (от 8 до 113) месяцев, после постановки диагноза в группе пациентов с сочетанием НКМП с ДКМП. Средний возраст на момент ВСС или ОТС составлял 37±12 лет. 3-летняя выживаемость в группе пациентов с изолированной формой составила 98.9 (96.7–100) %, и значимо различалась с группой пациентов с сочетанием НКМП с ДКМП 86.5 (79.6–94) %, и с группой пациентов с сочетанием НКМП с ГКМП 72.9 (50.7–100) %, p=0,0012. Тогда как 3-летняя выживаемость в группе с сочетанием НКМП с ДКМП и НКМП с ГКМП между собой значимо не различались.Заключение. Прогноз пациентов с изолированной формой НКМП в целом благоприятный и 3-летняя выживаемость их составила 98.9 (96.7–100) %. Наличие сочетания НКМП с другими кардиомиопатиями предсказывает риск неблагоприятных клинических исходов и 3-х летняя выживаемость сочетания НКМП с ДКМП составила 86.5 (79.6–94) %, а при сочетании НКМП с ГКМП 72.9 (50.7-100) %, что может быть полезным при выборе стратегии ведения пациентов с НКМП.

The aim is to evaluate the results of long-term follow-up of patients with various clinical forms of NCCM, associated clinical outcomes, as well as 3-year survival.Materials and methods. 211 patients with NCCM (median age 39 [18; 72] years) were examined, of which: 94 (44.5 %) were diagnosed with an isolated phenotype and 117 (55.5 %) with a combination with other cardiomyopathies, including 103 (48.8 %) patients with a combination with DCM; 14 (6.6 %) with HCM, who, in addition to traditional clinical research methods, underwent CMR imaging with late gadolinium enhancement. The endpoints of the study included SCD, SCD with successful resuscitation and implantation of ICD and the implementation of OHT.Results. During the follow–up period (median follow–up 36 [6-211] months), of 211 patients with NCCM, 24 (11.4 %) had adverse outcomes, including 1 (0.5 %) – SCD, 10 (4.7 %) – SCD with successful resuscitation and ICD implantation, 13 (6.2 %) had OHT. SCD occurred in 11 (5.2 %) patients with FC I-II NYHA, including 10 patients with successful ICD resuscitation and implantation after 23 (from 5 to 152) months after diagnosis. OHT was performed in 13 patients with FC III NYHA at 30 (from 8 to 113) months after diagnosis in a group of patients with a combination of NCCM and DCM. The average age at the time of SCD or OHT was 37±12 years.The 3-year survival rate in the group of patients with the isolated form was 98.9 (96.7-100) %, and significantly differed with the group of patients with a combination of NCCM with DCM 86.5 (79.6-94) %, and with the group of patients with a combination of NCCM with HCM 72.9 (50.7-100) %, p=0.0012. Whereas the 3-year survival rate in the group with a combination of NCCM with DCM and NCCM with HCM did not significantly differ from each other. Conclusion. The prognosis of patients with an isolated form of NCCM is generally favorable and their 3-year survival rate was 98.9 (96.7-100) %. The presence of a combination of NCCM with other cardiomyopathies predicts the risk of adverse clinical outcomes and the 3-year survival rate of a combination of NCCM with DCM was 86.5 (79.6-94) %, and when combined with NCCM with HCM 72.9 (50.7-100) %, which may be useful when choosing a management strategy for patients with NCCM.

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