cardiotomskСибирский журнал клинической и экспериментальной медициныSiberian Journal of Clinical and Experimental Medicine2713-29272713-265XTSU publishing10.29001/2073-8552-2023-39-3-163-171cardiotomsk-1960Research ArticleКЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯCLINICAL STUDIESКоличественная характеристика фенотипов кардиомиопатий у взрослых и детей по выраженности отклонения от нормы эхокардиографических показателейQuantitative characteristics of cardiomyopathy phenotypes in children and adults by the value of deviation from the norm of echocardiographic parametershttps://orcid.org/0000-0003-0513-9012СоколовА. А.SokolovA. A.

Соколов Александр Анатольевич, д-р мед. наук, профессор, заведующий лабораторией ультразвуковых и функциональных методов исследования

634012, Российская Федерация, Томск, ул. Киевская, 111а

Alexander A. Sokolov, Dr. Sci. (Med.), Professor, Head of the Department of Functional and Laboratory Diagnostics

111a, Kievskaya str., Tomsk, 634012, Russian Federation

asa@cardio-tomsk.ruhttps://orcid.org/0000-0002-6531-7223СморгонА. В.SmorgonA. V.

Сморгон Андрей Владимирович, младший научный сотрудник, лаборатория ультразвуковых и функциональных методов исследований

634012, Российская Федерация, Томск, ул. Киевская, 111а

Andrey V. Smorgon, Junior Researcher, Laboratory of Ultrasound and Functional Research Methods

111a, Kievskaya str., Tomsk, 634012, Russian Federation

sav@cardio-tomsk.ruhttps://orcid.org/0000-0002-0828-3995КожановР. М.KozhanovR. M.

Кривощеков Евгений Владимирович, д-р мед. наук, заведующий кардиохирургическим отделением № 2, врач сердечно-сосудистый хирург

634012, Российская Федерация, Томск, ул. Киевская, 111а

Evgeny V. Krivoshchekov, Dr. Sci. (Med.), Head of Cardiac Surgery Department No. 2, Cardiovascular Surgeon

111a, Kievskaya str., Tomsk, 634012, Russian Federation

kev@cardio-tomsk.ruhttps://orcid.org/0000-0002-0493-4762КривощековЕ. В.KrivoshchekovE. V.

Кожанов Роман Сергеевич, аспирант, кардиохирургическое отделение № 2, врач-сердечно-сосудистый хирург

634012, Российская Федерация, Томск, ул. Киевская, 111а

Roman S. Kozhanov, Postgraduate Student, Cardiosurgical Department № 2, Cardiovascular Surgeon

111a, Kievskaya str., Tomsk, 634012, Russian Federation

romankojanov@yandex.ruНаучно-исследовательский институт кардиологии, Томский национальный исследовательский медицинский центр Российской академии наукРоссияCardiology Research Institute, Tomsk National Research Medical Center, Russian Academy of SciencesRussian Federation202316102023383163171Copyright © Соколов А.А., Сморгон А.В., Кожанов Р.М., Кривощеков Е.В., 20232023Соколов А.А., Сморгон А.В., Кожанов Р.М., Кривощеков Е.В.Sokolov A.A., Smorgon A.V., Kozhanov R.M., Krivoshchekov E.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.sibjcem.ru/jour/article/view/1960

Широкое распространение и использование понятия «кардиомиопатия» в современной кардиологии диктует необходимость систематизации и конкретизации использования данного термина. На основании аппроксимации изменений функции, полостей и толщины стенок камер на известные дилатационную (ДКМП), гипертрофическую (ГКМП) и рестриктивную (РКМП) кардиомиопатии визуализационные методы в кардиологии позволили объединить многие патологические изменения камер сердца в определенные совокупности зрительного восприятия и сформировать понятия фенотипов и фенокопий большинства заболеваний сердца.

Цель

Цель: по результатам ретроспективного анализа большого объема цифровых данных эхокардиографических исследований у пациентов с болезнями миокарда и здоровых лиц разработать количественные критерии фенотипических изменений левого желудочка (ЛЖ), которые могут быть использованы для контроля динамики заболевания и оценки эффективности лечебных мероприятий.

Материал и методы

Материал и методы. Выполнен ретроспективный анализа протоколов эхокардиографии за период 2009–2021 гг. у 13 023 здоровых лиц в возрасте от 2 дней до 59 лет и 317 пациентов с различными заболеваниями сердца: ГКМП, ДКМП и РКМП, некомпактным миокардом (НКМ) в возрасте от 4 дней до 60 лет.

Результаты

Результаты. Разработаны и предложены критерии количественной оценки патологических фенотипов ЛЖ, имеющие высокую (более 95%) отрицательную специфичность у здоровых лиц любого возраста и положительную – у больных с кардиомиопатиями. Показана возможность количественно оценивать динамику течения заболеваний, протекающих с проявлениями рестрикции, дилатации и гипертрофии ЛЖ.

The widespread use of the term “cardiomyopathy” in modern cardiology dictates the need to systematize and concretize the use of this term. Based on the approximation of changes in the function, cavities and thickness of the walls of the chambers to known dilated, hypertrophic and restrictive cardiomyopathy, imaging methods in cardiology have made it possible to combine many pathological changes in the chambers of the heart into certain sets of visual perception and to form the concepts of phenotypes and phenotypes of most heart diseases.

Aim

Aim: To develop quantitative criteria for phenotypic changes in the left ventricle (LV), which can be used to control the dynamics of the disease and evaluate the effectiveness of treatment based on the results of a retrospective analysis of a large volume of digital data of echocardiographic studies in patients with myocardial diseases and healthy individuals.

Material and Methods

Material and Methods. A retrospective analysis of echocardiography protocols for the period 2009–2021 was performed in 13023 healthy individuals aged 2 days to 59 years and 317 patients with various heart diseases: hypertrophic, dilated and restrictive cardiomyopathies, and LV non-compaction myocardium aged 4 days to 60 years.

Results

Results. Criteria have been developed and proposed for the quantitative assessment by Z-index of pathological phenotypes of the left ventricle, which have a high (more than 95%) negative specificity in healthy individuals of any age and a positive one in patients with cardiomyopathies. The ability to quantify the dynamics of the course of diseases occurring with manifestations of restriction, dilation and LV hypertrophy was shown.

эхокардиографиякардиомиопатиифенотипZ-indexechocardiographycardiomyopathyphenotypeReferencesPorter K.E., Turner N.A. Cardiac fibroblasts: at the heart of myocardial remodeling. Pharmacol. Ther. 2009;123(2):255–278. DOI: 10.1016/j.pharmthera.2009.05.002.Porter K.E., Turner N.A. Cardiac fi broblasts: at the heart of myocardial remodeling. Pharmacol. Ther. 2009;123(2):255–278. DOI: 10.1016/j.pharmthera.2009.05.002.Kerkhof P.L., Yasha Kresh J., Li K.-J., Heyndrickx G.R. Left ventricular volume regulation in heart failure with preserved ejection fraction. Physiol. Rep. 2013;1(2):e0007. DOI: 10.1002/phy2.7.Kerkhof P.L., Yasha Kresh J., Li K.-J., Heyndrickx G.R. Left ventricular volume regulation in heart failure with preserved ejection fraction. Physiol. Rep. 2013;1(2):e0007. DOI: 10.1002/phy2.7.Jefferies J.L., Wilkinson J.D., Sleeper L.A., Colan S.D., Lu M., Pahl E. et al. Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: Results from the Pediatric Cardiomyopathy Registry. J. Card. Fail. 2015;21(11):877–884. DOI: 10.1016/j.cardfail.2015.06.381.Jefferies J.L., Wilkinson J.D., Sleeper L.A., Colan S.D., Lu M., Pahl E. et al. Cardiomyopathy phenotypes and outcomes for children with left ventricular myocardial noncompaction: Results from the Pediatric Cardiomyopathy Registry. J. Card. Fail. 2015;21(11):877–884. DOI: 10.1016/j.cardfail.2015.06.381.Paulus W.J., Tschöpe C. A novel paradigm for heart failure with preserved ejection fraction: comorbidities drive myocardial dysfunction and remodeling through coronary microvascular endothelial inflammation. J. Am. Coll. Cardiol. 2013;62(4):263–271. DOI: 10.1016/j.jacc.2013.02.092.Paulus W.J., Tschöpe C. A novel paradigm for heart failure with preserved ejection fraction: comorbidities drive myocardial dysfunction and remodeling through coronary microvascular endothelial infl ammation. J. Am. Coll. Cardiol. 2013;62(4):263–271. DOI: 10.1016/j.jacc.2013.02.092.Ciarambino T., Menna G., Sansone G., Giordano M. Cardiomyopathies: An overview. Int. J. Mol. Sci. 2021;22(14):7722. DOI: 10.3390/ijms22147722.Ciarambino T., Menna G., Sansone G., Giordano M. Cardiomyopathies: An overview. Int. J. Mol. Sci. 2021;22(14):7722. DOI: 10.3390/ijms22147722.Devereux R.B., de Simone G., Ganau A., Roman M.J. Left ventricular hypertrophy and geometric remodeling in hypertension: stimuli, functional consequences and prognostic implications. J. Hypertens. Suppl. 1994;12(10):S117–S127.Devereux R.B., de Simone G., Ganau A., Roman M.J. Left ventricular hypertrophy and geometric remodeling in hypertension: stimuli, functional consequences and prognostic implications. J. Hypertens. Suppl. 1994;12(10):S117–S127.Pfeffer M.A., Shah A.M., Borlaug B.A. Heart failure with preserved ejection fraction in perspective. Circ. Res. 2019;124(11):1598–1617. DOI: 10.1161/CIRCRESAHA.119.313572.Pfeffer M.A., Shah A.M., Borlaug B.A. Heart failure with preserved ejection fraction in perspective. Circ. Res. 2019;124(11):1598–1617. DOI: 10.1161/CIRCRESAHA.119.313572.Соколов А.А., Марцинкевич Г.И. Сердечная недостаточность у пациентов с сохраненной фракцией выброса левого желудочка – насосная несостоятельность сердца? Кардиология. 2018;58(6):79–84. DOI: 10.18087/cardio.2018.6.10125.Sokolov A.A., Martsinkevich G.I Heart failure in patients with preserved ejection fraction-pumping heart failure? Kardiologiia. 2018;58(6):79–84. (In Russ.) DOI: 10.18087/cardio.2018.6.10125.Navin P.J., Moynagh M.R., Atkinson E.J., Tirumanisetty P., LeBrasseur N.K., Kumar A. et al. Establishment of normative biometric data for body composition based on computed tomography in a North American cohort. Clin. Nutr. 2021;40(4):2435–2442. DOI: 10.1016/j.clnu.2020.10.046.Navin P.J., Moynagh M.R., Atkinson E.J., Tirumanisetty P., LeBrasseur N.K., Kumar A. et al. Establishment of normative biometric data for body composition based on computed tomography in a North American cohort. Clin. Nutr. 2021;40(4):2435–2442. DOI: 10.1016/j.clnu.2020.10.046.Hioki M., Kanehira N., Koike T., Saito A., Shimaoka K., Sakakibara H. et al. Age-related changes in muscle volume and intramuscular fat content in quadriceps femoris and hamstrings. Exp. Gerontol. 2020;132:110834. DOI: 10.1016/j.exger.2020.110834.Hioki M., Kanehira N., Koike T., Saito A., Shimaoka K., Sakakibara H. et al. Age-related changes in muscle volume and intramuscular fat content in quadriceps femoris and hamstrings. Exp. Gerontol. 2020;132:110834. DOI: 10.1016/j.exger.2020.110834.Shah S.J., Kitzman D.W., Borlaug B.A., van Heerebeek L., Zile M.R., Kass D.A. et al. Phenotype-specific treatment of heart failure with preserved ejection fraction: a multiorgan roadmap. Circulation. 2016;134(1):73–90. DOI: 10.1161/CIRCULATIONAHA.116.021884.Shah S.J., Kitzman D.W., Borlaug B.A., van Heerebeek L., Zile M.R., Kass D.A. et al. Phenotype-specifi c treatment of heart failure with preserved ejection fraction: a multiorgan roadmap. Circulation. 2016;134(1):73–90. DOI: 10.1161/CIRCULATIONAHA.116.021884.Lopez L., Colan S.D., Frommelt P.C., Ensing G.J., Kendall K., Younoszai A.K. et al. Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J. Am Soc. Echocardiogr. 2010;23(5):465–495; quiz 576–577. DOI: 10.1016/j.echo.2010.03.019.Lopez L., Colan S.D., Frommelt P.C., Ensing G.J., Kendall K., Younoszai A.K. et al. Recommendations for quantifi cation methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J. Am Soc. Echocardiogr. 2010;23(5):465–495; quiz 576–577. DOI: 10.1016/j.echo.2010.03.019.Jenni R., Oechslin E., Schneider J., Attenhofer Jost C., Kaufmann P.A. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: A step towards classification as a distinct cardiomyopathy. Heart. 2001;86(6):666–671. DOI: 10.1136/heart.86.6.666.Jenni R., Oechslin E., Schneider J., Attenhofer Jost C., Kaufmann P.A. Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: A step towards classifi cation as a distinct cardiomyopathy. Heart. 2001;86(6):666–671. DOI: 10.1136/heart.86.6.666.Chubb H., Simpson J.M. The use of Z-scores in paediatric cardiology. Ann. Pediatr. Cardiol. 2012;5(2):179–184. DOI: 10.4103/0974-2069.99622.Chubb H., Simpson J.M. The use of Z-scores in paediatric cardiology. Ann. Pediatr. Cardiol. 2012;5(2):179–184. DOI: 10.4103/0974-2069.99622.Lipshultz S.E., Orav E.J., Wilkinson J.D., Towbin J.A., Messere J.E., Lowe A.M. et al. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet. 2013;382(9908):1889–1897. DOI: 10.1016/S0140-6736(13)61685–2.Lipshultz S.E., Orav E.J., Wilkinson J.D., Towbin J.A., Messere J.E., Lowe A.M. et al. Risk stratifi cation at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet. 2013;382(9908):1889–1897. DOI: 10.1016/S0140-6736(13)61685–2.Augusto J.B., Eiros R., Nakou E., Moura-Ferreira S., Thomas A., Treibel T.A. et al. Dilated cardiomyopathy and arrhythmogenic left ventricular cardiomyopathy: a comprehensive genotype-imaging phenotype study. Eur. Heart J. Cardiovasc. Imaging. 2020;21(3):326–336. DOI: 10.1093/ehjci/jez188.Augusto J.B., Eiros R., Nakou E., Moura-Ferreira S., Thomas A., Treibel T.A. et al. Dilated cardiomyopathy and arrhythmogenic left ventricular cardiomyopathy: a comprehensive genotype-imaging phenotype study. Eur. Heart J. Cardiovasc. Imaging. 2020;21(3):326–336. DOI: 10.1093/ehjci/jez188.Gill R.S., Pelletie J.Sr., LaBossiere J., Bigam D.L., Cheung P.Y. Therapeutic strategies to protect the immature newborn myocardium during resuscitation following asphyxia. Can. J. Physiol. Pharmacol. 2012;90(6):689–695. DOI: 10.1139/y2012-041.Gill R.S., Pelletie J.Sr., LaBossiere J., Bigam D.L., Cheung P.Y. Therapeutic strategies to protect the immature newborn myocardium during resuscitation following asphyxia. Can. J. Physiol. Pharmacol. 2012;90(6):689–695. DOI: 10.1139/y2012-041.Dini F.L., Cortigiani L., Baldini U., Boni A., Nuti R., Barsotti L. et al. Prognostic value of left atrial enlargement in patients with idiopathic dilated cardiomyopathy and ischemic cardiomyopathy. Am. J. Cardiol. 2002;89(5):518–523. DOI: 10.1016/s0002-9149(01)02290-1.Dini F.L., Cortigiani L., Baldini U., Boni A., Nuti R., Barsotti L. et al. Prognostic value of left atrial enlargement in patients with idiopathic dilated cardiomyopathy and ischemic cardiomyopathy. Am. J. Cardiol. 2002;89(5):518–523. DOI: 10.1016/s0002-9149(01)02290-1.Blagova O, Alieva I, Kogan E, Zaytsev A, Sedov V, Chernyavskiy S. et al Mixed hypertrophic and dilated phenotype of cardiomyopathy in a patient with homozygous in-frame deletion in the MyBPC3 gene treated as myocarditis for a long time. Front. Pharmacol. 2020;11:579450. DOI: 10.3389/fphar.2020.579450.Blagova O, Alieva I, Kogan E, Zaytsev A, Sedov V, Chernyavskiy S. et al Mixed hypertrophic and dilated phenotype of cardiomyopathy in a patient with homozygous in-frame deletion in the MyBPC3 gene treated as myocarditis for a long time. Front. Pharmacol. 2020;11:579450. DOI: 10.3389/fphar.2020.579450.Towbin J.A., McKenna W.J., Abrams D.J., Ackerman M.J., Calkins H., Darrieux F.C.C. et al. 2019 HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy. Heart Rhythm. 2019;16(11):e301–e372. DOI: 10.1016/j.hrthm.2019.05.007.Towbin J.A., McKenna W.J., Abrams D.J., Ackerman M.J., Calkins H., Darrieux F.C.C. et al. 2019 HRS expert consensus statement on evaluation, risk stratifi cation, and management of arrhythmogenic cardiomyopathy. Heart Rhythm. 2019;16(11):e301–e372. DOI: 10.1016/j.hrthm.2019.05.007.

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