Register of adult patients with noncompact left ventricular myocardium: classification of clinical forms and a prospective assessment of progression

Павленко, Е. В.; Благова, О В; Вариончик, Н В; Недоступ, А. В.; Седов, В П; Поляк, М Е; Заклязьминская, Е. В.

doi:10.15829/1560-4071-2019-2-12-25

Cited by 6 publications

(3 citation statements)

References 24 publications

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“…В том случае, когда толщина некомпактного слоя не соответствует принятому в настоящее время критерию, изменения в миокарде нужно расценивать как повышенную трабекулярность ЛЖ. Однако, по мнению ряда авторов, в основе повышенной трабекулярности ЛЖ могут лежать те же генетические причины, что и при развитии НМЛЖ, поэтому трансформация гипертрабекулярности в НМЛЖ может быть лишь вопросом времени [3].…”

Section: Discussionunclassified

A clinical case of takotsubo syndrome in combination with left ventricular hypertrabeculation

et al. 2020

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The article describes the clinical case of takotsubo syndrome in female, who has also echocardiographic picture of a left ventricular noncompaction cardiomyopathy. According to the result of magnetic resonance imaging, the diagnosis of noncompaction cardiomyopathy was not confirmed, left ventricular hypertrabeculation was revealed. There are single descriptions of the combination of takotsubo syndrome and left ventricular hypertrabeculation, the pathogenetic relationship of these conditions has not yet been established.

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Section: Discussionunclassified

A clinical case of takotsubo syndrome in combination with left ventricular hypertrabeculation

et al. 2020

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“…Цель расширенного обследования -выявление критериев исключения, уточнение нозологической природы синдрома ДКМП (верификация диагноза миокардита). Клинические варианты (маски) НКМ определялись в соответствии с описанными ранее критериями [6].…”

Section: Noncompact Myocardium With Dilated Phenotype некомпактный ми...unclassified

Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome

Blagova

Павленко

Вариончик

et al. 2022

Racionalʹnaâ farmakoterapiâ v kardiologii

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Aim. To study the place of NCM in the structure of DCM, its clinical features and influence on prognosis in comparison with other forms of DCM syndrome.Methods. The NCM registry includes 125 patients, mean age 46.4±15.1 years, 74 men and 51 women, median follow-up 14 [4.0; 41.0] months. The DCM registry included 365 patients, mean age 46.4±15.1 years, 253 men and 112 women, median follow-up 14 [5; 43.75] months. The examination included electrocardiography (ECG), ECG Holter monitoring, echocardiography, blood anti-heart antibody level evaluation, and additionally cardiac computed tomography, magnetic resonance imaging, DNA diagnostics (in the MYH7, MYBPC3, TPM1, TNNI3, TNNT2, ACTC1, TAZ, ZASP (LDB3), MYL2, MYL3, DES, LMNA, EMD, TTR gene panel), coronary angiography, right ventricular endomyocardial biopsy.Results. The proportion of patients with DCM phenotype in the NCM registry was 40% (n=49), another 11% (n=15) had NCM diagnosed simultaneously with acute/subacute myocarditis. Lethality in these subgroups was 12.2% and 33.3%, respectively, and was significantly higher than in asymptomatic, ischemic and arrhythmic variants of NCM. In the DCM registry, the proportion of patients with NСM was 21% (n=78), and increased left ventricular (LV) trabecularity was detected in another 18% (n=64). DCM patients with and without NСM did not differ by baseline echocardiographic parameters, heart failure class, and cardiotropic therapy. Pathogenic mutations were detected in 14% of DCM patients with NCM and only 3% of other patients with DCM (p<0.001). Only in patients without NCM the presence of mutations had a significant effect on lethality. The patients with NCM compared with the others DCM patients showed significantly lower increase in EF in early and late period (from 31.0±10.2 to 34.8±11.0 and 37.1±10.9% [р<0.05] vs from 31.8±9.7 to 38.8±11.3 and 42.3±12.4% [р<0.01] respectively), a greater incidence of premature ventricular beats (1568 [105;7000] vs 543.5 [77.75; 3194], p<0.05), appropriate defibrillator shocks and sudden deaths (17.9 vs 5.9%, p<0.001), intracardiac thrombosis (21.8 vs 13.5%, p=0.069) despite a greater frequency of anticoagulants (73.1 vs 57.4%, p<0<05). There were no significant differences in death (19.2 vs 18.5%) and transplantation (7.7 vs 3.8%) between patients with and without NCM. There were no cases of NCM regression.Conclusion. NCM is an independent form of DCM syndrome, which is characterized by higher frequency of pathogenic mutations, arrhythmic events, worse response to cardiotropic therapy, higher frequency of intracardiac thrombosis. The absence of mortality differences can be explained by the higher frequency of preventive interventions in this category of patients with DCM (prescription of anticoagulants, defibrillator implantation, heart transplantation).

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“…К числу причин ХСН, которые стали чаще выявляться в последние годы, относятся некомпактная кардиомиопатия (НКМ) и кардиотоксичность химиотерапии при онкологических заболеваниях. НКМ характеризуется повышенной трабекулярностью в сочетании с глубокими межтрабекулярными лакунами, не связанными с коронарным кровотоком и предрасполагающими к образованию тромбов [3]. Специфических клинических проявлений НКМ нет и ХСН -ведущий симптомокомплекс, который носит как систолический, так и диастолический характер [4].…”

Section: Introductionunclassified

Four-year follow-up of a patient with mixed cardiomyopathy and an implanted cardiac contractility modulation device: a case report

Uskach

Safiullina

Шарапова

et al. 2021

Cardiovasc Ther Prev

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The article presents a case report of a 28-year-old male patient with mixed dilated cardiomyopathy: myocardial noncompaction and chemotherapy-related cardiotoxicity, which led to severe heart failure (HF). With optimal drug therapy, the patient was implanted with a cardiac contractility modulation device in order to improve exercise tolerance, quality of life and relieve HF symptoms. Complex therapy has led to significant clinical and echocardiographic improvement. This case demonstrates a 4-year follow-up of a patient with a reduced left ventricular ejection fraction and an implanted cardiac contractility modulation device, whose condition, after several severe HF decompensations, was stabilized.

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Register of adult patients with noncompact left ventricular myocardium: classification of clinical forms and a prospective assessment of progression

Cited by 6 publications

References 24 publications

A clinical case of takotsubo syndrome in combination with left ventricular hypertrabeculation

A clinical case of takotsubo syndrome in combination with left ventricular hypertrabeculation

Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome

Four-year follow-up of a patient with mixed cardiomyopathy and an implanted cardiac contractility modulation device: a case report

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