Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome
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 regi...
| Main Authors: | , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Столичная издательская компания
2022-03-01
|
| Series: | Рациональная фармакотерапия в кардиологии |
| Subjects: | |
| Online Access: | https://www.rpcardio.online/jour/article/view/2666 |
| _version_ | 1797232061040295936 |
|---|---|
| author | O. V. Blagova E. V. Pavlenko N. V. Varionchik V. P. Sedov N. V. Gagarina E. A. Mershina M. E. Polyak E. V. Zaklyazminskaya A. V. Nedostup |
| author_facet | O. V. Blagova E. V. Pavlenko N. V. Varionchik V. P. Sedov N. V. Gagarina E. A. Mershina M. E. Polyak E. V. Zaklyazminskaya A. V. Nedostup |
| author_sort | O. V. Blagova |
| collection | DOAJ |
| description | 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). |
| first_indexed | 2024-03-08T14:00:38Z |
| format | Article |
| id | doaj.art-fa8149e033874d1eb376784f7edbbc68 |
| institution | Directory Open Access Journal |
| issn | 1819-6446 2225-3653 |
| language | English |
| last_indexed | 2024-04-24T15:54:17Z |
| publishDate | 2022-03-01 |
| publisher | Столичная издательская компания |
| record_format | Article |
| series | Рациональная фармакотерапия в кардиологии |
| spelling | doaj.art-fa8149e033874d1eb376784f7edbbc682024-04-01T07:43:42ZengСтоличная издательская компанияРациональная фармакотерапия в кардиологии1819-64462225-36532022-03-01181273510.20996/1819-6446-2022-02-011983Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy SyndromeO. V. Blagova0E. V. Pavlenko1N. V. Varionchik2V. P. Sedov3N. V. Gagarina4E. A. Mershina5M. E. Polyak6E. V. Zaklyazminskaya7A. V. Nedostup8I.M. Sechenov First Moscow State Medical University (Sechenov University)I.M. Sechenov First Moscow State Medical University (Sechenov University)I.M. Sechenov First Moscow State Medical University (Sechenov University)I.M. Sechenov First Moscow State Medical University (Sechenov University)I.M. Sechenov First Moscow State Medical University (Sechenov University)Medical Research and Education Center, Lomonosov Moscow State UniversityB.V. Petrovsky Russian Scientific Center for SurgeryB.V. Petrovsky Russian Scientific Center for SurgeryI.M. Sechenov First Moscow State Medical University (Sechenov University)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).https://www.rpcardio.online/jour/article/view/2666noncompact myocardiumdilated cardiomyopathyprognosislethalityintracardiac thrombosis |
| spellingShingle | O. V. Blagova E. V. Pavlenko N. V. Varionchik V. P. Sedov N. V. Gagarina E. A. Mershina M. E. Polyak E. V. Zaklyazminskaya A. V. Nedostup Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome Рациональная фармакотерапия в кардиологии noncompact myocardium dilated cardiomyopathy prognosis lethality intracardiac thrombosis |
| title | Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome |
| title_full | Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome |
| title_fullStr | Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome |
| title_full_unstemmed | Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome |
| title_short | Noncompact Myocardium with Dilated Phenotype: Manifestations, Treatment and Outcomes in Comparison with Other Forms of Dilated Cardiomyopathy Syndrome |
| title_sort | noncompact myocardium with dilated phenotype manifestations treatment and outcomes in comparison with other forms of dilated cardiomyopathy syndrome |
| topic | noncompact myocardium dilated cardiomyopathy prognosis lethality intracardiac thrombosis |
| url | https://www.rpcardio.online/jour/article/view/2666 |
| work_keys_str_mv | AT ovblagova noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT evpavlenko noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT nvvarionchik noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT vpsedov noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT nvgagarina noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT eamershina noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT mepolyak noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT evzaklyazminskaya noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome AT avnedostup noncompactmyocardiumwithdilatedphenotypemanifestationstreatmentandoutcomesincomparisonwithotherformsofdilatedcardiomyopathysyndrome |