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Different contribution of T1- and T2-mapping to myocardial characterization in Fabry disease without heart failureтезисы доклада
- Авторы: Mershina E., Ryzhkova E., Moiseev A., Sinitsyn V., Pavlikova E.
- Сборник: The Ecr 2023 abstract book
- Тезисы
- Год издания: 2023
- DOI: 10.26044/ecr2023/C-16516
- Аннотация: Purpose. Fabry disease (FD) is X-linked recessive lysosomal storage disease. Chronic heart failure (CHF) is the leading cause of death in FD. Long-term follow-up studies and registries demonstrated that FD-specific therapy including enzyme replacement therapy (ERT) with agalsidase alfa and agalsidase beta may slow progression of cardiac disease reducing the rate of cardiovascular events, particularly when started early [1,2]. Cardiovascular magnetic resonance (CMR) is the gold standard for myocardial tissue characterization in FD patients. Т1- and T2- mapping in CMR can reveal subtle changes in myocardial tissue. That can help in early detection of cardiac involvement in FD patients. For this purpose in our study we decided to assess the value of T1-and T2-mapping in FD patients without signs and symptoms of CHF.Methods and materials. 18 FD patients (6 males/12 females, 36+14 years) were included in the study. For FD patients exclusion criterion was presence of myocardial hypertrophy in echocardiography. 18 healthy subjects (8 males/10 females, 45+16 years) were included in control group. Clinical (troponin T and/or NT-proBNP levels) and radiological parameters were analyzed. CMR was performed with 3T scanner (Siemens Magnetom Vida 3T, Germany). Native T1- and T2-mapping was performed before contrast administration and assessed in 16 segments. Only high quality CMR images were used for analysis. If apical segments of left ventricle (LV) had very thin wall, they were excluded from analysis of T1- and T2-mapping. Results. There was no difference in indexed LVEDV, indexed LVM or LVEF between FD patients and healthy subjects (64+13 vs 66+12 ml/m2, 65+12 vs 61+14 g/m2, 66+5 vs 64+6%, respectively, p>0,05). 1 FD patient had elevated level of NT-proBNP due to recent episode of atrial fibrillation. None of patients had elevation of troponin T level.LGE in the basal inferolateral segment was detected in 2 patients. Global native T1-mapping values were lower in FD group in comparison with control group (1123,1+37,7 vs 1212,9+50,0 ms, respectively, p<0,05).We analyzed global T2-mapping and T2-mapping in the basal inferolateral segment. No difference in T2-mapping was found between healthy subjects and FD patients (for global T2-mapping: 40,6+1,8 vs 39,5+1,8 ms, respectively, p>0,05; for T2-mapping in the basal inferolateral segment: 39,3+4,3 vs 40,2+3,0 ms, respectively, p>0,05).Conclusion. Т1- and T2-mapping with CMR permits the routine spatial visualization and quantification of changes in myocardial tissue based on changes in T1 and T2 relaxation times. These changes include specific disease pathways related to mainly intracellular disturbances of the cardiomyocyte, including glycosphingolipid accumulation in FD. [3] Early detection of cardiac involvement in FD and initiation ERT is crucial for FD patients due to prevention of cardiovascular complications.According to our data, T1-mapping alone can be used for detection of early cardiac involvement in FD. No difference in LVEDV, LVEF, indexed LVM, parameters of T2-mapping were found between healthy subjects and FD patients without myocardial hypertrophy and CHF.The limitation of our study was the small number of participants in each group.
- Добавил в систему: Гагарина Евгения Викторовна