Duchenne/Becker muscular dystrophy Cardiac care in Czech republic: a cardiovascular magnetic resonance study with T1 mapping

• Autoři: PEŠL Martin; PANOVSKÝ Roman; HOLEČEK Tomáš; MÁCHAL Jan; FEITOVÁ Vera; MRÁZOVÁ Lenka; HABERLOVÁ Jana; SLABÁ Alžběta; VÍT Pavel; STARÁ Veronika; KINCL Vladimír
• Rok publikování: 2018
• Druh: Vyžádané přednášky
• Fakulta / Pracoviště MU: Lékařská fakulta
• Popis: The progressive cardiomyopathy that develops in Duchenne and Becker muscular dystrophy (DMD/BMD) boys during their teenage is presumed to be a secondary consequence of the fibrosis within the myocardium. This is hard to follow by standard echocardiography, due to narrow intercostal spaces, skeletal abnormities and uneasy positioning of the patients. The same time there are only limited data on using parametric imaging in these patients. Our study aimed to assess native T1 and extracellular volume (ECV) values in DMD/BMD patients. The Czech population of males with DMD/BMD was screened with help of registry of muscular dystrophies, REaDY. 49 teenage males underwent cardiac magnetic resonance examination including T1 native and postcontrast mapping measurements. Eligible for evaluation were 48 of them and those were compared with Control group of gender matched controls (n=13). DMD/BMD patients without late gadolinium enhancement (LGE) (n=27) were set as group D1, those DMD/BMD patients with LGE (n=21) were set as group D2. Results: Both DMD/BMD groups had higher T1 native relaxation time compared to controls. These results are concordant in all 6 segments as well as in global values. Group D2 had significantly increased global ECV 0.277 ± 0.046 vs 0.243 ± 0.013, (p < 0.05) and segmental ECV in inferolateral and anterolateral segments in comparison with controls. The results were significant also after the adjustment for subjects’ age. Conclusion: DMD/BMD males had increased native T1 relaxation time independently on the presence or absence of myocardial fibrosis. Elevated ECV was found only in patients with LGE. These results supports timely prescription of ACE inhibitors as prevention and slowing of heart muscle remodeling, the same time authors have to stress importance of cardiac magnetic resonance, giving more detailed and repeatable outcomes compare to standard echocardiography.