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Progression of myocardial fibrosis by magnetic resonance imaging in patients with Duchenne and Becker muscular dystrophy and preserved left ventricular ejection fraction - a randomized clinical trial for treatment with ACE inhibitors

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Duchenne (DMD) and Becker (BMD) muscular dystrophies( MD) are inherited X-linked diseases characterized by absence or decrease of dystrophin, a sarcolemal protein that is essential for maintenance of the muscular membrane integrity during muscular contraction... Two patients died before follow-up CMR... For all 74 patients, MF increased significantly from 20.8 ± 17.3 % to 26.6 ± 18.7 % on the follow-up, p < 0,001... Lower progression of MF was also noted when comparing the two treated groups (LV dysfunction and normal LV function randomized to treated), 9.0 ± 9.9 % vs. 3.1 ± 7.4 %, p = 0,047, respectively... Using multivariate regression analysis, we found that belonging to the ACEI treated group decreases the progression of MF even after adjusting for age, creatine kinase level and baseline MF, p = 0.039) and indicated a trend for lower probability of presenting LVEF < 50% at follow-up CMR (OR = 3.18, p = 0.102, by logistic regression)... Myocardial fibrosis, detected by CMR, progressively increases in all patients with Duchenne and Becker muscular dystrophy over a period of 2.3 years... In patients with MF and preserved LVEF, the treatment with ACEI decreases the progression of MF... Patients with LV dysfunction at baseline show progression of MF despite of ACEI therapy... Our data suggest that early initiation of ACEI therapy, before LV dysfunction can be detected, can decrease the progression of MF in DMD and BMD.

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Myocardial Fibrosis Progression in DMD and Becker pts over a period of 2.3 years. SD Group - group with systolic dysfunction WSDF-T group - group without systolic dysfunction and with fibrosis, treated with ACEI WSDF-WT group - group without systolic dysfunction and with fibrosis, without treatment.
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Figure 1: Myocardial Fibrosis Progression in DMD and Becker pts over a period of 2.3 years. SD Group - group with systolic dysfunction WSDF-T group - group without systolic dysfunction and with fibrosis, treated with ACEI WSDF-WT group - group without systolic dysfunction and with fibrosis, without treatment.


Progression of myocardial fibrosis by magnetic resonance imaging in patients with Duchenne and Becker muscular dystrophy and preserved left ventricular ejection fraction - a randomized clinical trial for treatment with ACE inhibitors
Myocardial Fibrosis Progression in DMD and Becker pts over a period of 2.3 years. SD Group - group with systolic dysfunction WSDF-T group - group without systolic dysfunction and with fibrosis, treated with ACEI WSDF-WT group - group without systolic dysfunction and with fibrosis, without treatment.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4043786&req=5

Figure 1: Myocardial Fibrosis Progression in DMD and Becker pts over a period of 2.3 years. SD Group - group with systolic dysfunction WSDF-T group - group without systolic dysfunction and with fibrosis, treated with ACEI WSDF-WT group - group without systolic dysfunction and with fibrosis, without treatment.

View Article: PubMed Central - HTML

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Duchenne (DMD) and Becker (BMD) muscular dystrophies( MD) are inherited X-linked diseases characterized by absence or decrease of dystrophin, a sarcolemal protein that is essential for maintenance of the muscular membrane integrity during muscular contraction... Two patients died before follow-up CMR... For all 74 patients, MF increased significantly from 20.8 ± 17.3 % to 26.6 ± 18.7 % on the follow-up, p < 0,001... Lower progression of MF was also noted when comparing the two treated groups (LV dysfunction and normal LV function randomized to treated), 9.0 ± 9.9 % vs. 3.1 ± 7.4 %, p = 0,047, respectively... Using multivariate regression analysis, we found that belonging to the ACEI treated group decreases the progression of MF even after adjusting for age, creatine kinase level and baseline MF, p = 0.039) and indicated a trend for lower probability of presenting LVEF < 50% at follow-up CMR (OR = 3.18, p = 0.102, by logistic regression)... Myocardial fibrosis, detected by CMR, progressively increases in all patients with Duchenne and Becker muscular dystrophy over a period of 2.3 years... In patients with MF and preserved LVEF, the treatment with ACEI decreases the progression of MF... Patients with LV dysfunction at baseline show progression of MF despite of ACEI therapy... Our data suggest that early initiation of ACEI therapy, before LV dysfunction can be detected, can decrease the progression of MF in DMD and BMD.

No MeSH data available.


Related in: MedlinePlus