Blain Alison, Greally Elizabeth, Laval Steven H, Blamire Andrew M, MacGowan Guy A, Straub Volker W
Institute of Genetic Medicine, Newcastle University, International Centre for Life, Central Parkway, Newcastle Upon Tyne, NE1 3BZ, UK.
J Cardiovasc Transl Res. 2015 Apr;8(3):198-207. doi: 10.1007/s12265-015-9623-7. Epub 2015 Apr 21.
Most patients with Duchenne muscular dystrophy (DMD) will develop cardiomyopathy; however, the evidence for prophylactic treatment of children with cardiac medications is limited. We have used the mdx mouse model of DMD to assess if early combination treatment with beta blocker (BB) and ACE inhibitor (AI) is superior to single treatment with either one of these drugs. Mice were assessed with cardiac MRI (ventricular structure and function, in vivo calcium influx (manganese-enhanced MRI)), pressure-volume loops, and histopathology. Combination treatment did not show benefits over treatment with AI or BB alone. Indeed, some beneficial aspects of BB and AI were lost when used in combination. None of the treatments impacted RV function. Combination treatment had no significant effect on sarcolemmal damage or histopathology. The study suggests that combined BB and AI may not confer an advantage at an early stage in DMD cardiomyopathy. However, limitations of the mdx model should be considered.
大多数杜氏肌营养不良症（DMD）患者会发展为心肌病；然而，使用心脏药物对儿童进行预防性治疗的证据有限。我们利用DMD的mdx小鼠模型来评估β受体阻滞剂（BB）和血管紧张素转换酶抑制剂（AI）早期联合治疗是否优于这两种药物中的任何一种单独治疗。通过心脏磁共振成像（心室结构和功能、体内钙内流（锰增强磁共振成像））、压力-容积环和组织病理学对小鼠进行评估。联合治疗并未显示出比单独使用AI或BB治疗更具优势。事实上，BB和AI联合使用时，其一些有益方面丧失了。所有治疗均未影响右心室功能。联合治疗对肌膜损伤或组织病理学无显著影响。该研究表明，在DMD心肌病早期，BB和AI联合使用可能并无优势。然而，应考虑mdx模型的局限性。
