Institut Cardiovascular, Hospital Clínic de Barcelona Universitat de Barcelona, Spain.
Departament de Biomedicina, Facultat de Medicina, Universitat de Barcelona, Spain.
J Am Heart Assoc. 2017 Sep 25;6(9):e006438. doi: 10.1161/JAHA.117.006438.
Marfan syndrome (MF) leads to aortic root dilatation and a predisposition to aortic dissection, mitral valve prolapse, and primary and secondary cardiomyopathy. Overall, regular physical exercise is recommended for a healthy lifestyle, but dynamic sports are strongly discouraged in MF patients. Nonetheless, evidence supporting this recommendation is lacking. Therefore, we studied the role of long-term dynamic exercise of moderate intensity on the MF cardiovascular phenotype.
In a transgenic mouse model of MF (), 4-month-old wild-type and MF mice were subjected to training on a treadmill for 5 months; sedentary littermates served as controls for each group. Aortic and cardiac remodeling was assessed by echocardiography and histology. The 4-month-old MF mice showed aortic root dilatation, elastic lamina rupture, and tunica media fibrosis, as well as cardiac hypertrophy, left ventricular fibrosis, and intramyocardial vessel remodeling. Over the 5-month experimental period, aortic root dilation rate was significantly greater in the sedentary MF group, compared with the wild-type group (∆mm, 0.27±0.07 versus 0.13±0.02, respectively). Exercise significantly blunted the aortic root dilation rate in MF mice compared with sedentary MF littermates (∆mm, 0.10±0.04 versus 0.27±0.07, respectively). However, these 2 groups were indistinguishable by aortic root stiffness, tunica media fibrosis, and elastic lamina ruptures. In MF mice, exercise also produced cardiac hypertrophy regression without changes in left ventricular fibrosis.
Our results in a transgenic mouse model of MF indicate that moderate dynamic exercise mitigates the progression of the MF cardiovascular phenotype.
马凡综合征（MF）可导致主动脉根部扩张，并易发生主动脉夹层、二尖瓣脱垂、原发性和继发性心肌病。总的来说，建议健康的生活方式要经常进行体育锻炼，但 MF 患者应强烈避免剧烈运动。然而，缺乏支持这一建议的证据。因此，我们研究了长期适度动态运动对 MF 心血管表型的作用。
在 MF 的转基因小鼠模型（）中，4 月龄野生型和 MF 小鼠在跑步机上进行 5 个月的训练；每组的久坐同窝仔作为对照。通过超声心动图和组织学评估主动脉和心脏重塑。4 月龄 MF 小鼠表现出主动脉根部扩张、弹性层断裂和中膜纤维化，以及心脏肥大、左心室纤维化和心肌内血管重塑。在 5 个月的实验期间，与野生型组相比，久坐 MF 组的主动脉根部扩张率明显更高（∆mm，0.27±0.07 与 0.13±0.02，分别）。与久坐 MF 同窝仔相比，运动显著降低了 MF 小鼠的主动脉根部扩张率（∆mm，0.10±0.04 与 0.27±0.07，分别）。然而，这两组在主动脉根部硬度、中膜纤维化和弹性层断裂方面没有区别。在 MF 小鼠中，运动还使心脏肥大恢复正常，而不改变左心室纤维化。
我们在 MF 的转基因小鼠模型中的结果表明，适度的动力运动可减轻 MF 心血管表型的进展。
