Yilmaz Ali, Gdynia Hans-Jürgen, Mahrholdt Heiko, Sechtem Udo
Division of Cardiology, Robert-Bosch-Krankenhaus, Stuttgart, Germany.
J Magn Reson Imaging. 2009 Oct;30(4):876-7. doi: 10.1002/jmri.21905.
Cardiac involvement in patients with a sarcoglycanopathy (limb-girdle muscular dystrophy) has been described previously; however, this is the first cardiovascular magnetic resonance (CMR) study in such a patient demonstrating an interesting pattern of myocardial damage using late gadolinium enhancement (LGE) imaging. Moreover, the wall motion abnormality and the subepicardial pattern of LGE in this patient with a sarcoglycanopathy is in agreement with the findings in another patient with Becker muscular dystrophy. The predominance of LGE in the subepicardial layers of the left ventricular inferolateral wall suggests that such a myocardial damage pattern represents a nonspecific cardiac phenotype in response to exaggerated mechanical stress in this region, at least in patients with a sarcoglycanopathy or dystrophinopathy.
之前已有文献描述了肌聚糖病（肢带型肌营养不良症）患者的心脏受累情况；然而，这是首例针对此类患者的心血管磁共振（CMR）研究，该研究通过延迟钆增强（LGE）成像展示了一种有趣的心肌损伤模式。此外，该肌聚糖病患者的室壁运动异常和LGE的心外膜下模式与另一例贝克型肌营养不良症患者的研究结果一致。左心室下外侧壁心外膜下层LGE的优势表明，这种心肌损伤模式代表了该区域因过度机械应力而产生的一种非特异性心脏表型，至少在肌聚糖病或肌营养不良蛋白病患者中如此。
