基于肌营养不良蛋白重复的贝克型肌营养不良症中的自发性左心室小梁增多。
Spontaneous left ventricular hypertrabeculation in dystrophin duplication based Becker's muscular dystrophy.
作者信息
Finsterer J, Stöllberger C
机构信息
Ludwig Boltzmann Institute for Epilepsy and Neuromuscular Disorders, Vienna.
出版信息
Herz. 2001 Nov;26(7):477-81. doi: 10.1007/pl00002051.
BACKGROUND
Left ventricular hypertrabeculation is frequently associated with neuromuscular disorders. Whether left ventricular hypertrabeculation in these patients is congenital or develops during lifetime, is unknown.
CASE REPORT
In a 65-year-old man with Becker's muscular dystrophy, due to a duplication in the dystrophin gene on chromosome Xq21 (dystrophin molecular weight: 500 kD), left ventricular hypertrabeculation was detected on transthoracic echocardiography although being absent in repeated previous echocardiographic examinations. Additionally, there was thickening of the left ventricular myocardium. The spontaneous occurrence of left ventricular hypertrabeculation was interpreted as progression of cardiac involvement in Becker's muscular dystrophy.
CONCLUSION
Left ventricular hypertrabeculation may not exclusively be congenital, but may occasionally develop spontaneously during lifetime, being interpreted as progression of cardiac involvement in Becker's muscular dystrophy.
背景
左心室肌小梁增多常与神经肌肉疾病相关。这些患者的左心室肌小梁增多是先天性的还是在生命过程中发展而来尚不清楚。
病例报告
一名65岁患有贝克型肌营养不良症的男性，因Xq21染色体上的抗肌萎缩蛋白基因重复（抗肌萎缩蛋白分子量：500kD），经胸超声心动图检测发现左心室肌小梁增多，尽管之前多次超声心动图检查均未发现。此外，左心室心肌增厚。左心室肌小梁增多的自发出现被解释为贝克型肌营养不良症心脏受累的进展。
结论
左心室肌小梁增多不一定完全是先天性的，而是可能偶尔在生命过程中自发出现，被解释为贝克型肌营养不良症心脏受累的进展。
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