论文部分内容阅读
肥厚性心肌病(包括不对称室间隔肥厚)是一种原发性心肌病,直到20～30岁通常无临床表现,而且可能是成人发病和死亡的常见原因。通过超声心动描记显示肥厚的分布有着显著的变异。在无其它心脏病或可以引起心肌病的全身性疾病的情况下,不对称室间隔肥厚的许多病例是由于一种高外显率的常染色体显性遗传性缺陷。它是以室间隔左心室游离壁的不匀称增厚为其特征,当心肌收缩时,可以发生左心室阻塞,但在不对称间隔肥厚的多数病人是不出现的。肥厚性心肌病发生在婴儿
Hypertrophic cardiomyopathy, including asymmetric ventricular septal hypertrophy, is a cardiomyopathic primary disease that is usually not clinically manifest until age 20-30 and may be a common cause of morbidity and mortality in adults. Echocardiography showed a significant variation in the distribution of hypertrophy. In the absence of other heart diseases or systemic diseases that can cause cardiomyopathy, many cases of asymmetric septal hypertrophy result from a high penetrance autosomal dominant defect. It is characterized by an asymmetrical thickening of the left ventricular free wall in the septum and left ventricular occlusion when myocardial contractions occur, but not in most patients with asymmetric septal hypertrophy. Hypertrophic cardiomyopathy occurs in infants