β肌球蛋白重链及心脏型肌球蛋白结合蛋白C基因突变的
肥厚型心肌病患者生存分析
王曙霞;邹玉宝;王虎;王继征;薛浩;陈敬洲;惠汝太
【期刊名称】《中华老年心脑血管病杂志》 【年(卷),期】2012(014)004
【摘要】Objective To investigate the 6-year survival rate of patients with hypertrophiccardio-myopathy(HCM) due to gene mutations of (3 myosin heavy chain(MYH7) and cardiac myosin binding protein C(MYBPC3). Methods Gene mutations of MYH7 and MYBPC3 in 70 HCM patients were identified by sequencing during the prospective follow-up. Results The mean follow-up time was 5. 8±1. 8 years during which 14 patients died,of them,0 had MYH7 mutations and 4 had MYBPC3 mutations(P>0. 05). Sudden death occurred in 7 patients with MYH7 mutations in the head while no sudden death occurred in those with MYBPC3 mutations(P<0. 01). Conclusion MYH7 mutations and death occur in younger HCM patients with MYH7 mutations whose sudden death rate is high. The ventricular wall is thicker in HCM patients with MYH7 mutations in the head than in those with MYH7 mutations in the stem,which is easy to lead to heart failure. It is, thus, necessary to detect gene mutations in HCM patients.%目的 探讨携带β肌球蛋白重链(MYH7)及心脏型肌球蛋白结合蛋白C(MYBPC3)基因突变的肥厚型心肌病患者的6年生存情况.方法 对采用测序方法确定的携带MYH7及MYBPC3基因突变的70