肥厚型心肌病的治疗

Therapy for patients with hypertrophic cardiomyopathy

肥厚型心肌病(HCM)是一种相对常见的遗传性疾病,部分出现流出道梗阻,需要药物、介入治疗或外科治疗。心外科治疗是梗阻性HCM治疗的金标准,有近50年的历史。目前"改良扩大MORROW术式"治疗效果明显,绝大多数患者5年内症状改善,左室舒张和收缩功能恢复,晕厥次数减少。经皮经腔室间隔心肌化学消融术(PTSMA)1995年英国医生Sigwart首次在Lancet上报道了应用96%酒精阻塞间隔支成功治疗肥厚型心肌病。机制是使用化学的方法消除肥厚的室间隔,使左室流出道增宽,心脏后负荷减轻,心排出量增加。PTSMA需注意掌握好适应证、做好激发试验、术中注意靶血管的选择和心肌声学造影(MCE)以及无水酒精的使用。

Hypertrophic cardiomyopathy （HCM）, featured of left ventricular outflow tract obstruction in a subset of pa tients, is a common genetic disease that warrants medication, interventional or surgical therapy. Clinically, surgical myectomy has been the gold standard for treatment of obstructive HCM as a result of the history approximating 5 decades. It was not until recently that modified extended MORROW＇s surgical approach has been mostly associated with marked efficacy, as re flected by improvement in symptoms within 5 years, recovery in left ventricular diastolic and systolic capacity in addition to reduced episodes of syncope. The first septal ablation by using 96% alcohol,also termed PTSMA （percutaneous translumi nal septal myocardial ablation） concurrently, was conducted successfully and was reported by Sir Sigwart, a British doctor, in The Lancet in 1995. Eradication of hypertrophic ventricular septum was accomplished via chemical measures to extend left ventricular outflow, ameliorate cardiac after load and hence, improve cardiac output. Decisionmaking on the basis of indica tions, conducting challenge test prior to surgery, selecting target vessels according to myocardial contrast echocardiography （MCE） and use of alcohol absolute should be warranted for those underwent IYl＇SMA.