Title: Advances in Hypertrophic Cardiomyopathy
标题：肥厚性心肌病的研究进展
Author作者: Semhar Tewelde
翻译 ：于璐 何磊磊 校对： 陈都 肖锋
HCM is a genetically transmitted autosomal dominant disorder with two variants: hypertrophic obstructive cardiomyopathy (HOCM), also known as idiopathic hypertrophic subaortic stenosis (IHSS) or asymmetric septal hypertrophy, and non-obstructive hypertrophic cardiomyopathy (HNCM), also known as Yamaguchi syndrome.
肥厚型心肌病（HCM）是染色体显性遗传性疾病，有两个类型：第一个种为肥厚梗阻性心肌病（HOCM），也被称为特发性肥厚性主动脉瓣下狭窄（IHSS）或不对称性室间隔肥厚。另一个种是非梗阻性肥厚性心肌病（HNCM），也被称为山口综合征。
The most serious complication of both variants of HCM is sudden cardiac death (SCD) and end-stage heart failure, which rapidly progresses to cardiac death after its occurrence.
以上两种肥厚型心肌病的最严重并发症是心脏性猝死（SCD）和终末期心脏衰竭，并将进而发展为心源性死亡。
Beta-blockers (1st line) and non-dihydropyridine calcium channel blockers are effective at improving clinical symptoms (syncope, dyspnea, chest pain, and exertional intolerance, etc.) however neither alone nor combined halt the progressive LV remodeling and prevent end-stage heart failure.
β-受体阻滞剂（第一线）和非二氢吡啶类钙通道阻滞剂能有效改善（晕厥，胸痛，呼吸困难和乏力等）临床症状，却不能停止LV重塑和终末期心脏衰竭
Cardiac transplantation is the only treatment available for end-stage heart failure, but must occur before the onset of pulmonary hypertension, kidney malfunction, and thromboembolism for success.
心脏移植是终末期心脏衰竭的唯一治疗方法，但该手术必须在肺动脉高压，肾功能障碍和血栓栓塞出现之前进行才能成功。
Class Ia anti-arrhythmic, disopyramide has been shown to be effective for symptomatic improvement (NYHA classification), but does not improve overall LV function or hypertrophy.
Ia类抗心律失常药——丙吡胺已证明对症状的改善是有效的（基于NYHA分级），但其并不能改善整体的LV功能和肥厚。
A recent study found that another class Ia anti-arrhythmic, cibenzoline has been shown not only to reduce symptoms, but also improved LV diastolic dysfunction and induced a regression of LV hypertrophy. In this study cibenzoline has halted the progression of HCM to end-stage heart failure.
最近的一项研究发现，另外一个Ia类的抗心律失常药，西苯唑啉已被证明不仅能减轻症状，而且改善了LV舒张功能障碍和LV肥大。该研究已经能阻止肥厚型心肌病进而发展至终末期心脏衰竭。

References 参考文献：
Hamada M, Ikeda S, et al. Advances in medical treatment of hypertrophic cardiomyopathy. Journal of Cardiology. July 2014 (64):1;1-10.