MANAGEMENT OF SYMPTOMS AND ARRHYTHMIAS IN HYPERTROPHIC CARDIOMYOPATHY

肥厚型心肌病(HCM)是一种遗传性疾病 猝死(SD)的重要原因，特别是在年轻人。什么时候 合并左心室流出道梗阻 经常与呼吸困难、心悸、头晕目眩和 晕厥。过去一年，在以下方面取得了进展：1) 识别有SD和晕厥风险的肥厚型心肌炎患者；2) 建立心肌缺血作为心力衰竭最重要机制 肥厚性心肌病儿童的SD和晕厥；3)抗心律失常治疗 SD高危患者的室性心动过速(VT)； 双腔起搏(DDD)替代心脏起搏的评价 左心室流出道梗阻的手术治疗；5)获得更好的 了解这种疾病的遗传学。 一项对225例患者(随访3年)的研究表明，1)非持续性 动态心电图室上性心动过速对无症状患者有良性预后意义 意识受损，并且没有在 电生理研究，以及2)室速的诱导性，特别是当 与晕厥或心跳骤停有关，识别PTS的一个亚组 患有SD的风险很高。在一项涉及54名年轻患者的研究中，心肌梗塞 脑缺血是14岁患者晕厥/心脏骤停的唯一原因 几年前。新的III类药物UK68798被发现具有强大的 HCM的抗心律失常特性：室性心动过速不再能被诱导或被诱导 更难诱导的有13例。发现DDD起搏有改善 与左心室流出量减少相关的症状和运动能力 21/24例出现药物耐药症状的梗阻。正常 超声心动图在慢性肥厚性心肌炎患者中的应用 他们在谱系中的地位确立了发生 代际“跳跃”。疾病基因图谱中的两个家庭的孩子 14号染色体，但心脏形态正常的患者 已评估。1例9个月大的正常儿童因心动过缓而晕厥 提示HCM可能导致婴儿猝死综合征(SID)。

Hypertrophic cardiomyopathy (HCM), is a hereditary disease that is an important cause of sudden death (SD), particularly in the young. When complicated by obstruction to left ventricular outflow tract (LVOT) it is frequently associated with dyspnea, palpitations, lightheadedness, and syncope. In the past year, progress was made in the following areas: 1) identification of HCM pts who are at risk for SD and syncope; 2) establishment of myocardial ischemia as the most important mechanism of SD and syncope in children with HCM; 3) antiarrhythmic therapy for ventricular tachycardia (VT) in patients at high risk for SD; 4) evaluation of dual chamber (DDD) pacing as an alternative to cardiac surgery for relief of LVOT obstruction; and 5) obtain a better understanding of the genetics of the disease. A study of 225 pts (follow up, 3 years), revealed that 1) non-sustained VT on Holter is of benign prognostic significance in pts without symptoms of impaired consciousness, and who do not have VT induced at electrophysiologic study, and 2) inducibility of VT, especially when associated with syncope or cardiac arrest, identifies a subgroup of pts at high risk for SD. In a study involving 54 young pts, myocardial ischemia was the sole cause of syncope/cardiac arrest in pts aged <14 yrs. UK68798, a novel class III drug, was found to have potent antiarrhythmic properties in HCM: VT could no longer be induced or was more difficult to induce in 13 pts. DDD pacing was found to improve symptoms and exercise performance associated with reduction in LVOT obstruction in 21/24 pts with drug-refractory symptoms. Normal echocardiograms recorded in pts who were obligate carriers of HCM by virtue of their position in the pedigree established the occurrence of generational 'skips'. Children of two families in which disease gene maps to chromosome 14 but who have normal cardiac morphology are being evaluated. Syncope due to bradycardia in an otherwise normal 9-mth child suggest that HCM may cause sudden infant death syndrome (SID).