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，特别是在年轻人中。 当 并发左心室流出道（LVOT）梗阻， 常伴有呼吸困难、心悸、头晕， 晕厥。 过去一年，在以下方面取得了进展： 识别有SD和晕厥风险的HCM患者; 2） 建立心肌缺血作为最重要的机制 HCM患儿的SD和晕厥; SD高风险患者的室性心动过速（VT）; 4） 评价双腔（DDD）起搏作为心脏起搏的替代方法 手术缓解LVOT梗阻;和5）获得更好的 了解疾病的遗传学。 一项225例患者的研究（随访，3年）显示：1）非持续性 霍尔特显示的室性心动过速对无症状患者具有良性预后意义 意识受损的人，并且在 电生理研究，和2）VT的诱导，特别是当 与晕厥或心脏骤停相关，确定了患者亚组 有很高的SD风险。 在一项涉及54名年轻患者的研究中， 缺血是14岁以下患者晕厥/心脏骤停的唯一原因 多岁 UK 68798是一种新型III类药物，被发现具有强效 HCM中的抗心律失常特性：VT不再能被诱导或 13例患者更难诱导。 发现DDD起搏改善 与LVOT减少相关的症状和运动表现 21/24例有药物难治性症状的患者出现梗阻。正常 在HCM的绝对携带者患者中记录超声心动图， 由于他们在谱系中的地位， 世代“跳跃”疾病基因图谱中的两个家庭的孩子 但心脏形态正常的人 评估。 其他方面正常的9个月儿童心动过缓导致晕厥1例 提示HCM可引起婴儿猝死综合征（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).