AMIODARONE THERAPY IN PATIENTS WITH HCM AND REFRACTORY CARDIAC SYMPTOMS

一项正在进行的临床试验正在制定，以评估治疗效果 大剂量口服胺碘酮治疗肥厚型心肌病 难治性心脏症状与标准药物和 血流动力学和左心室功能反应的特征 与胺碘酮相关，以帮助确定作用机制。目前为止， 40例顽固性心脏症状患者接受标准药物治疗 已经作为心脏病服务的住院患者进行了研究，并已经 在建立后2个月、6个月和1年被视为门诊患者 口服胺碘酮治疗。在停药几天后 在医院，大剂量口服胺碘酮取得了有益的临床效果 响应(定义为运动成绩至少提高50% 与没有药物价值和心脏症状的减少相比， 至少一个功能类别)在68%的患者组中。在慢性病期间 门诊胺碘酮治疗剂量较小，总体改善 维持(63%的临床应答者)；一些患者进一步表现 症状受益，而在其他情况下，症状更差而不是更高 剂量急性治疗。就像在这群严重的 有症状的患者对标准药物治疗没有反应，许多人 患者未能体验到持续的临床益处。尽管如此， 45%的患者仍在接受胺碘酮治疗 平均随访14个月，并继续描述为持续 症状改善。所有左心室患者中有一半 流出道梗阻患者在手术前被认为是手术对象 起始胺碘酮的症状有了足够的改善 这样手术就可以推迟。通过这项正在进行的临床试验，我们 结论：大剂量口服胺碘酮可提高运动能力和 减轻大多数肥厚性心脏病患者的心脏症状 对标准药物治疗无效的心肌病患者 长期的临床随访表明，临床上的 有效，尽管几乎有一半的患者继续治疗 第一年后症状持续改善。

An ongoing clinical trial was formulated to assess therapeutic efficacy of high dose oral amiodarone in patients with hypertrophic cardiomyopathy and refractory cardiac symptoms compared with standard pharmacologic agents and to characterize hemodynamic and left ventricular functional responses associated with amiodarone to help define mechanisms of action. Thusfar, 40 patients with refractory cardiac symptoms on standard medical therapies have been studied as inpatients on the Cardiology Service and have been seen as outpatients at 2 months, 6 months, and 1 year after instituting oral amiodarone treatment. After stopping medications for several days in the hospital, high dose oral amiodarone resulted in beneficial clinical responses (defined as at least a 50% improvement in exercise performance compared with no medication values and a decrease in cardiac symptoms by at least one functional class) in 68% of the patient group. During chronic lower dose outpatient amiodarone treatment, improvement was generally maintained (63% clinical responders); some patients manifested further symptom benefit, and in others symptoms were worse compared with higher dose acute treatment. As might be expected in this group of severely symptomatic patients unresponsive to standard pharmacologic therapy, many patients failed to experience maintained clinical benefit. Nonetheless, 45% of the total patient cohort are still receiving amiodarone for an average follow-up of 14 months and have continued to describe persistent improvement in symptoms. One half of all patients with left ventricular outflow tract obstruction who were deemed operative candidates prior to initiating amiodarone have experienced sufficient symptomatic improvement so that surgery could be deferred. From this ongoing clinical trial we conclude that high dose oral amiodarone improves exercise capacity and reduces cardiac symptoms in the majority of patients with hypertrophic cardiomyopathy who have been refractory to standard medical therapy and long-term clinical follow-up indicates a modest diminution in clinical efficacy although almost one half of all patients have continued treatment after the first year with continued symptomatic improvement.