Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.
Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy.
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DOI：
10.1056/nejm200002103420601
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发表时间：
2000-02-10
影响因子：
158.5
通讯作者：
Spirito, P
中科院分区：
文献类型：
--
作者：
Maron, BJ;Shen, WK;Spirito, P
背景：肥厚型心肌病是一种与室性心律失常和猝死相关的遗传性疾病，特别是在年轻患者中。方法：我们对128例被判定为猝死高危的肥厚型心肌病患者进行了多中心的回顾性研究。结果：植入除颤器时，患者年龄8~82岁(平均[+/-SD]，40+/16岁)，69例(54%)小于41岁。平均随访时间为3.1年。29名患者(23%)在恢复窦性心律的情况下，通过提供除颤电击或抗心动过速起搏，适当地激活了除颤器；干预时的平均年龄为41岁。合适的除颤器放电率为每年7%。共有32名患者(25%)有过不适当的出院经历。在43名接受除颤器二级预防(在心脏骤停或持续性室性心动过速后)的患者中，有19名患者适当地激活了设备(每年11%)。在85名因危险因素(即一级预防)而接受预防性植入物的患者中，有10人进行了适当的干预(每年5%)。从植入到第一次适当的出院之间的间隔变化很大，但在6名患者中显著延长了(4-9年)。21例已存储心电数据并进行适当干预的患者均由室性心动过速或纤颤触发。结论：室性心动过速或纤颤是肥厚型心肌病猝死的主要机制。在肥厚型心肌病的高危患者中，植入式除颤器在终止此类心律失常方面非常有效，表明这些设备在猝死的一级和二级预防中具有作用。(N Engl J Med 2000；342：365-73)(C)2000年，马萨诸塞州医学会。
Background: Hypertrophic cardiomyopathy is a genetic disease associated with a risk of ventricular tachyarrhythmias and sudden death, especially in young patients.Methods: We conducted a retrospective multicenter study of the efficacy of implantable cardioverter-defibrillators in preventing sudden death in 128 patients with hypertrophic cardiomyopathy who were judged to be at high risk for sudden death.Results: At the time of the implantation of the defibrillator, the patients were 8 to 82 years old (mean [+/-SD], 40+/-16), and 69 patients (54 percent) were less than 41 years old. The average follow-up period was 3.1 years. Defibrillators were activated appropriately in 29 patients (23 percent), by providing defibrillation shocks or antitachycardia pacing, with the restoration of sinus rhythm; the average age at the time of the intervention was 41 years. The rate of appropriate defibrillator discharge was 7 percent per year. A total of 32 patients (25 percent) had episodes of inappropriate discharges. In the group of 43 patients who received defibrillators for secondary prevention (after cardiac arrest or sustained ventricular tachycardia), the devices were activated appropriately in 19 patients (11 percent per year). Of 85 patients who had prophylactic implants because of risk factors (i.e., for primary prevention), 10 had appropriate interventions (5 percent per year). The interval between implantation and the first appropriate discharge was highly variable but was substantially prolonged (four to nine years) in six patients. In all 21 patients with stored electrographic data and appropriate interventions, the interventions were triggered by ventricular tachycardia or fibrillation.Conclusions: Ventricular tachycardia or fibrillation appears to be the principal mechanism of sudden death in patients with hypertrophic cardiomyopathy. In high-risk patients with hypertrophic cardiomyopathy, implantable defibrillators are highly effective in terminating such arrhythmias, indicating that these devices have a role in the primary and secondary prevention of sudden death. (N Engl J Med 2000;342:365-73.) (C)2000, Massachusetts Medical Society.