Prophylactic Implantable Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia and No Prior Ventricular Fibrillation or Sustained Ventricular Tachycardia
Prophylactic Implantable Defibrillator in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia and No Prior Ventricular Fibrillation or Sustained Ventricular Tachycardia
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DOI：
10.1161/circulationaha.109.913871
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发表时间：
2010-09-21
期刊：
影响因子：
37.8
通讯作者：
Marcus, Frank I.
中科院分区：
文献类型：
--
作者：
Corrado, Domenico;Calkins, Hugh;Marcus, Frank I.
背景-植入式心律转复除颤器（ICD）在无室颤（VF）或持续性室性心动过速的致心律失常性右心室心肌病/发育不良患者中的作用是一个尚未解决的问题。（62名男子和44名妇女;年龄，35.6 +/- 18岁），患有致心律失常性右心室心肌病/发育不良，基于1个或多个心律失常风险因素（如晕厥）接受ICD，非持续性室性心动过速、家族性猝死和程序性心室刺激诱导性心律失常。在58 +/- 35个月的随访期间，25例患者（24%）接受了适当的ICD干预，17例（16%）因危及生命的VF或心室扑动接受了电击。48个月时，实际生存率为100%，而无VF/心室扑动生存率为77%（对数秩P = 0.01）。晕厥显著预测任何适当的ICD干预（风险比，2.94; 95%置信区间，1.83至4.67; P = 0.013）和VF/室扑的电击（风险比，3.16; 95%置信区间，1.39至5.63; P = 0.005）。对于任何适当的ICD干预，程控心室刺激的阳性预测值为35%，对于VF/心室扑动电击的阳性预测值为20%，阴性预测值为70%和74%。27例孤立性家族性猝死的无症状患者均未接受适当的ICD治疗。20例患者（19%）有不适当的ICD干预措施，18（17%）有设备相关的complications. Conclusions，四分之一的患者与致炎性右心室心肌病/发育不良，并没有以前持续的室性心动过速或VF有适当的ICD干预措施。晕厥是挽救生命的ICD干预的重要预测因素，也是ICD的适应症。无症状患者可能不适用预防性ICD，因为无论家族性猝死和程控心室刺激结果如何，其发病风险均较低。程控心室刺激对ICD治疗的预测准确性较低。（循环。2010;122：1144-1152）。
Background-The role of implantable cardioverter-defibrillator (ICD) in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior ventricular fibrillation (VF) or sustained ventricular tachycardia is an unsolved issue.Methods and Results-We studied 106 consecutive patients (62 men and 44 women; age, 35.6 +/- 18 years) with arrhythmogenic right ventricular cardiomyopathy/dysplasia who received an ICD based on 1 or more arrhythmic risk factors such as syncope, nonsustained ventricular tachycardia, familial sudden death, and inducibility at programmed ventricular stimulation. During follow-up of 58 +/- 35 months, 25 patients (24%) had appropriate ICD interventions and 17 (16%) had shocks for life-threatening VF or ventricular flutter. At 48 months, the actual survival rate was 100% compared with the VF/ventricular flutter-free survival rate of 77% (log-rank P = 0.01). Syncope significantly predicted any appropriate ICD interventions (hazard ratio, 2.94; 95% confidence interval, 1.83 to 4.67; P = 0.013) and shocks for VF/ventricular flutter (hazard ratio, 3.16; 95% confidence interval, 1.39 to 5.63; P = 0.005). The positive predictive value of programmed ventricular stimulation was 35% for any appropriate ICD intervention and 20% for shocks for VF/ventricular flutter, with a negative predictive value of 70% and 74%. None of the 27 asymptomatic patients with isolated familial sudden death had appropriate ICD therapy. Twenty patients (19%) had inappropriate ICD interventions, and 18 (17%) had device-related complications.Conclusions-One fourth of patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia and no prior sustained ventricular tachycardia or VF had appropriate ICD interventions. Syncope was an important predictor of life-saving ICD intervention and is an indication for ICD. Prophylactic ICD may not be indicated in asymptomatic patients because of their low arrhythmic risk regardless of familial sudden death and programmed ventricular stimulation findings. Programmed ventricular stimulation had a low predictive accuracy for ICD therapy. (Circulation. 2010;122:1144-1152.)