González-Torrecilla Esteban, Arenal Angel, Atienza Felipe, Datino Tomás, Bravo Loreto, Ruiz Pablo, Ávila Pablo, Fernández-Avilés Francisco
Cardiology Department, Hospital General Universitario "Gregorio Maranon". Doctor Esquerdo 46.
Rev Recent Clin Trials. 2015;10(2):111-27. doi: 10.2174/1574887110666150407104312.
Current indications for implantable cardioverter defibrillators (ICDs) in patients with channelopathies and cardiomyopathies of non-ischemic origin are mainly based on non-randomized evidence. In patients with nonischemic dilated cardiomyopathy (NIDCM), there is a tendency towards a beneficial effect on total mortality of ICD therapy in patients with significant left ventricular (LV) dysfunction. Although an important reduction in sudden cardiac death (SCD) seems to be clearly demonstrated in these patients, a net beneficial effect on total mortality is unclear mostly in cases with good functional status. Risk stratification has been changing over the last two decades in patients with hypertrophic cardiomyopathy (HCM). Its risk profile has been delineated in parallel with the beneficial effect of ICD in high risk patients. Observational results based on "appropriate" ICD interventions do support its usefulness both in primary and secondary SCD prevention in these patients. Novel risk models quantify the rate of sudden cardiac death in these patients on individual basis. Less clear risk stratification is available for cases of arrhythmogenic right ventricular cardiomyopathy (ARVC) and in other uncommon familiar cardiomyopathies. Main features of risk stratification vary among the different channelopathies (long QT syndrome -LQTS-, Brugada syndrome, etc) with great debate on the management of asymptomatic patients. For most familiar cardiomyopathies, ICD therapy is the only accepted strategy in the prevention of SCD. So far, genetic testing has a limited role in risk evaluation and management of the individual patient. This review aims to summarize these criticisms and to refine the current indications of ICD implantation in patients with cardiomyopathies and major channelopathies.
目前，植入式心脏复律除颤器（ICD）用于非缺血性病因的通道病和心肌病患者的指征主要基于非随机证据。在非缺血性扩张型心肌病（NIDCM）患者中，对于左心室（LV）功能严重不全的患者，ICD治疗对总死亡率有有益影响的趋势。虽然在这些患者中似乎已明确证明心脏性猝死（SCD）显著降低，但在功能状态良好的病例中，对总死亡率的净有益影响尚不清楚。在过去二十年中，肥厚型心肌病（HCM）患者的风险分层一直在变化。其风险特征已与ICD在高危患者中的有益作用并行描述。基于“适当的”ICD干预的观察结果确实支持其在这些患者的一级和二级SCD预防中的有用性。新型风险模型可根据个体情况量化这些患者的心脏性猝死发生率。对于致心律失常性右心室心肌病（ARVC）病例和其他罕见的家族性心肌病，风险分层不太明确。不同通道病（长QT综合征-LQTS-、Brugada综合征等）的风险分层主要特征各不相同，对于无症状患者的管理存在很大争议。对于大多数家族性心肌病，ICD治疗是预防SCD唯一被接受的策略。到目前为止，基因检测在个体患者的风险评估和管理中的作用有限。本综述旨在总结这些批评意见，并完善目前ICD植入在心肌病和主要通道病患者中的指征。
