Personalizing Risk Stratification for Sudden Death in Dilated Cardiomyopathy: The Past, Present, and Future.
Personalizing Risk Stratification for Sudden Death in Dilated Cardiomyopathy: The Past, Present, and Future.
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DOI：
10.1161/circulationaha.116.027134
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发表时间：
2017-07-11
期刊：
影响因子：
37.8
通讯作者：
Prasad SK
中科院分区：
文献类型：
--
作者：
Halliday BP;Cleland JGF;Goldberger JJ;Prasad SK
DANISH研究（评估ICD对非缺血性心血管热衰竭患者死亡率的有效性的丹麦研究）的结果表明，对于许多扩张型心肌病（DCM）患者，植入式心律转复除颤器（ICD）不会延长寿命。需要准确识别更有可能死于心律失常而不太可能死于其他原因的患者，以确保改善结局和明智地使用资源。到目前为止，左心室射血分数（LVEF）已被用作选择DCM患者植入ICD以进行一级预防的关键标准。然而，登记数据表明，许多DCM和院外心脏骤停患者的LVEF没有显著降低。此外，许多LVEF降低的患者死于非突发性死亡。预测猝死风险较高或较低的方法包括检测心肌纤维化（室性心律失常的基质），微伏T波电交替（MTWA;电生理脆弱性的标志物）和基因检测。在约30%的患者中，通过晚期钆增强心血管磁共振成像确定了中壁纤维化，并且除了LVEF之外，还提供了用于预测SCD事件的增量值。MTWA代表了另一个有希望的预测因子，得到了大型荟萃分析的支持，这些分析强调了该测试的阴性预测价值。然而，这两种策略在临床实践中均未常规用于风险分层。需要来自随机试验的更有说服力的数据来告知具有这些特征的患者的管理。对扩张型心肌病遗传学以及特定突变如何影响心律失常风险的了解也在迅速增加。LMNA突变的发现是约6%的特发性DCM的原因，由于相关表型的恶性性质，通常支持更积极的管理。随着基因测序的扩展，识别进一步的高风险突变似乎是可能的，从而更好地做出知情的临床决策，并提供对疾病机制的深入了解。在未来5-10年内，我们预计这些技术将被整合到现有的算法，形成一个更敏感，具体和成本效益的方法来选择DCM患者植入ICD。
Results from the DANISH Study (Danish Study to Assess the Efficacy of ICDs in Patients with Non-ischemic Systolic Heat Failure on Mortality) suggest that, for many patients with dilated cardiomyopathy (DCM), implantable cardioverter defibrillators (ICD) do not increase longevity. Accurate identification of patients who are more likely to die of an arrhythmia and less likely to die from other causes is required to ensure improvement in outcomes and wise use of resources. Until now, left ventricular ejection fraction (LVEF) has been used as a key criterion for selecting patients with DCM for an ICD for primary prevention purposes. However, registry data suggest that many patients with DCM and an out-of-hospital cardiac arrest do not have a markedly reduced LVEF. Additionally, many patients with reduced LVEF die from non-sudden causes of death. Methods to predict a higher or lower risk of sudden death include the detection of myocardial fibrosis (a substrate for ventricular arrhythmia), microvolt T-wave alternans (MTWA; a marker of electrophysiological vulnerability) and genetic testing. Mid-wall fibrosis is identified by late gadolinium enhancement cardiovascular magnetic resonance imaging in around 30% of patients and provides incremental value in addition to LVEF for the prediction of SCD events. MTWA represents another promising predictor, supported by large meta-analyses that have highlighted the negative predictive value of this test. However, neither of these strategies have been routinely adopted for risk stratification in clinical practice. More convincing data from randomized trials are required to inform the management of patients with these features. Understanding of the genetics of DCM and how specific mutations affect arrhythmic risk is also rapidly increasing. The finding of a mutation in LMNA, the cause of around 6% of idiopathic DCM, commonly underpins more aggressive management due to the malignant nature of the associated phenotype. With the expansion of genetic sequencing, the identification of further high-risk mutations appears likely, leading to better informed clinical decision-making as well as providing insight into disease mechanisms. Over the next 5–10 years we expect these techniques to be integrated into the existing algorithm to form a more sensitive, specific and cost-effective approach to the selection of DCM patients for ICD implantation.