Department of Cardiology, Westmead Hospital, Sydney, NSW, Australia; Westmead Applied Research Centre, University of Sydney, Sydney, NSW, Australia.
Department of Cardiology, Westmead Hospital, Sydney, NSW, Australia; Westmead Applied Research Centre, University of Sydney, Sydney, NSW, Australia.
Heart Lung Circ. 2020 Apr;29(4):594-606. doi: 10.1016/j.hlc.2019.12.009. Epub 2020 Jan 3.
This state-of-the art review discusses sudden cardiac death (SCD) risk stratification and prevention using implantable cardioverter defibrillator (ICD) therapy and the place of catheter ablation in the major inherited cardiomyopathies and primary arrhythmic syndromes. ICD therapy protects against SCD in many inherited cardiac conditions, particularly the cardiomyopathies in advanced stages, such as hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC). However, they are not usually indicated in most patients with cardiac ion channelopathies, particularly long QT syndrome, since medical management is safe and preferable for most cases. The general exception is the secondary prevention setting following a cardiac arrest, where guidelines mostly support ICD therapy. However, in the case of catecholaminergic polymorphic ventricular tachycardia (CPVT), ICD therapy is less clear, with some studies indicating increased mortality when an ICD is used following a cardiac arrest, compared to optimal medical therapy alone. When ICDs are placed, they are commonly associated with morbidity, and do not reduce the burden of ventricular arrhythmias (VA), such that multiple ICD shocks can ensue. Catheter ablation has been shown to reduce VA burden, VA related symptoms and ICD therapy in correctly identified patients in each condition. Its role is particularly important in cases where monomorphic ventricular tachycardia (VT) is prevalent, such as Lamin-related dilated cardiomyopathy (DCM) and ARVC. Evidence is growing to support the use of catheter ablation to treat premature ventricular contraction (PVC) induced VF in the setting of long and short QT syndromes, CPVT, idiopathic VF and early repolarisation syndromes. In Brugada syndrome, epicardial substrate ablation can even apparently eliminate the electrocardiographic (ECG) phenotype and reduce VA burden during follow-up.
这篇最新综述讨论了使用植入式心脏复律除颤器（ICD）治疗进行心脏性猝死（SCD）风险分层和预防，以及导管消融在主要遗传性心肌病和原发性心律失常综合征中的作用。ICD 治疗可预防许多遗传性心脏疾病中的 SCD，特别是在晚期心肌病中，如肥厚型心肌病（HCM）和致心律失常性右心室心肌病（ARVC）。然而，在大多数心脏离子通道病患者中，通常不建议使用 ICD，特别是在长 QT 综合征中，因为在大多数情况下，药物治疗是安全且优选的。一般的例外是在心脏骤停后的二级预防环境中，此时指南大多支持 ICD 治疗。然而，在儿茶酚胺能多形性室性心动过速（CPVT）的情况下，ICD 治疗的效果不太明确，一些研究表明，与单独使用最佳药物治疗相比，在心脏骤停后使用 ICD 会增加死亡率。当放置 ICD 时，它们通常与发病率相关，并且不能减轻室性心律失常（VA）的负担，从而可能导致多次 ICD 电击。在每种情况下，导管消融已被证明可减少 VA 负担、VA 相关症状和 ICD 治疗，在正确识别的患者中。在单形性室性心动过速（VT）普遍存在的情况下，如 lamin 相关扩张型心肌病（DCM）和 ARVC，其作用尤为重要。越来越多的证据支持在长 QT 综合征、CPVT、特发性 VT 和早期复极综合征中使用导管消融治疗因室性早搏（PVC）引起的 VT 诱发的 VF。在 Brugada 综合征中，心外膜基质消融甚至可以明显消除心电图（ECG）表型并减少随访期间的 VA 负担。
