Division of Cardiology, Inova Children's Hospital, Falls Church, Virginia, USA.
Montreal Heart Institute, Montreal, Quebec, Canada.
J Am Coll Cardiol. 2021 Feb 16;77(6):761-771. doi: 10.1016/j.jacc.2020.12.021.
Patients with tetralogy of Fallot are at risk for ventricular arrhythmias and sudden cardiac death. These abnormalities are associated with pulmonary regurgitation, right ventricular enlargement, and a substrate of discrete, slowly-conducting isthmuses. Although these arrhythmic events are rare, their prediction is challenging. This review will address contemporary risk assessment and prevention strategies. Numerous variables have been proposed to predict who would benefit from an implantable cardioverter-defibrillator. Current risk stratification models combine independently associated factors into risk scores. Cardiac magnetic resonance imaging, QRS fragmentation assessment, and electrophysiology testing in selected patients may refine some of these models. Interaction between right and left ventricular function is emerging as a critical factor in our understanding of disease progression and risk assessment. Multicenter studies evaluating risk factors and risk mitigating strategies such as pulmonary valve replacement, ablative strategies, and use of implantable cardiac-defibrillators are needed moving forward.
法洛四联症患者存在室性心律失常和心源性猝死风险。这些异常与肺动脉瓣反流、右心室扩大以及离散的、传导缓慢的峡部基质有关。尽管这些心律失常事件较为罕见，但对其进行预测具有挑战性。本综述将探讨当代风险评估和预防策略。目前已有许多变量被提出用于预测植入式心脏复律除颤器的获益人群。目前的风险分层模型将独立相关因素组合成风险评分。心脏磁共振成像、QRS 碎裂评估和在特定患者中进行电生理检查，可能会对这些模型进行一定程度的细化。左右心室功能之间的相互作用正成为我们理解疾病进展和风险评估的一个关键因素。需要开展多中心研究，评估风险因素和风险缓解策略，如肺动脉瓣置换术、消融策略以及植入式心脏除颤器的应用。
