Kukavica Deni, Pili Gianluca, Trancuccio Alessandro, Giannini Gala, Pergola Valerio, Memmi Mirella, Gambelli Patrick, Marino Maira, Morini Massimo, Bloise Raffaella, Napolitano Carlo, Mazzanti Andrea, Priori Silvia G
Molecular Cardiology Unit, IRCCS Istituti Clinici Scientifici Maugeri, Pavia, Italy.
Department of Molecular Medicine, University of Pavia, Pavia, Italy.
Europace. 2025 Feb 5;27(2). doi: 10.1093/europace/euae294.
Exercise stress test (EST) represents the gold standard for diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT). We aimed to determine the relevance of exercise induced VT for the occurrence of LAE at follow-up.
In RYR2-related CPVT patients who underwent a baseline EST, we assessed the incidence and severity of ventricular arrhythmias (VA). Data were analysed using logistic regression models and Cox proportional hazards models. The primary outcome was the occurrence of life-threatening arrhythmic event (LAE; composite of sudden cardiac death, aborted cardiac arrest, or hemodynamically non-tolerated VT) at follow-up. In 102 RYR2-related CPVT patients (65 females; median age 16 years, IQR: 11-36 years), exercise-induced VT (bidirectional in 64% of cases) was elicited in 56% patients. VT could not be induced in pre-school children. Lower basal heart rate, early onset VA (within the first step of EST) and heart rate at the first minute of recovery were associated with exercise-induced VT. Cox analyses showed that early onset VA (HR 6.0, 95% CI: 1.3-27.9, P = 0.022) and exercise-induced VT (HR 6.6, 95% CI: 1.5-29.1, P = 0.012) at baseline EST were significantly associated with the occurrence of LAE at follow-up, and remained associated even after correction for symptoms.
Early onset VA and exercise-induced VT at baseline EST was associated with LAE at follow-up, allowing to identify a sub-set of patients at higher risk already at diagnosis.
运动应激试验（EST）是诊断儿茶酚胺能多形性室性心动过速（CPVT）的金标准。我们旨在确定运动诱发的室性心动过速（VT）与随访时左心房扩大（LAE）发生的相关性。
在接受基线EST的RYR2相关CPVT患者中，我们评估了室性心律失常（VA）的发生率和严重程度。使用逻辑回归模型和Cox比例风险模型分析数据。主要结局是随访时发生危及生命的心律失常事件（LAE；包括心源性猝死、心脏骤停未遂或血流动力学不耐受的VT）。在102例RYR2相关CPVT患者（65例女性；中位年龄16岁，四分位间距：11 - 36岁）中，56%的患者诱发了运动诱发的VT（64%的病例为双向性）。学龄前儿童未诱发出VT。较低的基础心率、VA早期发作（在EST的第一步内）以及恢复第一分钟时的心率与运动诱发的VT相关。Cox分析显示，基线EST时VA早期发作（风险比[HR] 6.0，95%置信区间[CI]：1.3 - 27.9，P = 0.022）和运动诱发VT（HR 6.6，95% CI：1.5 - 29.1，P = 0.012）与随访时LAE的发生显著相关，即使在对症状进行校正后仍保持相关性。
基线EST时VA早期发作和运动诱发VT与随访时LAE相关，这使得在诊断时就能识别出一组风险较高的患者。
