植入式心电事件监测器揭示心脏淀粉样变性心律失常负荷：EXCALIBUR研究
EXCALIBUR研究为前瞻性单中心观察性研究，对110例新诊断、未治疗的ATTR-CM或AL-CA患者行全面表型评估（含心脏磁共振）后植入植入式心电事件监测器。结果显示需起搏器植入的缓慢性心律失常发生率为17.3%，ATTR-CM高于AL-CA；新发房颤发生率为28.2%，ATTR-CM亦更高；淀粉样负荷与心律失常事件相关。
要点
- 心脏淀粉样变性患者中需起搏器植入的缓慢性心律失常发生率为17.3%，ATTR-CM高于AL-CA。
- 无既往房颤患者中新发房颤发生率为28.2%，ATTR-CM高于AL-CA。
- 基线传导异常和心肌淀粉样负荷与缓慢性心律失常及新发房颤风险相关。
核心结论
- 心脏淀粉样变性中临床显著心律失常常见且常无症状，负荷与类型因亚型而异，并与疾病表型和心肌淀粉样负荷密切相关。
研究设计与样本
- 前瞻性单中心观察性研究，纳入110例新诊断、未治疗的ATTR-CM或AL-CA患者，行全面表型评估（含心脏磁共振）后植入植入式心电事件监测器。
主要发现
- 基线QRS时限与后续缓慢性心律失常事件相关（sHR 1.03，95%CI 1.01-1.04，P<0.001），心肌淀粉样负荷ECV亦相关（sHR 1.06，95%CI 1.02-1.10，P=0.002）。
- 心肌淀粉样负荷ECV与新发房颤风险相关（sHR 1.04，95%CI 1.00-1.08，P=0.038）。
- 随访期间21例（19.1%）死亡（ATTR-CM 10例[15.9%]，AL-CA 11例[23.4%]）；ATTR-CM终末心律均为无脉电活动，AL-CA中9例（81.8%）为无脉电活动、2例（18.2%）为持续性室性心律失常。
临床怎么用
- 对心脏淀粉样变性患者应关注无症状心律失常，植入式心电事件监测器可提高检出。
- ATTR-CM患者缓慢性心律失常和新发房颤风险高于AL-CA，需加强监测。
- 基线传导异常和心肌淀粉样负荷较高者可能需更密切心律监测。
局限性
- 单中心观察性研究，样本量110例，结果需多中心验证。
以上解读依据论文摘要（全文未开放获取或许可不允许翻译转载）。
原文
BACKGROUND: Cardiac amyloidosis (CA) is a progressive infiltrative cardiomyopathy associated with conduction disease and arrhythmias, although their true burden and relationship with disease phenotype remain incompletely defined. OBJECTIVES: The purpose of this study was to prospectively characterize arrhythmic burden using implantable loop recorders and explore associations with amyloid subtype and disease characteristics. METHODS: In this prospective single-center observational study, 110 treatment-naïve patients with a new diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM) or light-chain cardiac amyloidosis (AL-CA) underwent comprehensive phenotyping, including cardiac magnetic resonance, followed by implantable loop recorder implantation. RESULTS: Among 110 patients (ATTRwt-CM: 43, ATTRv-CM: 20, AL-CA: 47) bradyarrhythmias with a Class I indication for pacemaker implantation occurred in 17.3% and were more frequent in ATTR-CM than AL-CA (15 [23.8%] vs 4 [8.5%]; P = 0.036). Baseline conduction abnormalities (QRS duration: sHR: 1.03; [95% CI: 1.01-1.04]; P < 0.001) and higher myocardial amyloid burden were associated with subsequent bradyarrhythmic events (ECV: sHR: 1.06 [95% CI: 1.02-1.10]; P = 0.002). New atrial fibrillation occurred in 28.2% of patients without prior atrial fibrillation and was more frequent in ATTR-CM than AL-CA (15 [50.0%] vs 5 [12.2%]; P < 0.001) with higher amyloid burden associated with increased risk (ECV: sHR: 1.04; 95% CI: 1.00-1.08; P = 0.038). During follow-up 21 (19.1%) patients died (ATTR-CM: 10 [15.9%]; AL-CA: 11[23.4%]). In patients with ATTR-CM, the terminal cardiac rhythm was uniformly pulseless electrical activity; in patients with AL-CA, PEA was the terminal rhythm in 9 (81.8%) patients and 2 (18.2%) had sustained ventricular arrhythmias. CONCLUSIONS: In CA, clinically significant arrhythmias are common and frequently asymptomatic. Arrhythmic burden and patterns differ between amyloid subtypes and are closely associated with disease phenotype and myocardial amyloid burden. These findings provide prospective insights into arrhythmogenesis in CA and support the need for further studies to refine risk stratification and inform management strategies. (Exploration of Arrhythmia Burden in Cardiac Amyloidosis Using Implantable Loop Recorders [EXCALIBUR]; NCT04856267).
引用
- Ioannou A, Patel R, Mansell J, et al. Exploration of Arrhythmia Burden in Cardiac Amyloidosis Using Implantable Loop Recorders: The EXCALIBUR Study. J Am Coll Cardiol. 2026. PMID: 42201646. doi:10.1016/j.jacc.2026.04.030
供执业医师工作参考，不替代临床判断；数值与分级以指南原文为准。