Objective Transthyretin amyloid cardiomyopathy (ATTR-CM) is an infiltrative cardiac disorder caused by deposition of wild type or mutated transthyretin. As ATTR-CM is associated with conduction disease, we sought to determine its prevalence in patients with idiopathic high-degree atrioventricular (AV) block requiring permanent pacemaker (PPM) implantation. Methods Consecutive patients aged 70–85 years undergoing PPM implantation for idiopathic high-degree AV block between November 2019 and November 2021 were offered a 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) scan. Demographics, comorbidities, electrocardiographic and imaging data from the time of device implantation were retrospectively collected. Results 39 patients (79.5% male, mean (SD) age at device implantation 76.2 (2.9) years) had a DPD scan. 3/39 (7.7%, all male) had a result consistent with ATTR-CM (Perugini grade 2 or 3). Mean (SD) maximum wall thickness of those with a positive DPD scan was 19.0 mm (3.6 mm) vs 11.4 mm (2.7 mm) in those with a negative scan (p=0.06). All patients diagnosed with ATTR-CM had spinal canal stenosis and two had carpal tunnel syndrome. Conclusions ATTR-CM should be considered in older patients requiring permanent pacing for high-degree AV block, particularly in the presence of left ventricular hypertrophy, carpal tunnel syndrome or spinal canal stenosis. All data relevant to the study are included in the article or uploaded as online supplemental information.

中文翻译：

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高度房室传导阻滞患者转甲状腺素蛋白心脏淀粉样变的患病率

目的转甲状腺素蛋白淀粉样心肌病（ATTR-CM）是一种由野生型或突变型转甲状腺素蛋白沉积引起的浸润性心脏病。由于 ATTR-CM 与传导疾病相关，我们试图确定其在需要植入永久起搏器 (PPM) 的特发性高度房室 (AV) 传导阻滞患者中的患病率。方法 对 2019 年 11 月至 2021 年 11 月期间连续接受 PPM 植入治疗特发性高度房室传导阻滞的 70-85 岁患者进行 3,3-二磷酸-1,2-丙二甲酸 (DPD) 扫描。回顾性收集装置植入时的人口统计、合并症、心电图和影像数据。结果 39 名患者（79.5% 为男性，装置植入时的平均 (SD) 年龄 76.2 (2.9) 岁）接受了 DPD 扫描。 3/39（7.7%，全部为男性）的结果与 ATTR-CM（Perugini 2 级或 3 级）一致。阳性 DPD 扫描的平均 (SD) 最大壁厚为 19.0 毫米 (3.6 毫米)，而阴性扫描的平均 (SD) 最大壁厚为 11.4 毫米 (2.7 毫米) (p=0.06)。所有诊断为 ATTR-CM 的患者均患有椎管狭窄，其中两人患有腕管综合征。结论 对于因高度房室传导阻滞而需要永久起搏的老年患者，尤其是存在左心室肥大、腕管综合征或椎管狭窄的患者，应考虑 ATTR-CM。与研究相关的所有数据都包含在文章中或作为在线补充信息上传。