Incidence, clinical characteristics, and diagnostic approach in transthyretin amyloid cardiomyopathy: The Kumamoto Cardiac Amyloidosis Survey
Incidence, clinical characteristics, and diagnostic approach in transthyretin amyloid cardiomyopathy: The Kumamoto Cardiac Amyloidosis Survey
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DOI：
10.1016/j.jjcc.2022.01.002
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发表时间：
2022-05-19
影响因子：
2.5
通讯作者：
Tsujita, Kenichi
中科院分区：
文献类型：
--
作者：
Takashio, Seiji;Tokitsu, Takanori;Tsujita, Kenichi
背景：近年来，运甲状腺素蛋白淀粉样变心肌病（ATTR-CM）日益受到关注；然而，ATTR-CM 在日本的流行病学尚不清楚。在熊本心脏淀粉样蛋白调查中，我们评估了 ATTR-CM 的当前发病率、临床特征、诊断方法和治疗策略，并将服用 tafamidis 处方的医院与地区医院进行了比较。 方法：我们进行了一项回顾性多中心观察队列研究。该登记册包括 2018 年 1 月至 2020 年 12 月期间在熊本市的两家 Tafamidis 处方医院机构 [日本循环学会 (JCS) 认证机构] 和 15 个地区心血管机构诊断出的 ATTR-CM 患者。 结果：总共有 174 名患者被诊断为 ATTR-CM。据估计，熊本市老年人群（> 65 岁）中 ATTR-CM 的发病率约为每 10,0 0 0 人年 1 例。与 JCS 认证设施队列 (n = 115) 相比，地区医院队列 (n = 59) 的诊断年龄明显较大（84.5 +/- 5.6 岁 vs. 77.5 +/- 6.3 岁；p < 0.01）。组织学诊断（25% vs. 81%；p < 0.01）和基因诊断（7% vs. 82%）的执行频率也较低。与 JCS 认证的设施队列相比，地区医院队列中的大多数病例中，可能（如骨显像结果阳性，并确认单克隆蛋白不存在）和可能（如骨显像结果阳性，但未确认单克隆蛋白缺失）ATTR-CM 占大多数病例（75% 对比 19%；p < 0.01）。接受TTR基因检测的患者中没有遗传性ATTR-CM病例（n = 98）。结论：我们确认了熊本市 ATTR-CM 的发病率以及 ATTR-CM 患者使用的诊断方法。需要进行更大样本的进一步前瞻性研究来验证我们的结果并进一步阐明日本 ATTR-CM 的流行病学。(c) 2022 年日本心脏病学院。由爱思唯尔有限公司出版。保留所有权利。版权所有。
Background: In recent years , transthyretin amyloid cardiomyopathy (ATTR-CM) has received increasing attention; however, the epidemiology of ATTR-CM in Japan is not yet understood. In the Kumamoto Cardiac Amyloid Survey, we evaluated the current incidence, clinical characteristics, diagnostic approaches, and treatment strategies for ATTR-CM and compared tafamidis-prescription hospitals with regional hospitals.Methods: We conducted a retrospective multicenter observational cohort study. The registry included patients with ATTR-CM diagnosed in two tafamidis-prescription hospital institutes [Japanese Circulation Society (JCS)-certified facilities] and 15 regional cardiovascular facilities in Kumamoto between January 2018 and December 2020.Results: In total, 174 patients were diagnosed with ATTR-CM. The incidence of ATTR-CM was estimated to be approximately 1 per 10,0 0 0 person-years in the elderly population ( > 65 years old) in Kumamoto. Compared with that in the JCS-certified facilities cohort (n = 115), age at diagnosis was significantly older (84.5 +/- 5.6 vs. 77.5 +/- 6.3 years old; p < 0.01) in the regional hospitals cohort (n = 59). Histological (25% vs. 81%; p < 0.01) and genetic diagnosis (7% vs. 82%) were also less frequently performed. Probable (as indicated by positive bone scintigraphy findings with confirmation of monoclonal protein absence) and possible (as indicated by positive bone scintigraphy findings without confirmation of monoclonal protein absence) ATTR-CM accounted for the majority of cases (75% vs. 19%; p < 0.01) in the regional hospitals cohort compared to the JCS-certified facilities cohort. There were no cases of hereditary ATTR-CM among the patients who underwent TTR genetic testing (n = 98). Conclusions: We confirmed the incidence of ATTR-CM in Kumamoto and the diagnostic approach used in patients with ATTR-CM. Further prospective studies with a larger sample are needed to validate our results and to further shed light on the epidemiology of ATTR-CM in Japan.(c) 2022 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. All rights reserved.