Rossi Maddalena, Varrà Guerino Giuseppe, Porcari Aldostefano, Saro Riccardo, Pagura Linda, Lalario Andrea, Dore Franca, Bussani Rossana, Sinagra Gianfranco, Merlo Marco
Center for Diagnosis and Treatment of Cardiomyopathies, Cardiovascular Department, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) and University of Trieste, 34149 Trieste, Italy.
Department of Nuclear Medicine, Azienda Sanitaria Universitaria Giuliano-Isontina (ASUGI) and University of Trieste, 34149 Trieste, Italy.
Biomedicines. 2022 Jun 30;10(7):1566. doi: 10.3390/biomedicines10071566.
The epidemiology of cardiac amyloidosis (CA), traditionally considered a rare and incurable disease, has changed drastically over the last ten years, particularly due to the advances in diagnostic methods and therapeutic options in the field of transthyretin CA (ATTR-CA). On the one hand, the possibility of employing cardiac scintigraphy with bone tracers to diagnose ATTR-CA without a biopsy has unveiled the real prevalence of the disease; on the other, the emergence of effective treatments, such as tafamidis, has rendered an early and accurate diagnosis critical. Interestingly, the following subgroups of patients have been found to have a higher prevalence of CA: elderly subjects > 75 years, patients with cardiac hypertrophy hospitalized for heart failure with preserved ejection fraction, subjects operated on for bilateral carpal tunnel syndrome, patients with cardiac hypertrophy not explained by concomitant factors and individuals with aortic valve stenosis. Many studies investigating the prevalence of CA in these particular populations have contributed to rewriting the epidemiology of the disease, increasing the awareness of the medical community for a previously underappreciated condition. In this review, we summarized the latest evidence on the epidemiology of CA according to the different clinical settings typically associated with the disease.
心脏淀粉样变性(CA)传统上被认为是一种罕见且无法治愈的疾病,在过去十年中其流行病学发生了巨大变化,这尤其归因于转甲状腺素蛋白型CA(ATTR-CA)领域诊断方法和治疗选择的进步。一方面,使用骨显像剂进行心脏闪烁扫描来诊断ATTR-CA而无需活检的可能性揭示了该疾病的实际患病率;另一方面,诸如氯苯唑酸等有效治疗方法的出现使得早期准确诊断变得至关重要。有趣的是,已发现以下亚组患者的CA患病率较高:75岁以上的老年人、因射血分数保留的心力衰竭住院的心脏肥大患者、接受双侧腕管综合征手术的患者、无伴随因素解释的心脏肥大患者以及主动脉瓣狭窄患者。许多针对这些特定人群中CA患病率的研究有助于重新书写该疾病的流行病学,提高了医学界对这种先前未得到充分认识的疾病的认识。在本综述中,我们根据通常与该疾病相关的不同临床情况总结了关于CA流行病学的最新证据。