Abstract：
Amyloid cardiomyopathy (CA) was previously considered a rare disease; however, rapid advancements in imaging modalities have led to an increased frequency of its diagnosis. The aim of this prospective study was to assess the prevalence and clinical phenotype of transthyretin amyloidosis (ATTR) cardiomyopathy in patients exhibiting unexplained increased left ventricular (LV) wall thickness. From 2020 to 2022, we enrolled 100 consecutive adults with unexplained increased LV wall thickness in the study. The analysis included clinical data, electrocardiography, transthoracic echocardiography, single-photon emission computed tomography/computed tomography with 3,3-disphono-1,2-propanodicarboxylic acid, genetic testing. Overall, 18% of patients were diagnosed with CA, comprising 5% with light-chain amyloidosis, and 12% with ATTR. To evaluate associations with the ATTR diagnosis, a LOGIT model and multivariate analysis were applied. Notably, age, polyneuropathy, gastropathy, carpal tunnel syndrome, lumbar spine stenosis, low voltage, ventricular arrhythmia, LV mass, LV ejection fraction, global longitudinal strain (GLS), E/A, E/E\', right ventricle (RV) thickness, right atrium area, RV VTI, TAPSE, apical sparing, ground glass appearance of myocardium, thickening of interatrial septum, thickening of valves, and the \"5-5-5\" sign were found to be significantly associated with ATTR (p < 0.05). The best predictive model for ATTR diagnoses exhibited an area under the curve of 0.99, including LV mass, GLS and RV thickness. This study, conducted at a cardiology referral center, revealed that a very considerable proportion of patients with unexplained increased LV wall thickness may suffer from underlying CA. Moreover, the presence of ATTR should be considered in patients with increased LV mass accompanied by reduced GLS and RV thickening.
摘要：
淀粉样心肌病（CA）以前被认为是一种罕见的疾病；然而，影像学模式的快速发展导致其诊断频率增加.这项前瞻性研究的目的是评估甲状腺激素水平淀粉样变性（ATTR）心肌病在无法解释的左心室（LV）壁厚增加的患者中的患病率和临床表型。从2020年到2022年，我们在研究中连续招募了100名原因不明的LV壁厚增加的成年人。分析包括临床数据，心电图,经胸超声心动图,单光子发射计算机断层扫描/计算机断层扫描与3,3-二音-1,2-丙二羧酸，基因测试。总的来说,18%的患者被诊断为CA，包含5％的轻链淀粉样变性，和12％与ATTR。为了评估与ATTR诊断的关联，采用LOGIT模型和多变量分析。值得注意的是,年龄,多发性神经病,胃病,腕管综合征,腰椎管狭窄,低电压，室性心律失常,低压质量,左心室射血分数,全局纵向应变(GLS)，E/A,E/E\',右心室(RV)厚度,右心房区,RVVTI,TAPSE,顶端备用，心肌的毛玻璃外观，房间隔增厚，瓣膜增厚，发现“5-5-5”征与ATTR显着相关（p<0.05）。ATTR诊断的最佳预测模型显示曲线下面积为0.99，包括左心室质量，GLS和RV厚度。这项研究,在心脏病转诊中心进行，揭示了相当大比例的左心室壁厚不明原因增加的患者可能患有潜在的CA。此外，对于左心室质量增加并伴有GLS减少和RV增厚的患者,应考虑存在ATTR.
