Department of Cardiology, Rabin Medical Center, Petah Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Department of Cardiology, Rabin Medical Center, Petah Tikva, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Am J Cardiol. 2020 Oct 15;133:126-133. doi: 10.1016/j.amjcard.2020.07.050. Epub 2020 Jul 28.
Since the diagnosis of cardiac amyloidosis (CA) is often delayed, echocardiographic findings are frequently indicative of advanced cardiomyopathy. We aimed to describe early echocardiographic features in patients subsequently diagnosed with CA. Preamyloid diagnosis echocardiographic studies were screened for structural and functional parameters and stratified according to the pathogenetic subtype (immunoglobulin light-chain [AL] or amyloid transthyretin [ATTR]). Abnormalities were defined based on published guidelines. Our cohort included 75 CA patients of whom 42 (56%) were diagnosed with AL and 33 (44%) with ATTR. Forty-two patients had an earlier echocardiography exam available for review. Patients presented with increased wall thickness (1.3 [interquartile range {IQR} 1.0, 1.5] cm) ≥3 years before the diagnosis of CA and relative wall thickness was increased (0.47 [IQR 0.41, 0.50]) ≥7 years prediagnosis. One to 3 years before CA diagnosis restrictive left ventricular (LV) filling pattern was present in 19% of patients and LV ejection fraction ≤50% was present in 21% of patients. Right ventricular dysfunction was detected concomitantly with disease diagnosis. The echocardiographic phenotype of ATTR versus AL-CA showed increased relative wall thickness (0.74 [IQR 0.62, 0.92] versus 0.62 [IQR 0.54, 0.76], p = 0.004) and LV mass index (144 [IQR 129, 191] versus 115 [IQR 105, 146] g/m, p = 0.020) and reduced LV ejection fraction (50 [IQR 44, 58] versus (60 [IQR 53, 60]%, p = 0.009) throughout the time course of CA progression, albeit survival time was similar. In conclusion, increased wall thickness and diastolic dysfunction in CA develop over a time course of several years and can be diagnosed in their earlier stages by standard echocardiography.
由于心脏淀粉样变性（CA）的诊断常常被延误，因此超声心动图的发现常常提示晚期心肌病。我们旨在描述随后被诊断为 CA 的患者的早期超声心动图特征。筛选了前淀粉样变诊断的超声心动图研究，以评估结构和功能参数，并根据致病亚型（免疫球蛋白轻链[AL]或淀粉样转甲状腺素[ATTR]）进行分层。根据已发表的指南定义异常。我们的队列包括 75 名 CA 患者，其中 42 名（56%）被诊断为 AL，33 名（44%）为 ATTR。42 名患者有可供复查的早期超声心动图检查。患者在 CA 诊断前 3 年以上出现壁增厚（1.3[四分位距{IQR}1.0，1.5]cm）和相对壁厚度增加（0.47[IQR 0.41，0.50]）≥7 年。在 CA 诊断前 1 至 3 年，19%的患者出现限制性左心室（LV）充盈模式，21%的患者出现 LV 射血分数≤50%。同时发现右心室功能障碍。ATTR 与 AL-CA 的超声心动图表型显示相对壁厚度增加（0.74[IQR 0.62，0.92]与 0.62[IQR 0.54，0.76]，p=0.004）和 LV 质量指数增加（144[IQR 129，191]与 115[IQR 105，146]g/m，p=0.020）和 LV 射血分数降低（50[IQR 44，58]与（60[IQR 53，60]%，p=0.009）贯穿 CA 进展的整个过程，尽管生存时间相似。总之，CA 中的壁增厚和舒张功能障碍在数年内发展，可以通过标准超声心动图在早期阶段诊断。
