Heart and Vascular Center, Semmelweis University, Budapest, Hungary.
Department of Internal Medicine and Haematology, Semmelweis University, Budapest, Hungary.
PLoS One. 2022 Jun 13;17(6):e0269807. doi: 10.1371/journal.pone.0269807. eCollection 2022.
While patients with cardiac transthyretin amyloidosis are easily diagnosed with bone scintigraphy, the detection of cardiac light chain (AL) amyloidosis is challenging. Cardiac magnetic resonance (CMR) analyses play an essential role in the differential diagnosis of cardiomyopathies; however, limited data are available from cardiac AL-Amyloidosis. Hence, the purpose of the present study was to analyze the potential role of CMR in the detection of cardiac AL-amyloidosis.
We included 35 patients with proved cardiac AL-amyloidosis and two control groups constituted by 330 patients with hypertrophic cardiomyopathy (HCM) and 70 patients with arterial hypertension (HT), who underwent CMR examination. The phenotype and degree of left ventricular (LV) hypertrophy and the amount and pattern of late gadolinium enhancement (LGE) were evaluated. In addition, global and regional LV strain parameters were also analyzed using feature-tracking techniques. Sensitivity and specificity of several CMR parameters were analyzed in diagnosing cardiac AL-amyloidosis.
The sensitivity and specificity of diffuse septal subendocardial LGE in diagnosing cardiac AL-amyloidosis was 88% and 100%, respectively. Likewise, the sensitivity and specificity of septal myocardial nulling prior to blood pool was 71% and 100%, respectively. In addition, a LV end-diastolic septal wall thickness ≥ 15 mm had an optimal diagnostic performance to differentiate cardiac AL-amyloidosis from HT (sensitivity 91%, specificity 89%). On the other hand, a reduced global LV longitudinal strain (< 15%) plus apical sparing (apex-to-base longitudinal strain > 2) had a very low sensitivity (6%) in detecting AL-Amyloidosis, but with very high specificity (100%).
The findings from this study suggest that CMR could have an optimal diagnostic performance in the diagnosis of cardiac AL-amyloidosis. Hence, further larger studies are warranted to validate the findings from this study.
虽然患有心脏转甲状腺素淀粉样变性的患者很容易通过骨闪烁显像来诊断，但心脏轻链（AL）淀粉样变性的检测具有挑战性。心脏磁共振（CMR）分析在心肌病的鉴别诊断中起着至关重要的作用；然而，心脏 AL 淀粉样变性的相关数据有限。因此，本研究旨在分析 CMR 在检测心脏 AL 淀粉样变性中的潜在作用。
我们纳入了 35 例经证实的心脏 AL 淀粉样变性患者，并将其与两个对照组进行比较，对照组分别由 330 例肥厚型心肌病（HCM）患者和 70 例高血压（HT）患者组成，所有患者均接受了 CMR 检查。评估了左心室（LV）肥厚的表型和程度，以及晚期钆增强（LGE）的量和模式。此外，还使用特征追踪技术分析了整体和局部 LV 应变参数。分析了几种 CMR 参数在诊断心脏 AL 淀粉样变性中的敏感性和特异性。
弥漫性间隔心内膜下 LGE 诊断心脏 AL 淀粉样变性的敏感性和特异性分别为 88%和 100%。同样，血池前间隔心肌消影的敏感性和特异性分别为 71%和 100%。此外，LV 舒张末期间隔壁厚度≥15mm 对区分心脏 AL 淀粉样变性与 HT 具有最佳的诊断性能（敏感性 91%，特异性 89%）。另一方面，整体 LV 纵向应变降低（<15%）伴心尖保留（心尖至基底纵向应变>2）对 AL 淀粉样变性的检测敏感性（6%）非常低，但特异性（100%）非常高。
本研究结果表明，CMR 对心脏 AL 淀粉样变性的诊断具有最佳的诊断性能。因此，需要进一步的大规模研究来验证本研究的结果。
