a Cardiology Department , CHU de Poitiers , Poitiers , France.
b Imaging Center, University Hospital of Rangueil , Toulouse , France.
Amyloid. 2018 Mar;25(1):18-25. doi: 10.1080/13506129.2017.1417121. Epub 2017 Dec 20.
Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis.
We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN. The prognostic value of these parameters was analysed in the AL amyloidosis group.
Free-wall right ventricular longitudinal strain (FWRVLS) worse than -21.2% discriminates AL amyloidosis [area under the curve (AUC) = 0.744)] from patients with IWT due to other aetiologies. In AL amyloidosis, FWRVLS is the strongest echocardiographic prognostic marker with AUC =0.722 and -16.5% as the optimal cut-off value, beyond which overall mortality increases significantly. It is also the only independent echocardiographic predictor of overall mortality (HR =1.113; 95%CI 1.029-1.204; p = .007), even when adjusted to the Mayo stage and global left ventricular longitudinal strain.
FWRVLS should be considered in the diagnostic and prognostic workup in light-chain amyloidosis.
轻链（AL）淀粉样变性可导致浸润性心肌病，伴有壁增厚（IWT），预后极差。我们的主要目的是分析 IWT 患者的右心室（RV），以将 AL 淀粉样变性与肥厚型心肌病（HCM）或动脉高血压（HTN）引起的 IWT 区分开来。我们的次要目的是评估 RV 功能障碍是否可预测心脏 AL 淀粉样变性的总死亡率。
我们回顾性连续比较了 315 例 IWT 患者的临床、生物学和超声心动图数据：105 例经活检证实的 AL 淀粉样变性患者、105 例 HCM 患者和 105 例 HTN 患者。分析了这些参数在 AL 淀粉样变性组中的预后价值。
游离壁右心室纵向应变（FWRVLS）小于-21.2%可将 AL 淀粉样变性与其他病因引起的 IWT 患者区分开来（曲线下面积[AUC]为 0.744）。在 AL 淀粉样变性中，FWRVLS 是最强的超声心动图预后标志物，AUC=0.722，-16.5%为最佳截断值，超过该值，总死亡率显著增加。它也是总死亡率的唯一独立超声心动图预测因子（HR=1.113；95%CI 1.029-1.204；p=0.007），即使在调整了 Mayo 分期和整体左心室纵向应变后也是如此。
在轻链淀粉样变性的诊断和预后评估中应考虑 FWRVLS。
