心脏淀粉样变性患者的心肺运动试验。
Cardiopulmonary exercise testing in patients with Cardiac Amyloidosis.
机构信息
Division of Hematology and Oncology, Department of Medicine Columbia University Irving Medical Center.
Department of Biostatistics, Columbia University Irving Medical Center.
出版信息
Clin Lymphoma Myeloma Leuk. 2021 Aug;21(8):545-548. doi: 10.1016/j.clml.2021.03.015. Epub 2021 Apr 23.
BACKGROUND
Cardiac involvement and dysfunction are common in patients presenting with AL and ATTR Amyloidosis. Cardiopulmonary exercise testing (CPET) performance is the gold standard to quantify functional capacity.
PATIENTS AND METHODS
In this study, we evaluated CPET measurements in 41 patients with cardiac Amyloidosis and their correlation with current amyloid specific staging criteria.
RESULTS
In both AL and ATTR cardiac Amyloidosis, percent predicted peak VO2 is significantly reduced and correlates with biomarker abnormalities. The association of cardiac biomarkers with peak VO2 is stronger for AL Amyloidosis (NT-proBNP (r = -0.57, P=0.006), Troponin (r = -0.70, p < 0.001) than ATTR (NT-proBNP (r = -0.4, P = 0.04) and Troponin (r = -0.57, P = 0.002) despite lower left ventricular mass in the former, suggesting that this may be further evidence for light chain toxicity in AL amyloidosis.
CONCLUSION
Our findings suggest further evidence for AL toxicity.
背景
心脏受累和功能障碍在患有 AL 和 ATTR 淀粉样变性的患者中很常见。心肺运动测试（CPET）是量化功能能力的金标准。
患者和方法
在这项研究中，我们评估了 41 例心脏淀粉样变性患者的 CPET 测量值及其与当前淀粉样蛋白特异性分期标准的相关性。
结果
在 AL 和 ATTR 心脏淀粉样变性中，预计峰值 VO2 的百分比均显著降低，并与生物标志物异常相关。心脏生物标志物与峰值 VO2 的相关性在 AL 淀粉样变性中更强（NT-proBNP（r=-0.57，P=0.006），肌钙蛋白（r=-0.70，p<0.001），而在 ATTR 淀粉样变性中较弱（NT-proBNP（r=-0.4，P=0.04）和肌钙蛋白（r=-0.57，P=0.002），尽管前者的左心室质量较低，这表明这可能是 AL 淀粉样变性中轻链毒性的进一步证据。
结论
我们的研究结果进一步证明了 AL 毒性。
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