心脏淀粉样变性
医学
淀粉样变性
限制性心肌病
心肌病
心力衰竭
心功能曲线
内科学
淀粉样变性
淀粉样蛋白(真菌学)
心脏病学
舒张期
病理
免疫球蛋白轻链
抗体
免疫学
血压
作者
Ronglih Liao,Mohit Jain,Paige Teller,Lawreen H. Connors,Soeun Ngoy,Martha Skinner,Rodney H. Falk,Carl S. Apstein
出处
期刊:Circulation
[Ovid Technologies (Wolters Kluwer)]
日期:2001-10-02
卷期号:104 (14): 1594-1597
被引量:252
标识
DOI:10.1161/circ.104.14.1594
摘要
Background Primary (AL) amyloidosis is a plasma cell dyscrasia characterized by clonal production of immunoglobulin light chains (LC) resulting in the subsequent systemic deposition of extracellular amyloid fibrils. Cardiac involvement is marked by the hemodynamic pattern of impaired diastolic filling and restrictive cardiomyopathy. Although cardiac death in patients with AL amyloidosis is usually associated with extensive myocardial infiltration, the infiltration alone does not correlated with the degree of heart failure or survival. We hypothesized that circulating monoclonal LC may directly impair cardiac function, in addition to any mechanical effects of amyloid fibril deposition. Therefore, we examined the effects of amyloid LC proteins on diastolic and systolic cardiac function, as measured in an isolated mouse heart model. Methods and Results LC were obtained from patients with nonamyloid disease or from those with noncardiac, mild cardiac, and severe cardiac involved AL amyloidosis. Saline or LC (100 μg/mL) was infused into a Langendorff-perfused, isovolumically contracting mouse heart. Saline and control, noncardiac, and mild-cardiac LC infusions did not alter ex vivo cardiac function. In contrast, infusion of sever cardiac LC resulted in marked impairment of ventricular relaxation with preservation of contractile function. Conclusion These results demonstrate that infusion of LC from patients with AL amyloidosis result in diastolic dysfunction similar to that observed in patients with cardiac involved AL amyloidosis, and they suggest that amyloid LC proteins may contribute directly to the pathogenesis and the rapid progression of amyloid cardiomyopathy, independent of extracellular fibril deposition.
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