Biočić Stanko, Jurinjak Sandra Jakšić, Vincelj Josip, Barić Davor, Manojlović Spomenka, Brkljačić Diana Delić
Coll Antropol. 2015 Jun;39(2):441-5.
Primary light-chain (AL) amyloidosis is a plasma cell dyscrasia associated with the deposition of immunoglobulin-derived amyloid in multiple organs. In the heart, this results in an infiltrative cardiomyopathy, with increased left ventricular wall thickness, normal or decreased left ventricular (LV) cavity size and congestive heart failure. Cardiac involvement is a major determinant of prognosis of AL amyloidosis. We report a patient with cardiac amyloidosis proven by cardiac biopsy, and aim to point out at transthoracic echocardiography as the hallmark of diagnostics. Echocardiography revealed increased LV thickness at 20mm, impaired LV ejection fraction (EF) at 35%, enlarged atria, transmitral deceleration time at 156 ms and increased E/A ratio at 4.25. Early diagnosis and intervention can have a significant impact on the patient's response to treatment, especially when the underlying condition involves a malignancy or infiltrative disorder. Standard transthoracic echocardiography as a noninvasive diagnostic tool is valuable and has a significant role in diagnosis and prognosis.
原发性轻链(AL)淀粉样变性是一种浆细胞异常增生性疾病,与免疫球蛋白衍生的淀粉样物质在多个器官沉积有关。在心脏,这会导致浸润性心肌病,表现为左心室壁厚度增加、左心室(LV)腔大小正常或减小以及充血性心力衰竭。心脏受累是AL淀粉样变性预后的主要决定因素。我们报告一例经心脏活检证实的心脏淀粉样变性患者,旨在指出经胸超声心动图是诊断的关键特征。超声心动图显示左心室厚度增加至20mm,左心室射血分数(EF)受损,为35%,心房扩大,二尖瓣减速时间为156ms,E/A比值增加至4.25。早期诊断和干预对患者的治疗反应有重大影响,尤其是当潜在疾病涉及恶性肿瘤或浸润性疾病时。标准经胸超声心动图作为一种非侵入性诊断工具很有价值,在诊断和预后方面具有重要作用。