Cardiovascular Department, 'Ospedali Riuniti' and University of Trieste, Trieste, Italy.
J Cardiovasc Med (Hagerstown). 2013 Apr;14(4):281-8. doi: 10.2459/JCM.0b013e3283536534.
Amyloidosis is a systemic disease, related to different underlying causes, with frequent cardiac involvement. Clinical evaluation, echocardiography and electrocardiography represent important noninvasive tools in identification of cardiac involvement. The aim of this study was to assess the clinical-laboratory features of a series of patients affected by cardiac amyloidosis in order to evaluate the risk of cardiac mortality.
We evaluated 48 patients (men 65%, mean age 63 ± 11 years) with biopsy-proven diagnosis of amyloidosis and heart involvement observed from 1991 to 2009. All patients underwent clinical-laboratory evaluation at baseline and were followed up.
During a median follow-up of 9.5 months (first to third interquartile: 3-41.5 months), 24 patients (50%) died as a result of a cardiac cause. Survival free from cardiac death was 69, 50, 48 and 41% at 6, 12, 24 and 60 months from diagnosis, respectively. At multivariable Cox regression analysis, the presence of heart failure at enrolment [hazard ratio (HR) 4.67, 95% confidence interval (CI) 1.07-20.27, P = 0.04] and history of recent syncope (HR 3.97, 95% CI 1.28-12.34, P = 0.017) emerged as independent predictors of cardiac death. By using the equation derived from the multivariate analysis, individual survival probability at different times of follow-up was calculated.
We confirm the particularly poor outcome of cardiac amyloidosis in the short term. A careful clinical evaluation emerges as the most important tool for the prognostic stratification and quantification of risk in patients with cardiac amyloidosis.
淀粉样变性是一种全身性疾病，与不同的潜在病因有关，常伴有心脏受累。临床评估、超声心动图和心电图是识别心脏受累的重要非侵入性工具。本研究旨在评估一系列心脏淀粉样变性患者的临床-实验室特征，以评估心脏死亡的风险。
我们评估了 1991 年至 2009 年间经活检证实为淀粉样变性且心脏受累的 48 例患者（男性占 65%，平均年龄 63±11 岁）。所有患者均在基线时接受临床-实验室评估，并进行随访。
在中位数为 9.5 个月（第一至第三四分位数：3-41.5 个月）的随访期间，24 例（50%）患者因心脏原因死亡。诊断后 6、12、24 和 60 个月时，无心脏死亡的生存率分别为 69%、50%、48%和 41%。多变量 Cox 回归分析显示，入组时存在心力衰竭（危险比[HR]4.67，95%置信区间[CI]1.07-20.27，P=0.04）和近期晕厥史（HR3.97，95%CI1.28-12.34，P=0.017）是心脏死亡的独立预测因素。通过使用多变量分析得出的方程，计算了不同随访时间的个体生存概率。
我们证实心脏淀粉样变性在短期内的预后特别差。仔细的临床评估是对心脏淀粉样变性患者进行预后分层和量化风险的最重要工具。
