Department of Cardiology, The First Affiliated Hospital of China Medical University, Shenyang, China; Department of Cardiology, West China Hospital, Sichuan University, Chengdu, China.
Department of Cardiology, The First Affiliated Hospital of China Medical University, Shenyang, China.
Hellenic J Cardiol. 2019 Nov-Dec;60(6):375-383. doi: 10.1016/j.hjc.2019.01.015. Epub 2019 Feb 8.
Light-chain amyloidosis and transthyretin-related amyloidosis (wild-type and mutated) are three main types of systemic amyloidosis associated with a clinically relevant cardiac involvement. In this study, we compared prognosis in patients with different categories of cardiac amyloidosis using meta-analysis and present a systematic review.
A systematic literature search was performed through Jan 1, 2018, and two reviewers independently extracted data and assessed risk of bias. We extracted MACE and death endpoint events and hazard ratios from regression models and performed a meta-analysis of the multiple prognosis association studies.
We observed that there were significant MACE differences between patients diagnosed with transthyretin amyloidosis and light-chain amyloidosis (OR: 2.09; 95% CI: 1.06-4.12; P = 0.03), and the same is true in the sub-comparison between AL and mATTR or wtATTR (AL vs. mATTR: OR: 1.72; 95% CI: 1.06-2.82; P = 0.03; AL vs. wtATTR: OR: 1.48; 95% CI: 0.85-2.58; P = 0.17). However, no significant difference was observed between two transthyretin types (P = 0.17). Overall death rate evaluated showed that compared with transthyretin-related amyloidosis, light-chain type showed a significant difference (P < 0.05). The prognostic analysis showed that types of amyloidosis, LVEF, NYHA, restrictive filling pattern, E-wave deceleration time, E/E' ratio, and low QRS voltage were predictors of cardiac-related mortality.
Patients diagnosed with light-chain amyloidosis has a poor prognosis compared with transthyretin-related amyloidosis, while no difference was proved in prognostic analysis between wild-type and mutated TTR amyloidosis. Some clinical factors related to the death prognosis, such as the LVEF, restrictive filling pattern, E-wave deceleration time, and E/E' ratio are important prognostic factors.
轻链淀粉样变和转甲状腺素蛋白相关淀粉样变（野生型和突变型）是三种与临床相关的心脏受累相关的系统性淀粉样变。在本研究中，我们使用荟萃分析比较了不同类型心脏淀粉样变性患者的预后，并进行了系统评价。
通过 2018 年 1 月 1 日进行系统文献检索，两位审查员独立提取数据并评估偏倚风险。我们从回归模型中提取 MACE 和死亡终点事件以及风险比，并对多个预后关联研究进行荟萃分析。
我们观察到，转甲状腺素蛋白淀粉样变性和轻链淀粉样变性患者之间存在显著的 MACE 差异（OR：2.09；95%CI：1.06-4.12；P=0.03），在 AL 与 mATTR 或 wtATTR 之间的亚比较中也是如此（AL 与 mATTR：OR：1.72；95%CI：1.06-2.82；P=0.03；AL 与 wtATTR：OR：1.48；95%CI：0.85-2.58；P=0.17）。然而，两种转甲状腺素蛋白类型之间没有观察到显著差异（P=0.17）。总体死亡率评估显示，与转甲状腺素蛋白相关的淀粉样变性相比，轻链型显示出显著差异（P<0.05）。预后分析表明，淀粉样变性类型、LVEF、NYHA、限制性充盈模式、E 波减速时间、E/E'比值和低 QRS 电压是心脏相关死亡率的预测因素。
与转甲状腺素蛋白相关的淀粉样变性相比，诊断为轻链淀粉样变性的患者预后较差，而野生型和突变型 TTR 淀粉样变性的预后分析中未证明存在差异。一些与死亡预后相关的临床因素，如 LVEF、限制性充盈模式、E 波减速时间和 E/E'比值是重要的预后因素。
