2022 年第 1 期 第 17 卷

原发性轻链型心肌淀粉样变性患者预后的影响因素

Prognostic factors of patients with primary light-chain cardiac amyloidosis

作者：辛亮草郝从青刘源

英文作者：Xin Liangcao Hao Congqing Liu Yuan

单位：郑州大学第一附属医院心血管内科，郑州450052

英文单位：Department of Cardiology the First Affiliated Hospital of Zhengzhou University Zhengzhou 450052 China

关键词：心肌淀粉样变性；每搏输出量指数；左心室质量指数；心脏磁共振成像

英文关键词：Cardiacamyloidosis;Strokevolumeindex;Leftventricularmassindex;Cardiovascularmagneticresonance

• 摘要：

• 目的 探讨原发性轻链型心肌淀粉样变性（AL-CA）患者预后的影响因素。方法 回顾性分析郑州大学第一附属医院2015年8月至2021年1月收治的56例原发性AL-CA患者的临床资料。收集患者的性别、年龄和心脏磁共振成像（CMR）晚期钆增强（LGE）检查相关参数。通过门诊随访或电话随访，随访截止时间为2021年6月1日。比较不同预后组患者的性别、年龄和CMR LGE检查相关参数，分析影响原发性AL-CA患者生存的危险因素，评价筛查出的危险因素对AL-CA患者生存率的影响。结果 56例患者中死亡24例（死亡组），存活32例（存活组）。死亡组中位左心室质量指数（LVMI）高于存活组［90.0(78.4，108.8)g/m2 比71.4(52.4，98.1)g/m2］（P＜0.05）；2组性别、年龄、室间隔最大厚度、左心室壁最大厚度、左心房前后径、左心室舒张末期容积、左心室射血分数、每搏输出量指数（SVI）、心脏指数，瓣膜反流、心包积液、胸腔积液和LGE强化比例比较差异均无统计学意义（均P＞0.05）。多因素Cox比例风险分析结果显示，SVI＜29 ml/m2、女性LVMI＞98.5 g/m2和男性LVMI＞103.0 g/m2为影响AL-CA患者生存的危险因素（风险比=3.07、3.56,95%置信区间：1.22~7.72、1.34~9.43,P=0.017、0.011）。低SVI（＜29 ml/m2）组患者的生存率低于高SVI（≥29 ml/m2）组（P=0.001），高LVMI（女性＞98.5 g/m2和男性＞103.0 g/m2）组的生存率低于低LVMI（女性≤98.5 g/m2和男性≤103.0 g/m2）组（P=0.018）。结论  SVI下降和LVMI升高均为原发性AL-CA患者预后的危险因素。

• Objective   To investigate the prognostic factors of patients with primary light-chain myocardial amyloidosis (AL-CA). MethodThe clinical data of 56 patients with primary AL-CA admitted to the First Affiliated Hospital of Zhengzhou University from August 2015 to January 2021 were retrospectively analyzed. The gender, age and cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) parameters of patients were collected. Through outpatient follow-up or telephone follow-up, the deadline for follow-up was June 1, 2021. The gender, age and CMR LGE examination related parameters of patients were compared, the risk factors affecting the survival of patients with AL-CA were analyzed, and the impact of the screened risk factors on the survival rate of patients with AL-CA was evaluated. Results  Among 56 patients, 24 died (death group) and 32 survived (survival group). The median left ventricular myocardial mass index (LVMI) in the death group was higher than that in the survival group ［90.0(78.4，108.8)g/m2 vs 71.4(52.4, 98.1)g/m2］ (P<0.05). There were no significant differences in gender, age, maximum thickness of ventricular septum, maximum thickness of left ventricular wall, left atrial diameter, left ventricular end diastolic volume, left ventricular ejection fraction, stroke output index(SVI), cardiac index, and proportions of valve reflux, pericardial effusion, pleural effusion and LGE enhancement (all P>0.05). Multivariate Cox proportional hazards analysis showed that SVI<29 ml/m2, female LVMI >98.5 g/m2 and male LVMI >103.0 g/m2 were the risk factors affecting the survival of patients with AL-CA (hazard ratio=3.07, 3.56, 95% confidence interval: 1.22-7.72, 1.34-9.43, P=0.017,0.011). The survival rate of patients in low SVI group (<29 ml/m2) was lower than that of patients in high SVI group (≥29 ml/m2) (P=0.001), and the survival rate of patients in high LVMI group (female >98.5 g/m2 and male >103.0 g/m2) was lower than that of patients in low LVMI group (female ≤98.5 g/m2 and male ≤103.0 g/m2) (P=0.018). Conclusion  The decrease of SVI and the increase of LVMI are risk factors for the prognosis of patients with AL-CA.