ICU心脏淀粉样变性患者的临床特征和预后因素研究

Research of clinical characteristics and prognostic factors in patients with ICU cardiac amyloidosis

目的分析因急性心力衰竭(AHF)进入ICU治疗的轻链蛋白(AL)型心脏淀粉样变性(CA)患者的临床特征和预后,评价AL型CA患者死亡和进展为心源性休克的独立相关因素。方法采用回顾性研究方法选取2012年10月至2018年9月因AHF进入山东省日照市人民医院心内科ICU治疗的AL型CA患者的临床资料。收集患者的人口统计学特征、病史、临床特征、生化指标、超声心动图参数、治疗情况与临床预后。总结AL型CA患者的临床特征及发生心源性休克AL型CA患者的治疗情况,根据患者出院3个月内死亡情况将患者分为存活组与死亡组,采用单变量与多变量Logistics回归分析评价患者出院3个月内死亡的独立相关因素。选取处于临床稳定状态的心源性休克患者为心源性休克组,采用倾向性评分匹配法选取同期治疗且1年内未发生心脏事件的CA患者为对照组,采用单变量与多变量Logistics回归分析评价患者发生心源性休克的独立相关因素。结果纳入的53例AC型CA患者3个月病死率为52.8%(28/53),23例应用多巴酚丁胺的患者有17例死亡,患者应用多巴酚丁胺至死亡时间间隔为(5.3±2.8)d。28例(52.8%)年龄<65岁,多数心源性休克患者(86.2%,25/29)死亡。多变量分析表明,心源性休克(OR=10.329,95%CI1.761~60.712,P<0.001)与血尿素氮(BUN)(OR=1.256,95%CI1.088~1.561,P=0.003)与患者3个月内死亡独立相关。BUN的最佳诊断截点为7.5mmol/L。血清氨基末端B型利钠肽前体(NT-proBNP)(OR=8.707,95%CI1.856~34.693,P=0.020)与AL型CA患者发生心源性休克独立相关。NT-proBNP的最佳诊断截点为4050pg/mL。结论因AHF进入ICU治疗的AL型CA患者具有较高的心源性休克发病率和病死率,年轻患者的病死率更高,NT-proBNP对于评价患者预后具有较高的预测价值,多巴酚丁胺的疗效欠佳,应当积极寻找更好的多巴酚丁胺替代方案进行急救治疗。

Objective To retrospectively analyze the clinical characteristic and prognosis of patients with acute heart failure(AHF)entering the ICU for light-chain cardiac amyloidosis(AL-CA).To evaluate the independent and related factors of death and progression to cardiogenic shock in patients with AL-CA.Methods The clinical data of patients with AL-CA who underwent AHF into the intensive care unit(ICU)of Cardiology of Rizhao Municipal People's Hospital from October 2012 to September 2018 were included.The demographic characteristics,medical history,clinical characteristics,biochemical indicators,echocardiographic parameter,treatment status and clinical prognosis of the patients were collected.The clinical characteristics of AL-CA patients and the treatment of AL-CA patients with cardiogenic shock were analyzed.The patients were divided into survival group and death group according to the death of patients within 3 months after discharge from hospital.Univariate and multivariate logistic regression analysis were used to evaluate the independent factors related to the death of patients within 3 months after discharge.The patients with cardiogenicshock who were in clinical stable state were enrolled in the cardiogenic shock group,and patients with CA who were treated at the same time and had no cardiac events within 1 year were selected as the control group by propensity score matching.Univariate and multivariate logistic regression analysis were used to evaluate independent factors associated with cardiogenic shock in patients.Of the 23 patients who received dobutamine,17 died.Results The time interval from dobutamine to patient death was(5.3±2.8)d.Twenty-eight(52.8%)patients were younger than 65 years old.Most patients with cardiogenic shock(86.2%,25/29)died.Multivariate analysis showed that cardiogenic shock(OR=10.329,95%CI1.761-60.712,P<0.001)and blood urea nitrogen(BUN,OR=1.256,95%CI 1.088-1.561,P=0.003)were independently associated with death within 3 months of the patient.The best diagnostic cut-off value for BUN is 7.5 mmol/L.N-terminal prohormone of B-typenatriuretic peptide(NT-proBNP)(OR=8.707,95%CI 1.856-34.693,P=0.020)was independently associated with cardiogenic shock in AL-CA patients.The best diagnostic cut-off value for serum NT-proBNP is 4050 pg/mL.Conclusion AL-CA patients admitted to ICU for AHF have higher incidence and mortality of cardiogenic shock,and young patients have higher mortality.NT-proBNP has a higher predictive value for evaluating prognosis.The efficacy of phentermamine is poor,and should be actively looking for better dobutamine replacement for first aid treatment.