未合并易栓症的慢性血栓栓塞性肺动脉高压患者的预后影响因素分析

Prognostic Influencing Factors of Chronic Thromboembolic Pulmonary Hypertension Patients without Thrombophilia

背景国内外目前尚缺乏未合并易栓症的慢性血栓栓塞性肺动脉高压(CTEPH)患者预后影响因素的研究。目的分析未合并易栓症的CTEPH患者预后的影响因素。方法选取中国医学科学院阜外医院血栓性疾病诊治中心2013年6月—2019年6月收治的未合并易栓症的CTEPH患者279例为研究对象。收集患者的一般资料、临床化验指标和右心导管检查结果、治疗情况。对所有患者进行电话或门诊随访。根据预后将患者分为存活组和死亡组。未合并易栓症的CTEPH患者预后的影响因素分析采用多因素Cox回归分析。结果共纳入未合并易栓症的CTEPH患者279例,随访时间为1~84个月,中位随访时间为24个月,删失7例(2.5%),最终纳入患者272例。随访结束时,队列的总生存概率为91.2%(248/272)。死亡组出现症状到诊断时间长于存活组,6 min步行距离短于存活组(P<0.05)。死亡组N末端脑钠肽前体(NT-proBNP)、右心房压(RAP)、肺血管阻力(PVR)高于存活组,动脉血氧饱和度(SaO_(2))、混合静脉血氧饱和度(SVO_(2))、心指数(CI)低于存活组(P<0.05)。死亡组单独靶向药物治疗率高于存活组,单独经皮肺血管成形术(PTPA)率、单独肺动脉血栓内膜剥脱术(PEA)率低于存活组(P<0.05)。多因素Cox回归分析结果显示,SaO_(2)≥60%〔HR=0.356,95%CI(0.139,0.916)〕、血运重建〔HR=0.089,95%CI(0.028,0.279)〕是未合并易栓症的CTEPH患者死亡的保护因素,而出现症状到诊断时间≥25个月〔HR=1.009,95%CI(1.002,1.016)〕是未合并易栓症的CTEPH患者死亡的危险因素(P<0.05)。结论较高的SaO_(2)和血运重建是未合并易栓症的CTEPH患者死亡的保护因素,而出现症状到诊断时间延长是其死亡的危险因素,早期诊断和积极的血运重建有望进一步改善未合并易栓症的CTEPH患者的长期预后。

Background At present,there is a lack of research on prognostic influencing factors of chronic thromboembolic pulmonary hypertension(CTEPH)patients without thrombophilia at home and abroad.Objective To analyze the prognostic influencing factors of CTEPH patients without thrombophilia.Methods A total of 279 CTEPH patients without thrombophilia admitted to Thrombosis Center,Fuwai Hospital,CAMS&PUMC from June 2013 to June 2019 were selected as the research objects.General information,clinical laboratory indexes,right heart catheterization results and treatment were collected.All patients were followed up by telephone or outpatient,and ccording to the prognosis,the patients were divided into survival group and death group.Multivariate Cox regression analysis was used to analyze the prognostic influencing factors of CTEPH patients without thrombophilia.Results A total of 279 CTEPH patients without thrombophilia were included.During the median follow-up of 24 months(1-84 months),7 cases(2.5%)were lost to follow-up and 272 patients were finally included.At the end of follow-up,the overall survival rate of the cohort was 91.2%(248/272).The time from symptom to diagnosis of the death group was longer than that of the survival group,and the 6 min walking distance was shorter than that of the survival group(P<0.05).N-terminal pro-brain natriuretic peptide(NT-proBNP),right atrial pressure(RAP)and pulmonary vascular resistance(PVR)in the death group were higher than those in the survival group,however,arterial oxygen saturation(SaO_(2)),mixed venous oxygen saturation(SVO_(2))and cardiac index(CI)were lower than those in the survival group(P<0.05).The rate of targeted drug therapy alone in the death group was higher than that in the survival group,and the rates of percutaneous transluminal pulmonary angioplasty(PTPA)and pulmonary endarterectomy(PEA)alone in the death group were lower than those in the survival group(P<0.05).Multivariate Cox regression analysis showed that SaO_(2)≥60%〔HR=0.356,95%CI(0.139,0.916)〕and revascularization〔HR=0.089,95%CI(0.028,0.279)〕were protective factors for death of CTEPH patients without thrombophilia,while the time from symptom to diagnosis≥25 months〔HR=1.009,95%CI(1.002,1.016)〕was a risk factor for death of CTEPH patients without thrombophilia(P<0.05).Conclusion Higher SaO_(2) and revascularization are protective factors for death of CTEPH patients without thrombophilia,while prolonged time from symptom to diagnosis is an independent risk factor for death of these patients.Early diagnosis and active revascularization are expected to further improve the long-term prognosis of CTEPH patients without thrombophilia.