成人特发性肺动脉高压患者临床特点及预后分析

发布时间：2018-07-05 14:52

  本文选题：特发性肺动脉高压 + 临床特点　； 参考：《临床心血管病杂志》2018年01期

【摘要】：目的:探讨成人特发性肺动脉高压(IPAH)临床特点及相关预后因素。方法:对广西医科大学第一附属医院2009-10-2016-04住院的24例成年IPAH患者临床资料进行回顾性分析。随访至2017年4月,用COX生存分析进行预后因素分析。结果:(1)24例IPAH患者平均年龄(35.33±13.93)岁,男女比1∶3.8;(2)几乎所有患者出现劳力性气促的临床表现,晕厥、胸痛、咯血、声音嘶哑的发生率分别为33.3%、8.3%、20.8%、12.5%;(3)所有患者均有P2亢进,50%的患者可闻及三尖瓣听诊区收缩期杂音;(4)70.8%的IPAH患者世界卫生组织肺动脉高压功能分级(WHO-FC)为Ⅲ~Ⅳ级,6分钟步行距离(6MWD)为(396.91±53.14)m,Borg呼吸困难指数范围为0~5(中位数为3);(5)尿酸(UA)、氨基末端脑钠肽前体(NT-proBNP)、红细胞体积分布宽度(RDW)平均值分别为(447.40±151.11)μmol/L、(2 548.40±1 256.10)pg/ml、(15.50±2.30)%,且均高于正常上限值。WHO-FCⅢ/Ⅳ级IPAH患者UA及NT-proBNP浓度明显高于WHO-FC I/Ⅱ级IPAH患者;(6)心电图以右室肥大为主要表现,经胸心脏超声心动图检查右心室明显增大,肺动脉压力明显升高;(7)右心导管检查平均肺动脉压(mPAP)和肺血管阻力(PVR)的平均值分别为(58.6±12.9)mmHg、(16.1±6.5)WU。(8)平均随访(29.2±21.3)个月,8例死亡,出院后1、2、3、4年的生存率分别为79.1%、70.8%、70.8%、66.6%。单因素COX分析示6MWD(P=0.025,RR=7.7×1015)、NT-proBNP(P=0.011,RR=1.001)、RDW(P=0.011,RR=1.001)、UA(P=0.09,RR=1.002)对IPAH生存有影响。进一步多因素COX分析结果示NT-proBNP(P=0.010,RR=1.002)和RDW(P=0.032,RR=10×1018)是IPAH患者独立预后因素。结论:成人IPAH好发青年女性;以劳力性气促为主要临床症状,可有晕厥、胸痛、咯血、声音嘶哑等;所有患者均有P2亢进,50%的患者可闻及三尖瓣听诊区收缩期杂音;患者存在不同程度右室增大,并且有肺动脉压及肺血管阻力明显增高的血流动力学特征;患者预后不良,病死率高,NT-proBNP和RDW是成人IPAH患者的独立预后因素。
[Abstract]:Objective: to investigate the clinical features and prognostic factors of adult idiopathic pulmonary hypertension (IPAH). Methods: the clinical data of 24 adult IPAH patients in the first affiliated Hospital of Guangxi Medical University 2009-10-2016-04 were retrospectively analyzed. Follow-up to April 2017, Cox survival analysis for prognostic factors. Results: (1) the average age of 24 patients with IPAH was (35.33 卤13.93) years, the ratio of male and female was 1: 3.8; (2) almost all the patients had the clinical manifestations of exertional dyspnea, syncope, chest pain, hemoptysis, The incidence of hoarseness was 33.3and 8.3R 20.8T 12.5m; (3) 50% of all patients had P2 hyperactivity and systolic murmur in tricuspid auscultation area; (4) 70.8% IPAH patients with World Health Organization Pulmonary Hypertension (WHO-FC) were in grade 鈪，

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