本文選題：特發(fā)性肺動(dòng)脈高壓 + 臨床特點(diǎn)　； 參考：《臨床心血管病雜志》2018年01期

【摘要】：目的:探討成人特發(fā)性肺動(dòng)脈高壓(IPAH)臨床特點(diǎn)及相關(guān)預(yù)后因素。方法:對(duì)廣西醫(yī)科大學(xué)第一附屬醫(yī)院2009-10-2016-04住院的24例成年IPAH患者臨床資料進(jìn)行回顧性分析。隨訪至2017年4月,用COX生存分析進(jìn)行預(yù)后因素分析。結(jié)果:(1)24例IPAH患者平均年齡(35.33±13.93)歲,男女比1∶3.8;(2)幾乎所有患者出現(xiàn)勞力性氣促的臨床表現(xiàn),暈厥、胸痛、咯血、聲音嘶啞的發(fā)生率分別為33.3%、8.3%、20.8%、12.5%;(3)所有患者均有P2亢進(jìn),50%的患者可聞及三尖瓣聽(tīng)診區(qū)收縮期雜音;(4)70.8%的IPAH患者世界衛(wèi)生組織肺動(dòng)脈高壓功能分級(jí)(WHO-FC)為Ⅲ~Ⅳ級(jí),6分鐘步行距離(6MWD)為(396.91±53.14)m,Borg呼吸困難指數(shù)范圍為0~5(中位數(shù)為3);(5)尿酸(UA)、氨基末端腦鈉肽前體(NT-proBNP)、紅細(xì)胞體積分布寬度(RDW)平均值分別為(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級(jí)IPAH患者UA及NT-proBNP濃度明顯高于WHO-FC I/Ⅱ級(jí)IPAH患者;(6)心電圖以右室肥大為主要表現(xiàn),經(jīng)胸心臟超聲心動(dòng)圖檢查右心室明顯增大,肺動(dòng)脈壓力明顯升高;(7)右心導(dǎo)管檢查平均肺動(dòng)脈壓(mPAP)和肺血管阻力(PVR)的平均值分別為(58.6±12.9)mmHg、(16.1±6.5)WU。(8)平均隨訪(29.2±21.3)個(gè)月,8例死亡,出院后1、2、3、4年的生存率分別為79.1%、70.8%、70.8%、66.6%。單因素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)對(duì)IPAH生存有影響。進(jìn)一步多因素COX分析結(jié)果示NT-proBNP(P=0.010,RR=1.002)和RDW(P=0.032,RR=10×1018)是IPAH患者獨(dú)立預(yù)后因素。結(jié)論:成人IPAH好發(fā)青年女性;以勞力性氣促為主要臨床癥狀,可有暈厥、胸痛、咯血、聲音嘶啞等;所有患者均有P2亢進(jìn),50%的患者可聞及三尖瓣聽(tīng)診區(qū)收縮期雜音;患者存在不同程度右室增大,并且有肺動(dòng)脈壓及肺血管阻力明顯增高的血流動(dòng)力學(xué)特征;患者預(yù)后不良,病死率高,NT-proBNP和RDW是成人IPAH患者的獨(dú)立預(yù)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 鹵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，

本文編號(hào)：2100586