特发性肺动脉高压39例临床分析

对39例确诊为特发性肺动脉高压(IPAH)患者的病例资料进行回顾性分析.结果显示患者均有肺动脉压力增高和心功能不全表现,辅助检查提示有明显的肺动脉高压征象.认为IPAH早期缺乏特异性临床表现,临床容易漏诊和误诊,心导管检查是确诊的可靠依据,早期诊断及治疗是决定预后的关键.     

特发性肺动脉高压的诊断与治疗

特发性肺动脉高压(Idiopathic Pulmonary ArterialHypertension,IPAH)是指没有明确原因呈单克隆细胞增生和致丛性病变的肺动脉高压.早期诊断和及时治疗有助于改善IPAH患者的预后,因此综合分析患者的个人史、家族史、临床表现、体格检查和实验室检查,进行规范的IPAH诊断评估,进而明确诊断和规范治疗,对于提高IPAH患者的生存期和改善生活质量具有十分重要的意义.     

特发性肺动脉高压的临床和血液动力学分析

目的:初步分析特发性肺动脉高压(IPAH)患者的临床特征和血流动力学特点。方法:分析2008年3月至2011年3月,北京世纪坛医院住院的27例IPAH患者的临床资料,所有患者均通过静息状态下超声心动图及右心导管检查确诊,并进行PAH功能分级及6 min步行距离检查。结果:患者首发症状至确诊时间为(34.16±39.85)个月,74%PAH患者确诊时WHO功能分级为III-IV级;男女两性之比1:4,6 min步行距离平均(363.04±101.48)m,肺动脉平均压(64.60±16.28)mmHg(1 mmHg=0.133kPa),心脏指数(3.12±1.22)L.min-1.m-2,右心房平均压(10.40±4.78)mmHg,肺血管阻力(18.04±4.65)Wood单位,N末端脑钠肽前体(NT-proBNP)测定值为(3225.26±728.5)ng/L,超声心动图测得肺动脉收缩压及经右心导管检查测得肺动脉收缩压,分别为(88.00±22.67)mmHg,(94.89±30.24)mmHg,二者相关性(r=0.795,P<0.01)。结论:本组PAH的人口学特点与NIH注册研究接近,大多数患者确诊时已出现功能受损的严重症状,Ⅲ/Ⅳ级患者其血流动力学指标较Ⅰ/Ⅱ级患者明显恶化,建议应用超声心动图检查、心功能分级、6 min步行距离及肺血流动力学参数的变化,来对IPAH进行筛查及病情评估、治疗效果评价及病情恶化的监测,可以做到早期发现、早期干预。     

特发性肺纤维化与肺动脉高压

特发性肺纤维化（idiopathic pulmonary fibrosis,IPF）是指原因不明并以普通型间质性肺炎（usual interstitial pneumonia,UIP）为特征性病理改变的一种慢性炎症性间质性疾病,主要表现为弥漫性肺泡炎、肺泡单位结构紊乱和肺纤维化。病情多呈进行性发展,晚期可出现发绀、肺动脉高压（pulmonary arterial hypertension, PAH）、肺源性心脏病、右心功能不全等。     

特发性肺动脉高压的诊断

特发性肺动脉高压是一原因不明的肺血管阻力增加的少见疾病,预后险恶。近年由于治疗的进展,患者的生存质量改善,生存时间延长,再也不是一种不可治疗的疾病。然而．治疗效果和预后与治疗的早晚密切相关,因此早期诊断就显得十分重要。特发性肺动脉高压的诊断基本采用排除诊断法,需除外一切继发性肺动脉高压方可成立。本文在诊断和鉴别诊断方面做了介绍,希望对临床工作能有所帮助。     

儿童先天性心脏病术后肺动脉高压与特发性肺动脉高压的临床特点及预后

目的:因特发性肺动脉高压(IPAH)及先天性心脏病术后肺动脉高压(CD)患者均不存在心内分流,解剖、病生理及血流动力学方面存在相似性,描述分析两类患者的临床特征、功能分级、中远期结局,并分析影响预后的因素。方法:回顾性分析2005年6月至2018年7月,于北京安贞医院小儿心脏中心住院的儿童患者的预后转归情况。结果:共入组儿童222例,包括123例IPAH和99例CD患者。诊断中位年龄为11.2(7.4, 14.2)岁。末次随访时共160例(72.1%)患者接受PAH靶向药物治疗,68例(30.1%)患者应用联合治疗。CD患者的心功能及运动能力优于IPAH患者。两组患者血流动力学参数相似,IPAH的肺血管阻力指数较低,心指数稍高。随访中位时间为3.9(1.9, 6.2)年,随访期间44例患者死亡(IPAH 39例,CD 5例),右心衰竭和心源性猝死为最常见死亡原因。IPAH和CD患儿1年、3年及5年的生存率分别为89.2%,74.7%,63.2%及95.7%,95.7%,93.7%(HR=4.2,95%CI:2.3～7.6,P<0.0001)。结论:儿童IPAH和CD患者中远期预后有显著差异。死亡率在IPAH患儿中较高,且与较晚的诊断年龄有关。CD患儿的预后情况与基线状态下心功能分级相关。诊断后早期积极应用靶向药物治疗对预后有益。     

特发性肺动脉高压4例

例1：女性,25岁,呼吸困难3月。右心导管测量肺动脉压97/56（68）mmHg,肺循环阻力2623达因·s·cm^-5·m^2,心脏指数2.0 L·min^-1·m^-1,腺苷急性肺血管扩张试验阴性;6 min步行距离421m,Borg scale呼吸困难分级5级。例2：男性,59岁,呼吸困难2年。右心导管测量肺动脉压83/46（56）mmHg,肺循环阻力2502达因·s·cm^-5·m^2,心脏指数1.8 L·min^-1·m^m-1,腺苷急性肺血管扩张试验阴性;6 min步行距离221 m,     

特发性肺动脉高压的药物治疗进展

特发性肺动脉高压(IPAH)是指原因不明的肺血管阻力增加引起持续性肺动脉压力升高,其病理改变为肺血管的收缩和重塑、原位血栓形成和血管内皮损伤。由于缺乏有效的治疗措施,病死率极高,预后差,明确诊断后平均存活时间为2~3年。近年来随着对IPAH的分子生物学水平研究的深入,一些新的治疗方法如前列环素及其衍生物、内皮素受体拮抗剂等不断应用于临床,使IPAH的生存率明显提高,生活质量明显改善。而一些被证明对IPAH具有潜在治疗意义的药物如肾上腺髓质素、西地那非、他汀类药物等仍在临床研究中,在不久的将来可能成为治疗IPAH的一线用药。     

特发性肺动脉高压患者睡眠结构特点分析

【摘要】 目的 探讨特发性肺动脉高压（IPAH）患者睡眠结构特点。方法 选择2015年2～6月于北京世纪坛医院肺血管疾病组就诊的女性特发性肺动脉高压患者15例,进行右心导管、肺功能、多导睡眠仪监测、6 min步行试验、动脉血气分析等检查。同期选择20例排除肺动脉高压的女性健康者为对照组。结果 ①特发性肺动脉高压患者呼吸暂停低通气指数（AHI）为（1.2±2.2）次/h,对照组为（0.3±0.5）次/h,两组之间比较未见统计学差异（P＞0.05）,患者组各睡眠分期占睡眠期时间比例及各睡眠期觉醒次数与正常对照组比较未见统计学差异（P＞0.05）。②患者组夜间最低脉搏血氧饱和度（LSpO2）水平为（86.40±4.2）%、夜间平均脉搏血氧饱和度（MSpO2）为（90.51±3.4）%,脉搏血氧饱和度小于90%的累积时间占总睡眠时间的百分比（%TRT SpO2＜90%）为（36.67±38.3）%,对照组分别为（93.14±3.3）%,（97.33±0.5）%,（0.03±0.1）%,肺动脉高压组与正常对照组比较差异有统计学意义（P＜0.05）。③患者组非快动眼睡眠（NREM）N1、N2、N3各期的夜间平均脉搏血氧饱和度、平均呼吸频率与正常对照组比较差异有统计学意义（P＜0.05）。④N2和N3期平均呼吸频率与CO呈负相关（t=-0.652、-0.657,P＜0.05）,夜间LSpO2与CO、CI呈负相关（t=-0.413、-0.346,P＜0.05）。结论 特发性肺动脉高压患者睡眠结构正常,无睡眠呼吸紊乱,但存在显著的夜间低氧血症,其与进行性发展的肺高压和右心功能不全有密切关系。     

成人特发性肺动脉高压患者临床特点及预后分析

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。     

特发性肺动脉高压患者血清骨保护素及其受体的变化及临床意义

目的探讨特发性肺动脉高压（idiopathic pulmonary arterial hypertension,IPAH）患者肺动脉压力和血清骨保护素（osteoprotegerin,OPG）及其受体（核因子-κβ受体活化因子配体,RANKL）水平的关系及临床意义。方法纳入IPAH患者28例,同期纳入性别和年龄匹配的健康体检者28例作为对照组。采用酶联免疫吸附法（ELISA）检测所有入组人员血清OPG/RANKL水平、N末端脑钠肽前体（NT-proBNP）水平;对IPAH组患者采用右心漂浮导管测定肺动脉压力的水平,并对肺动脉压和OPG/RANKL水平进行相关性分析。结果与正常对照组相比,IPAH患者血清中OPG、RANKL和NT-proBNP水平均更高[OPG：（190.91±43.39）pg/mL vs.（122.59±41.20）pg/mL;RANKL：（194.05±50.31）pg/mL vs.（117.73±39.89）pg/mL;NT-proBNP：（1894.78±591.97）pg/mL vs.（224.18±60.11）pg/mL],差异均有统计学意义（P＜0.05）。直线相关分析显示血清中OPG/RANKL水平和肺动脉压力呈正相关,相关系数分别为r=0.525（P＜0.05）和r=0.419（P＜0.05）。结论骨保护素及其受体轴（OPG/RANKL）可能参与肺动脉高压的形成,且与肺动脉压力有一定的相关性。     

Long-term survival in idiopathic pulmonary arterial hypertension associated with massive pulmonary artery dilation

The present report describes two patients with long-term survival after being diagnosed with idiopathic pulmonary arterial hypertension more than 20 years earlier. Both patients were treated with calcium channel blockers for several years and are currently maintained on bosentan, an oral endothelin receptor antagonist. Severe dilation of the main pulmonary arteries is present in both patients and may be related to long-term survival with idiopathic pulmonary arterial hypertension.     

Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension

Clinical aspects and pathology of pulmonary hypertension (PH) in patients with systemic lupus erythematosus (SLE) have been reported to be similar to those in patients with idiopathic pulmonary arterial hypertension (IPAH). To determine whether PH in these patients is similar, we compared the clinical characteristics, hemodynamics at diagnosis, and survival in groups of patients with SLE–PH and IPAH. We reviewed the case records of 20 patients with SLE–PH and 34 patients with IPAH, who had been assessed by echocardiography or right cardiac catheterization at Asan Medical Center, Seoul, Korea, from January 1995 to October 2003. Clinical features, laboratory data, chest X-rays, electrocardiogram results, pulmonary function tests, pulmonary perfusion scans, echocardiographic findings, serologic profiles, and survival were compared in the two groups of patients. The mean follow-up period was 18.1±20.6 months for patients with SLE–PH and 33.0±23.4 months for patients with IPAH. During follow-up, 12 SLE–PH (60%) and 11 IPAH (32%) patients died. For SLE–PH, the 3-year survival rate was 44.9% and the 5-year survival rate was 16.8%. For IPAH, the 3-year survival rate was 73.4% and the 5-year survival rate was 68.2% (p=0.02). There were no other significant differences in clinical characteristics and laboratory data between the two groups. In contrast to previous reports that the prognosis of patients with pulmonary arterial hypertension associated with collagen vascular disease was better than that of patients with IPAH, we found that the prognosis of patients with SLE–PH was much worse than that of patients with IPAH.     

肺血管内皮细胞功能障碍与肺动脉高压

肺动脉高压的发病机制是一个复杂的、多因素参与的过程.近年来,逐步形成内皮功能紊乱学说,认为肺血管内皮是引起血管收缩和特征性病理改变的首要因素.本文就肺血管内皮细胞功能障碍和肺动脉高压的研究进展作一综述.     

特发性肺纤维化相关性肺高压的诊断与治疗

肺高压(pulmonary hypertension,PH)作为特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)的严重并发症,预后极差.其发病机制目前尚未完全阐明,PH的发生、发展严重降低了IPF患者的生活质量并缩短了患者的生存期.但早期诊断及合理治疗可提高该病患者的生存率并改善患者的生存质量.近年,对IPF相关性PH的认识不断深入,现将研究的最新进展作一综述.     

Determinants of pulmonary arterial hypertension in scleroderma

OBJECTIVE: To define risk factors associated with pulmonary arterial hypertension (PAH) in a large cohort of patients with systemic sclerosis (SSc). METHODS: SSc patients undergoing screening for PAH by means of Doppler echocardiography were identified and their charts were retrospectively reviewed. In all patients, we recorded systolic pulmonary artery pressure along with pulmonary function testing, clinical, and laboratory data. PAH was defined as right ventricular systolic pressure equal or greater than 40 mm Hg. RESULTS: Of 114 SSc patients with echocardiographic measurements, PAH was found in 33 (29%) patients. In a multiple logistic regression analysis, the presence of pulmonary fibrosis on thoracic computed tomography (OR 6.78, CI 1.54 to 29.9), forced vital capacity less than 80% predicted (OR 3.03, CI 1.1 to 8.35), and duration of Raynaud's phenomenon preceding the onset of skin changes for at least 3 years (OR 5.75, CI 1.9 to 17.41) were found to be independent predictors of PAH. Age, disease duration, disease subtype, or autoantibodies were not associated with PAH in our patients. CONCLUSIONS: The present analysis identified pulmonary fibrosis and Raynaud's phenomenon preceding SSc skin manifestations by at least 3 years as risk factors for PAH in our scleroderma cohort. Screening for PAH in these high-risk patients may detect PAH at an earlier stage and guide decisions on therapeutic interventions.