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特发性肺动脉高压 Idiopathic Pulmonary Arterial Hypertension

作者:大江 | 时间:2019-2-24 00:00:13 | 阅读:641| 显示全部楼层
Pulmonary hypertension.png
动脉高压

肺动脉高压(PH或PHTN)是肺动脉内血压升高的一种疾病[6]。症状包括呼吸急促,晕厥,疲倦,胸痛,腿部肿胀,心跳加快。[6] [2]这种情况可能使其难以锻炼。[6]发作通常是渐进的。[7]

原因往往是未知的。[1]风险因素包括家族史,肺部先前的血栓,HIV / AIDS,镰状细胞病,可卡因使用,COPD,睡眠呼吸暂停,高海拔生活以及二尖瓣问题。[4] [3]潜在的机制通常涉及肺部动脉的炎症。[4]诊断包括首先排除其他潜在原因。[1]

没有治愈方法。[6]治疗取决于疾病的类型。[5]可以使用一些支持性措施,如氧疗,利尿剂和抑制凝血的药物。[1]特别针对该病症的药物包括依前列醇,曲前列环素,伊洛前列素,波生坦,安立生坦,马西替坦和西地那非。[1]在某些情况下,肺移植可能是一种选择。[5]

虽然病情的确切频率未知,但据估计,美国每年约有1,000例新病例发生。[3] [2]女性比男性更容易受到影响。[2]发病年龄通常在20至60岁之间。[3]它最初由Ernst von Romberg在1891年发现。[8] [1]

目录
1 症状和体征
2 原因
2.1 分类
2.2 遗传学
3 发病机制
3.1 分子病理
4 诊断
4.1 体检
4.2 超声心动图
4.3 排除其他疾病
4.4 CT扫描
4.5 右心导管插入术
4.6 其他
5 治疗
5.1 血管活性物质
5.2 手术
5.3 监测
6 预后
7 流行病学
8 值得注意的案例
9 研究
10 参考文献

体征和症状
肺动脉高压的症状包括:[9] [10] [11]

呼吸急促
疲劳
胸痛
心悸(心跳率增加)
疼痛(腹部右侧)
胃口不好
头晕
昏厥或晕厥
肿胀(腿/脚踝)
发绀
不太常见的体征/症状包括非生产性咳嗽和运动引起的恶心和呕吐。[10]某些患者可能会出现咳血,特别是那些患有肺动脉高压特定亚型的患者,如遗传性肺动脉高压,艾森曼格综合征和慢性血栓栓塞性肺动脉高压[12]。肺静脉高压通常表现为平躺或睡眠时呼吸短促(端坐呼吸或阵发性夜间呼吸困难),而肺动脉高压(PAH)通常不会。[13]

肺动脉高压的其他典型体征包括第二心音的强调肺部分,右心室第三心音和胸骨旁隆起,表明肥大的右心房。右侧心力衰竭引起的全身性充血迹象包括颈静脉扩张,腹水和肝颈静脉反流[10] [11] [14]。还寻求三尖瓣关闭不全和肺动脉反流的证据,并且如果存在,则与肺动脉高压的存在一致[10] [11] [15]。

原因
肺动脉高压是一种病理生理状况,有许多可能的原因。实际上,这种情况经常伴随着严重的心脏或肺部疾病。[10] 1973年世界卫生组织会议首次尝试按其原因对肺动脉高压进行分类,并对原发性PH(由肺动脉疾病引起)和继发性PH(继发于其他非血管原因)进行了区分。 。此外,原发性PH分为“动脉丛状”,“静脉闭塞”和“血栓栓塞”形式。[16] 1998年,在évian-les-Bains举行的第二次会议讨论了二级PH的原因。[17]随后的第三届,[18]第四届,[19]和第五届(2013年)[20]世界PAH专题讨论会进一步确定了PH的分类。基于对疾病机制的更好理解,分类继续发展。[需要引证]

最近在2015年,WHO指南由欧洲心脏病学会(ESC)和欧洲呼吸学会(ERS)更新。[10]这些指南得到了国际心肺移植学会的认可,并为理解和治疗肺动脉高压提供了现有框架。[21]

分类
根据WHO分类,有5组PH,其中I组(肺动脉高压)进一步细分为I组和I组。[19] [20]最新的世界卫生组织分类系统(最近以斜体显示的最新ESC / ERS指南进行了调整)可归纳如下:[20] [10]

世卫组织第一组 - 肺动脉高压(PAH)

特发性
遗传(BMPR2,ALK1,SMAD9,caveolin 1,KCNK3突变)
药物和毒素诱导(例如使用甲基苯丙胺)
相关病症:结缔组织病,HIV感染,门静脉高压症,先天性心脏病,血吸虫病
WHO I组 - 肺静脉闭塞性疾病(PVOD),肺毛细血管血管瘤病(PCH)

特发性
遗传(EIF2AK4突变)
药物,毒素和辐射诱发
相关病症:结缔组织病,HIV感染
世卫组织第一组“ - 新生儿持续性肺动脉高压

世卫组织第II组 - 继发于左心病的肺动脉高压

左心室收缩功能障碍
左心室舒张功能障碍
心脏瓣膜病
先天性/后天性左心流入/流出道梗阻和先天性心肌病
先天性/后天性肺静脉狭窄
WHO III组 - 由肺病,慢性缺氧引起的肺动脉高压

慢性阻塞性肺病(COPD)
间质性肺病
混合限制性和阻塞性模式肺病
睡眠呼吸紊乱
肺泡通气不足
慢性接触高海拔地区
发育异常
世界卫生组织第IV组 - 慢性动脉阻塞

慢性血栓栓塞性肺动脉高压(CTEPH)
其他肺动脉阻塞
血管瘤中的血管肉瘤或其他肿瘤
动脉炎
先天性肺动脉狭窄
寄生虫感染(包虫病)
世卫组织第V组 - 肺动脉高压不明确或多因素机制

血液系统疾病:慢性溶血性贫血(包括镰状细胞病)
系统性疾病:结节病,肺朗格汉斯细胞组织细胞增多症:淋巴管平滑肌瘤病,神经纤维瘤病,血管炎
代谢紊乱:糖原贮积病,戈谢病,甲状腺疾病
其他:肺肿瘤血栓性微血管病变,纤维化纵隔炎,慢性肾功能衰竭,节段性肺动脉高压(肺动脉高压局限于肺部的一个或多个肺叶)
遗传学
几种基因的突变与这种情况有关[22] [23]这些包括骨形态发生蛋白受体2型(BMPR2)和真核翻译起始因子2α激酶4基因(EIF2AK4)。

发病

Right ventricle (on left side).gif
右心室(左侧)

Micrograph showing arteries in pulmonary hypertensive with marked thickening of .jpg
显微照片显示肺动脉高压的动脉,壁明显增厚。
肺动脉高压的发病机制(WHO I组)涉及连接肺部和肺部的血管变窄。这使得心脏更难以将血液泵入肺部,就像更难以使水流过狭窄的管道而不是宽的管道一样。随着时间的推移,受影响的血管在称为纤维化的过程中变得更硬和更厚。这种缩小过程涉及的机制包括血管收缩,血栓形成和血管重塑(过度细胞增殖,纤维化,血管壁细胞凋亡/程序性细胞死亡减少,由炎症,代谢紊乱和某些生长因子失调引起)[24]。 ] [25]随着时间的推移,血管重塑导致受影响的血管逐渐变硬和变厚。这进一步增加了肺内的血压并损害了它们的血液流动。与其他类型的肺动脉高压一样,这些变化导致心脏右侧的工作量增加。[11] [26]右心室通常是低压系统的一部分,收缩期心室压低于左心室通常遇到的压力。因此,右心室不能应对更高的压力,虽然右心室适应(肥大和心肌收缩力增加)最初有助于保持每搏输出量,但最终这些补偿机制是不充分的;右心室肌肉无法获得足够的氧气来满足其需要,随后出现右心衰竭。[11] [25] [26]随着流经肺部的血液减少,心脏的左侧接收的血液减少。这种血液也可以携带比正常情况更少的氧气。因此,心脏左侧泵送以向身体其他部位供应足够的氧气变得越来越困难,特别是在身体活动期间。[27] [28] [19]

在PVOD(WHO组1')中,肺血管狭窄在毛细血管后静脉血管中优先(但不是唯一地)发生[29]。 PVOD与PAH有几个共同特征,但也存在一些重要差异,例如预后差异和对药物治疗的反应。[引证需要]

当新生儿的循环系统不能适应子宫外的生活时,新生儿持续存在肺动脉高压;它的特点是对肺部血流的高度抵抗,从右到左的心脏分流和严重的低氧血症。[11]

左心病引起的肺动脉高压的发病机制(WHO II组)完全不同,因为肺血管的收缩或损伤不是问题。相反,左心脏不能有效地泵血,导致肺部血液汇集和肺部系统内的背压。这会导致肺水肿和胸腔积液。[30]在没有肺血管狭窄的情况下,增加的背压被描述为“分离的毛细血管后肺动脉高压”(较早的术语包括'被动'或'比例'肺动脉高压或'肺静脉高压')。然而,在一些患者中,肺血管中升高的压力触发血管变窄的叠加部分,这进一步增加了心脏右侧的工作量。这被称为“具有毛细血管前成分的毛细血管后肺动脉高压”或“毛细血管后毛细血管和毛细血管前肺动脉高压”(较早的术语包括“反应性”或“不成比例”肺动脉高压)。 [10] [13] [31]

在由肺病和/或缺氧引起的肺动脉高压中(WHO组3),肺泡中的低水平氧(由于呼吸系统疾病或生活在高海拔地区)导致肺动脉收缩。这种现象称为缺氧性肺血管收缩,最初是一种保护性反应,旨在阻止过多的血液流入肺部受损且不含氧的区域。当肺泡缺氧被广泛和延长时,这种缺氧介导的血管收缩发生在肺血管床的大部分上并导致肺动脉压的增加,肺血管壁的增厚导致持续肺动脉高压的发展。 [19] [32] [33] [34]

在CTEPH(WHO第4组)中,起始事件被认为是肺血管阻塞或变窄,血栓未解决;这些凝块可导致肺循环其余部分的压力和剪切应力增加,导致血管壁的结构变化(重塑)类似于在其他类型的严重肺动脉高压中观察到的那些。血管闭塞和血管重塑的这种组合再次增加了对血流的抵抗力,因此系统内的压力升高。[35] [36]

分子病理学

Three major signaling pathways involved in the pathogenesis of pulmonary arteria.png
三种主要信号通路参与肺动脉高压的发病机制
肺动脉高压(PAH)的分子机制尚不清楚,但据信内皮功能障碍导致内皮源性血管扩张剂如一氧化氮和前列环素的合成减少[37]。此外,还有刺激血管收缩剂的合成,例如血栓素和血管内皮生长因子(VEGF)。这些导致PAH患者出现严重的血管收缩和血管平滑肌以及外膜肥大[37]。

一氧化氮可溶性鸟苷酸环化酶途径
在正常情况下,血管内皮型一氧化氮合酶在氧气存在下从L-精氨酸产生一氧化氮。[38]

这种一氧化氮扩散到邻近细胞(包括血管平滑肌细胞和血小板)中,在那里它增加了酶可溶性鸟苷酸环化酶的活性,导致三磷酸鸟苷(GTP)形成环鸟苷酸(cGMP)。[39]然后cGMP激活cGMP依赖性激酶或PKG(蛋白激酶G)。活化的PKG促进血管舒张(通过降低细胞内钙水平),改变参与平滑肌细胞收缩,迁移和分化的基因的表达,并抑制血小板活化。[40]一氧化氮可溶性鸟苷酸环化酶信号传导也会导致抗炎作用。[41]

在肺组织中丰富的5型磷酸二酯酶(PDE5)水解cGMP的环状键。因此,cGMP的浓度(以及因此PKG活性)降低。[42] [43]

内皮素
内皮素-1是在内皮细胞中产生的肽(包含21个氨基酸)。它作用于各种细胞类型中的内皮素受体ETA和ETB,包括血管平滑肌细胞和成纤维细胞,导致血管收缩,肥大,增殖,炎症和纤维化。它还作用于内皮细胞中的ETB受体;这导致血管收缩剂和血管扩张剂从这些细胞中释放出来,并从系统中清除内皮素-1 [44] [45]。

前列环素(和血栓素)
前列环素由内皮细胞中的花生四烯酸合成。在血管平滑肌细胞中,前列环素主要与前列腺素I受体结合。这发出信号以增加腺苷酸环化酶活性,这导致环腺苷一磷酸(cAMP)的合成增加。这反过来导致cAMP依赖性蛋白激酶或PKA(蛋白激酶A)活性增加,最终促进血管舒张和抑制细胞增殖。前列环素信号传导还导致抗血栓形成,抗纤维化和抗炎作用。磷酸二酯酶3和4降低了cAMP(介导前列环素的大部分生物学作用)的水平[46] [47]。血管收缩剂血栓素也由花生四烯酸合成。在PAH中,平衡从前列环素的合成转向血栓素的合成。[46]

其他途径
上述三种途径都是目前可用于PAH的医学疗法的目标。然而,已经鉴定了几种在PAH中也发生改变的其他途径,并且正在研究这些途径作为未来治疗的潜在靶标。例如,线粒体酶丙酮酸脱氢酶激酶(PDK)在PAH中被病理激活,导致代谢从氧化磷酸化转变为糖酵解,并导致细胞增殖增加和细胞凋亡受损。[46] [48]血管活性肠肽(一种具有抗炎和免疫调节作用的强效血管扩张剂)的表达在PAH中降低,而其受体的表达增加[46] [48]。虽然血清素在PAH的发病机制中所起的作用仍然不确定,但PAH患者血浆中促进血管收缩,肥大和增殖的血浆水平增加[11] [46]。几种生长因子(包括血小板衍生生长因子,碱性成纤维细胞生长因子,表皮生长因子和血管内皮生长因子)的表达或活性增加,并有助于PAH中的血管重塑。[46]肺血管平滑肌细胞增殖状态的其他因素包括OPG [49]和TRAIL [50]。仅关注肺血管系统提供了PAH的不完整图像;右心室适应增加的工作量的能力因患者而异,是生存的重要决定因素。因此,正在研究右心室中PAH的分子病理学,并且最近的研究已经转向将心肺单元视为单个系统而不是两个单独的系统。重要的是,右心室重塑与细胞凋亡增加有关;这与肺血管重塑相反,肺血管重塑涉及抑制细胞凋亡。[51]

诊断

Phonocardiogram and jugular venous pulse tracing from a middl.jpg
中年男性心肌病合并肺动脉高压的心电图和颈静脉脉搏示踪。 颈静脉脉搏追踪表明没有观察到c或v波的突出波。 心音图(第四左间隙和心尖)显示三尖瓣功能不全和心室和心房疾病的杂音。

Pulmonary artery catheter.JPG
肺动脉导管

Severe tricuspid regurgitation.jpg
严重的三尖瓣反流
在肺动脉高压的诊断方面,它有五种主要类型,必须进行一系列测试以区分肺动脉高压与静脉,缺氧,血栓栓塞或不明确的多因素变种。 PAH在排除其他可能的肺动脉高压原因后被诊断出来。[11]

体检
进行体格检查以寻找肺动脉高压的典型症状(如上所述),[52]并建立详细的家族史以确定该疾病是否可遗传。[53] [54] [55]接触药物如苯氟尿嘧啶(一种芬氟拉明衍生物),达沙替尼,可卡因,甲基苯丙胺,导致肝硬化的乙醇以及导致肺气肿的烟草的历史被认为是重要的。[11] [56] [20]在怀孕期间(特别是妊娠晚期)使用选择性5-羟色胺再摄取抑制剂与婴儿患新生儿持续性肺动脉高压的风险增加有关[20]。

超声心动图
如果基于上述评估怀疑肺动脉高压,则进行超声心动图作为下一步。[10] [11] [55]多普勒超声心动图预测右心导管检查结果的荟萃分析报告敏感性和特异性分别为88%和56%[57]。因此,多普勒超声心动图可以提示存在肺动脉高压,但右心导管(如下所述)仍然是诊断PAH的金标准。[10] [11]超声心动图还可以帮助检测先天性心脏病是导致肺动脉高压的原因。[10]

1-4 month old with pulmonary hypertension as seen on ultrasound[58].gif
超声检查发现4个月大的肺动脉高压[58]

2-4 month old with pulmonary hypertension as seen on ultrasound[58].gif
超声检查发现4个月大的肺动脉高压[58]

3-4 month old with pulmonary hypertension as seen on ultrasound[58].gif
超声检查发现4个月大的肺动脉高压[58]

4 month old with pulmonary hypertension as seen on ultrasound[58].jpg
超声检查发现4个月大的肺动脉高压[58]

Long standing pulmonary hypertension[59].gif
长期肺动脉高压[59]

排除其他疾病
如果超声心动图与肺动脉高压的诊断相容,则考虑肺动脉高压(左心病和肺病)的常见原因,并相应地进行进一步的测试。 这些测试通常包括心电图(ECG),肺功能测试,包括一氧化碳和动脉血气测量的肺扩散能力,胸部X射线和高分辨率计算机断层扫描(CT)扫描。[10] [11] [55]] [60]

通气/灌注闪烁扫描
如果排除心脏病和肺病,则进行通气/灌注扫描以排除CTEPH。 如果发现不匹配的灌注缺损,则进行CT肺动脉造影,右心导管检查和选择性肺血管造影的进一步评估。[10] [55]

CT 扫描

Pulmonary artery hypertension and emphysema as seen on a CT scan with contrast.png
肺动脉高压和肺气肿在CT扫描中可见对比剂
胸部CT扫描显示肺动脉高压的迹象是:

扩张肺动脉干(在其分叉处测量)。然而,它是间质性肺病患者肺动脉高压的不良预测因子。[61]
建议女性直径超过27毫米,男性直径超过29毫米。[61]
在没有间质性肺病的个体中,31.6 mm的截止值可能更具统计学意义。[61]
增加主肺动脉(肺动脉干)直径与升主动脉(在其分叉处测量)的比率。
建议将比率​​设为1.0作为成年人的临界值。[61]
儿童截止约1.09。[61]
增加节段性动脉与支气管的直径比。这种发现在三个或四个肺叶中,存在扩张的肺动脉干(≥29mm),并且没有显着的结构性肺病,这使肺动脉高压的特异性达到100%。[61]
Eisenmenger综合征患者最常见于中央肺动脉壁层钙化[61]。
右心导管插入术
尽管可以在超声心动图的基础上估计肺动脉压(PAP),[62]通过心脏右侧插入Swan-Ganz导管的压力测量提供了最明确的评估。[42]肺动脉高压定义为静息时平均PAP至少为25 mm Hg(3300 Pa),PAH定义为毛细血管前肺动脉高压(即平均PAP≥25mm Hg,肺动脉闭塞压[PAOP]≤15mm Hg和肺血管阻力[PVR]> 3木单位)。[55] PAOP和PVR不能用超声心动图直接测量。因此,PAH的诊断需要右侧心脏导管插入术。 Swan-Ganz导管还可以测量心输出量;这可用于计算心脏指数,这在测量疾病严重程度方面远比肺动脉压更重要。[10] [63]平均PAP(mPAP)不应与收缩期PAP(sPAP)相混淆,后者通常在超声心动图报告中报告。收缩压为40 mm Hg通常意味着平均压力超过25 mm Hg。粗略地说,mPAP = 0.61 sPAP + 2. [64]

其他
对于根据上述测试认为可能患有PAH的人,然后根据体格检查,医疗/家族病史和进一步的特定诊断测试(例如,用于检测潜在结缔组织疾病的血清学检测,HIV感染)确定特定的相关病症。或肝炎,超声检查确认门静脉高压的存在,先天性心脏病的超声心动图/心脏磁共振成像,血吸虫病的实验室检查,PVOD和肺毛细血管血管瘤病的高分辨率CT)。 PAH患者不鼓励进行常规肺活检,因为患者存在风险,因为这些结果不太可能改变诊断和治疗[10] [26] [55]。

治疗
肺动脉高压的治疗取决于PH是动脉,静脉,缺氧,血栓栓塞还是其他。如果它是由左心病引起的,治疗方法是通过使用药物或维修/更换二尖瓣或主动脉瓣来优化左心室功能。[65]患有左心衰竭或低氧血症的肺病(II组或III组肺动脉高压)患者不应常规使用包括前列腺素,磷酸二酯酶抑制剂或内皮素拮抗剂在内的血管活性药物治疗,因为这些药物被批准用于称为原发性肺动脉高压的不同病症。 66]为了区分,医生至少会进行右心脏心导管检查,超声心动图,胸部CT,6分钟步行试验和肺功能检查。[66]对患有这些病症的患者使用其他类型的肺动脉高压治疗可能会伤害患者并浪费大量医疗资源。[66]

高剂量钙通道阻滞剂仅适用于通过Swan-Ganz导管血管反应的5%IPAH患者。不幸的是,钙通道阻滞剂在很大程度上被滥用,被许多非血管反应性PAH患者开处方,导致发病率和死亡率过高。[15]血管反应性的标准已经改变。只有那些平均肺动脉压力下降超过10 mm Hg至低于40 mm Hg并且在用腺苷,依前列醇或一氧化氮攻击时心输出量没有增加或增加的患者被认为是血管反应性的。[67]其中,只有一半的患者长期对钙通道阻滞剂有反应。[68]

最近已经引入了许多用于原发性和继发性PAH的药剂。支持使用这些药剂的试验相对较少,并且用于比较其有效性的唯一措施是“6分钟步行试验”。许多人没有关于死亡率受益或进展时间的数据。[69]

血管活性物质
许多途径涉及肺动脉高压患者肺动脉平滑肌细胞的异常增殖和收缩。其中三种途径很重要,因为它们已经被药物靶向 - 内皮素受体拮抗剂,5型磷酸二酯酶(PDE-5)抑制剂和前列环素衍生物。[70]

前列腺素
前列环素(前列腺素I2)通常被认为是PAH最有效的治疗方法。依前列醇(合成前列环素)通过连续输注给予,需要半永久性中心静脉导管。该递送系统可引起败血症和血栓形成。前列环素不稳定,因此在给药期间必须保持在冰上。由于它的半衰期为3至5分钟,因此输液必须是连续的,并且中断可能是致命的。[71]因此开发了其他前列腺素类。曲前列环素可以静脉内或皮下给药,但皮下形式可能非常疼痛。 CDC报告了静脉注射Remodulin导致脓毒症的风险增加。伊洛前列素也在欧洲静脉使用,半衰期较长。 Iloprost是唯一被批准在美国和欧洲使用的前列环素吸入剂,直到吸入形式的曲前列环素于2009年7月被FDA批准。[需要医疗引证]

内皮素受体拮抗剂
双重(ETA和ETB)内皮素受体拮抗剂波生坦于2001年获得批准.Sitaxentan(Thelin)被批准用于加拿大,澳大利亚和欧盟,[72]但不在美国。 2010年,由于致命的肝脏并发症,辉瑞公司在全球范围内撤回了Thelin。一种类似的药物,ambrisentan,由Gilead Sciences在美国以Letairis销售。[73]

磷酸二酯酶5型抑制剂
美国FDA于2005年批准了西地那非,一种cGMP特异性磷酸二酯酶5型(PDE5)的选择性抑制剂,用于治疗PAH。它作为Revatio在市场上销售。 2009年,他们还批准了另一种PDE5抑制剂他达拉非,以Adcirca的名义进行销售。[74]据信,PDE5抑制剂可增加肺动脉血管舒张,抑制血管重塑,从而降低肺动脉压和肺血管阻力。[75]

他达拉非口服,西地那非,并迅速吸收(血清水平可在20分钟检测到)。 T1 / 2(生物半衰期)在健康受试者中徘徊在17.5小时左右。[76]此外,如果我们考虑药物经济学的影响,服用他达拉非的患者将支付西地那非治疗费用的三分之二。[77]然而,这种药物有一些副作用,如头痛,腹泻,恶心,背痛,消化不良,潮红和肌痛。[78]

可溶性鸟苷酸环化酶的活化剂
可溶性鸟苷酸环化酶(sGC)是NO的细胞内受体。截至2009年4月,sGC激活剂cinaciguat和riociguat正在接受PAH治疗的临床试验。[79]

外科
房间隔造口术是一种外科手术,可在右心房和左心房之间建立联系。它减轻了心脏右侧的压力,但代价是血液中的氧含量较低(缺氧)。肺移植治疗肺动脉高压,但患者出现移植并发症,手术后中位生存期仅为5年[80]。

肺血栓内膜切除术(PTE)是一种用于慢性血栓栓塞性肺动脉高压的外科手术。它是手术切除有组织的血栓(凝块)以及肺动脉的内层;这是一个非常困难的主要程序,目前在一些选定的中心进行。[81]

监控
已确立的临床实践指南规定了肺结节评估和监测的频率,[66] [82]患者通常通过常用的测试进行监测,例如:

脉搏血氧仪
动脉血气测试
胸部X光片
串行心电图测试
连续超声心动图
肺活量测定或更先进的肺功能研究
6分钟步行测试[83]

预测

Cor pulmonale.jpg
肺心病
肺动脉高压的预后(WHO I组)从诊断时起未经治疗的中位生存期为2  -  3年,死因通常为右心室衰竭(肺心病)。[84]最近对那些开始接受波生坦治疗的患者(Tracleer)进行的一项研究显示,89%的患者在2年时仍然存活[85]。随着新疗法,存活率正在增加。 2006年3月至2009年12月期间入选登记处评估早期和长期肺动脉高血压疾病管理(REVEAL登记处)的2,635名患者,1年,3年,5年和7年生存率为85%,68 %,57%和49%。对于患有特发性/家族性PAH的患者,存活率分别为91%,74%,65%和59%。[86]患有严重肺动脉高压的孕妇的死亡率非常高(WHO I组)。怀孕有时被描述为这些女性的禁忌症。[87] [88] [89]

流行病学
IPAH的流行病学在美国每年约有125-150人死亡,而在世界范围内,其发病率与美国相似,为百万分之四。然而,在欧洲部分地区(法国),有迹象显示每百万IPAH有6例。女性的发病率高于男性(2-9:1)。[90]

其他形式的PH更为常见。在系统性硬皮病中,估计发病率为所有患者的8%至12%; [91]在类风湿性关节炎中罕见。[92]然而,在系统性红斑狼疮中,它为4%至14%,[93]而在镰状细胞病中,其范围为20%至40%。[94]高达4%的肺栓塞患者继续发展为慢性血栓栓塞性疾病,包括肺动脉高压[95]。一小部分COPD患者发生肺动脉高压而没有其他疾病来解释高压。[96]另一方面,肥胖 - 通气不足综合征通常与肺动脉高压引起的右心衰竭有关。[97]

值得注意的案例
Elaine Kaufman,美国餐馆老板[98]
Priya Balachandran,电视制片人[99]
Ina Balin,美国百老汇和电视女演员[100]
Chloe Temtchine,美国创作歌手[101] [102]
Natalie Cole,美国歌手[103]

研究
对于继承该疾病的人,正在研究基因治疗。[104]

另见
Pulmonary Hypertension Association
Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)

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