PULMONARY HYPERTENSION Updated classification and management of pulmonary hypertension
PULMONARY HYPERTENSION Updated classification and management of pulmonary hypertension
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DOI：
10.1136/hrt.2008.156299
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发表时间：
2010-04-01
期刊：
影响因子：
5.7
通讯作者：
Ghofrani, Hossein-Ardeschir
中科院分区：
文献类型：
--
作者：
Nef, Holger M.;Moellmann, Helge;Ghofrani, Hossein-Ardeschir
肺动脉高压（PAH）是一种严重的疾病，其特征是肺动脉压力和阻力进行性增加，导致右心衰竭。 PAH 通常在疾病晚期才被诊断出来，并与进行性临床恶化和过早死亡有关。根据最近的共识会议，肺动脉高压（PH）分为五个主要组：第1组，PAH；第2组，与左心疾病相关的PH；第3组，与肺部疾病和/或低氧血症相关的PH；第4组，慢性血栓和/或栓塞性疾病引起的PH；第5组，杂项总结了各种罕见且特征不明的疾病，其特征是非特异性体征和症状。 1 PH 筛查通常通过经胸超声心动图根据三尖瓣反流射流的速度进行，最后通过右心导管插入术进行确认。 2 如果不进行治疗，严重PH患者的预后很差。据报道，特发性 PAH 的中位预期寿命为 2.8 年（自诊断起）w1；然而，最近对 PAH 领域试验的荟萃分析表明，PAH 治疗对生存具有有益影响。 3 目前的 PAH 特异性药物，包括前列腺素、内皮素受体拮抗剂 (ERA) 和磷酸二酯酶 5 抑制剂 (PDE5i)，试图解决与这种情况相关的肺血管内皮功能障碍和血管收缩。一些新药已经在动物模型中产生了令人鼓舞的结果，旨在更直接地针对结构性血管变化（重塑），目前正在进行临床研究（例如可溶性鸟苷酸环化酶激活剂/刺激剂、酪氨酸激酶抑制剂、5-HT2B受体拮抗剂）。本文回顾了最近更新的临床分类。此外，还总结了评估 PH 的既定方法以及目前推荐的 PAH 治疗方法。
Pulmonary arterial hypertension (PAH) is a severe disease characterised by a progressive increase of pulmonary pressure and resistance leading to right heart failure. PAH is commonly diagnosed at a late stage of the disease and is associated with progressive clinical deterioration and premature death. According to the most recent consensus conference, pulmonary hypertension (PH) is categorised into five main groups: group 1, PAH; group 2, PH associated with left sided heart diseases; group 3, PH associated with lung disease and/or hypoxaemia; group 4, PH due to chronic thrombotic and/or embolic disease; and group 5, miscellaneousdsummarising a variety of rare and not well characterised disorders characterised by non-specific signs and symptoms. 1 Screening for PH is usually made by transthoracic echocardiography on the basis of the velocity of the regurgitant tricuspid jet, and final confirmation is done by right heart catheterisation. 2 Without treatment, the prognosis for patients with significant PH is poor. The reported median life expectancy of idiopathic PAH is 2.8 years from the diagnosisw1; however, recent meta-analysis of trials in the field of PAH have provided indications of a beneficial influence of PAH treatments on survival. 3 Current PAH specific medications, including prostanoids, endothelin receptor antagonists (ERA), and phosphodiesterase 5 inhibitors (PDE5i), have sought to address the pulmonary vascular endothelial dysfunction and vasoconstriction associated with this condition. Several novel drugs that have already produced encouraging results in animal models aim to target more directly the structural vascular changes (remodelling) and are currently under clinical investigation (eg, soluble guanylyl cyclase activators/stimulators, tyrosine kinase inhibitors, 5-HT2B receptor antagonists). This article reviews the clinical classification, which has been recently updated. Moreover, established approaches to evaluate PH and the currently recommended treatments for PAH are summarised.