肺动脉高压的发病机制及治疗进展

肺动脉高压可由许多心、肺和肺血管疾病引起。引起肺动脉高压的原因很多 ,而原发性肺动脉高压是其中的一个特殊的类型。目前 ,关于肺动脉高压的发病机制仍不十分清楚 ,但体内舒张与收缩血管物质的不平衡是发病的主要因素。随着对发病机理的认识 ,治疗观点已从传统的扩张血管治疗过渡到调整体内舒缩血管物质的平衡。本文仅就近年来肺动脉高压的发病机制及治疗进展作简要介绍     

肺动脉高压血管收缩与重塑的研究

肺动脉高压（PH）可以出现在原发于肺动脉的疾病（如特发性肺动脉高压，IPAH）或者继发于心肺疾病同时伴有肺动脉高压的疾病（如继发性肺动脉高压，SPH）。由于肺动脉高压发病机制复杂且有许多机制不明，治疗效果不理想。特别是IPAH，一经确诊，平均存活时间只有2．5年，有学者认为其预后比恶性肿瘤还差。诸多致病因素参与了肺动脉高压的发生与发展并最终引起血管收缩，导致血管重塑。     

肺动脉高压的发病机制和药物治疗进展

肺动脉高压(PAH)是一种少见的,预后不良的进展性疾病,其主要特征为肺血管阻力增加和肺血管压力持续升高,最终结局是右心衰竭和过早死亡[1]。PAH的病因不尽相同,所以针对不同的临床症状和发病原因,PAH的治疗药物和治疗方法也较多。本文就PAH的发病机制以及临床中常见的治疗药物进行综述。     

肺动脉高压的分类和研究进展

肺动脉高压(Pulmonary hypertention PH)是不同病因导致的、以肺动脉压力和肺血管阻力升高为特点的一组病理生理综合征,主要病理机制是血管收缩、血管重塑和原位血栓形成,最终导致右心负荷增加,右心衰竭.目前肺动脉高压的定义仍然采用了1987年美国国立卫生研究院进行肺动脉高压登记注册时规定的血流动力学标准.     

肺动脉高压:挑战与机遇并存

肺动脉高压(pulmonary arterial hypertension,PAH)是以肺血管阻力进行性增加为主要特点的临床-病理生理综合征。尽管近30余年来对这类疾病的认识不断深入,对PAH的发病机制、疾病的分类、筛查和诊断技术以及特异性治疗药物的研究取得迅速进展,但是,尚有许多问题亟待解决,阐述如下。关于发病机制PAH的发病机制至今尚未完全清楚,新的研究正在逐步加深对该疾病的认识,使其轮廓日渐清     

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。     

肺动脉高压分子机制的研究进展

<正>肺动脉高压(pulmonary arterial hypertension,PAH)是以肺血管阻力进行性升高,和右心功能进行性衰竭为主要特征的病理过程,是很多心、肺血管疾病发生、发展的重要病理生理基础。最近的临床实验、基础研究中发现[1],一些细胞因子、信号通路、基因表达、离子通道功能等发生改变后,血管内大量异常增殖反应,导致了不可逆性肺血管的重构,肺动脉高压形成的结局,而且它们之间并不是独立的,有很多的相互作用。这里,就其分子机制综述如下。     

肺动脉高压发病机制新进展

肺动脉高压是指肺血管压力超过一定界值而引起的一系列临床表现,最终导致心功能不全甚至死亡。目前发现离子通道机制、血管活性物质失衡机制、免疫炎症机制和遗传机制均与该病发生有关,现就以上机制做一综述。     

肺动脉高压药物治疗的新进展

近年来，随着国际上对肺动脉高压诊断分类的更新和药物治疗肺动脉高压研究的进展，使得本领域的临床研究成为备受关注的热点，尤其在肺动脉高压药物治疗的观念上，已从最初的单纯降低肺动脉压力发展到根据发病机制中的重要靶点进行干预，如阻止血管内皮细胞无序增生和血管重构、诱导内皮细胞凋亡等治疗上，并在临床上取得了明显的疗效。应该说，药物治疗正在发挥越来越重要的作用。现将近年药物治疗肺动脉高压的新进展概述如下。     

门脉高压性肺动脉高压研究进展

门脉高压性肺动脉高压是一种特殊类型的肺动脉高压,现从发病率、发病机制、临床特点、诊断及治疗等方面阐述门脉高压性肺动脉高压的研究进展。     

肺动脉高压靶向药物治疗现状和展望

肺动脉高压(PAH)是一种高死亡率的肺血管疾病,其特征是肺血管重构和肺血管阻力增加,如果不及时治疗,最终导致右心衰竭和死亡。在过去的几十年中,靶向药物的出现显著改善了患者结局。但目前的治疗仍难以阻止或逆转PAH的病程进展,PAH仍然是一种致命性的疾病,为此开发新的药物治疗靶点尤为重要。目前,针对肺动脉平滑肌细胞异常增殖、迁移和凋亡的潜在逆转肺血管重构途径的药物正成为研究热点,为治愈PAH带来了新的希望。该文对PAH靶向药物的治疗现状和展望进行阐述,并讨论靶向治疗PAH的循证医学证据。     

Current treatment strategies for pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a disease characterized by an elevation in pulmonary artery pressure that can lead to right ventricular failure and death. Although there is no cure for PAH, newer medical therapies have been shown to improve a variety of clinically relevant end-points including survival, exercise tolerance, functional class, haemodynamics, echocardiographic parameters and quality of life measures. Since the introduction of continuous intravenous prostacyclin, the treatment armamentarium of approved drugs for PAH has expanded to include prostacyclin analogues with differing routes of administration, a dual endothelin receptor antagonist, and a phosphodiesterase-5 inhibitor. Selective endothelin-A receptor antagonists have shown promise in clinical trials and are likely to be added to the list of options. As the number of medications available for PAH continues to increase, treatment decisions regarding first-line therapy, combination treatments, and add-on strategies are becoming more complex. This article reviews the current treatments strategies for PAH and provides guidelines for its management.     

Current insights on the pathogenesis of pulmonary arterial hypertension

Regardless of the initial trigger, the elevated pulmonary arterial pressure and vascular resistance in patients with pulmonary arterial hypertension are primarily caused by remodeling and thrombosis of small- and medium-sized pulmonary arteries and arterioles, as well as sustained vasoconstriction. The process of pulmonary vascular remodeling involves all layers of the vessel wall and is complicated by cellular heterogeneity within each compartment. Indeed, each cell type (endothelial cells, smooth muscle cells, and fibroblasts), as well as inflammatory cells and platelets, may play significant roles in this condition. Recent studies have emphasized the relevance of several mediators in this condition, including prostaglandin-I (2) (prostacyclin), nitric oxide, endothelin-1, angiopoietin-1, 5-hydroxytryptamine (serotonin), cytokines, chemokines, and members of the transforming growth factor beta (TGF-beta) superfamily. Targeting some of these dysfunctional pathways (prostacyclin, nitric oxide, and endothelin-1) has been beneficial in subjects displaying pulmonary arterial hypertension.     

Current status of bosentan for treatment of pulmonary hypertension

Pulmonary arterial hypertension (PAH) is a debilitating disease associated with significant morbidity and a high mortality if left untreated. Over the past 5 years, there have been significant advances with regard to the understanding of the pathogenesis, diagnosis and classification of PAH. The availability of newer drugs has resulted in a radical change in the management of this disease with significant improvement in both the quality of life and mortality. One of the recent drugs is an orally active dual endothelin receptor antagonist, bosentan; this drug has shown to improve the exercise capacity and survival in patients with PAH. This review article discusses the pharmacology of bosentan and summarises the current available evidence for the safety and efficacy of bosentan for the treatment of PAH.     

慢性血栓栓塞性肺高压的治疗现状

慢性血栓栓塞性肺高压（chronic thromboembolic puhnonary hypertension, CTEPH）是指由于机化血栓阻塞近端或远端肺动脉后,造成以肺动脉压力增高为特点的一类疾病。过去曾认为CTEPH是非常罕见的疾病,目前国际上虽仍缺乏确切可靠的流行病学资料,但实际上其发病率被远远低估。CTEPH已成为引起严重肺高压的重要病因之一。     

An epidemiological study of pulmonary arterial hypertension.

All hospitalisations for pulmonary arterial hypertension (PAH) in the Scottish population were examined to determine the epidemiological features of PAH. These data were compared with expert data from the Scottish Pulmonary Vascular Unit (SPVU). Using the linked Scottish Morbidity Record scheme, data from all adults aged 16-65 yrs admitted with PAH (idiopathic PAH, pulmonary hypertension associated with congenital heart abnormalities and pulmonary hypertension associated with connective tissue disorders) during the period 1986-2001 were identified. These data were compared with the most recent data in the SPVU database (2005). Overall, 374 Scottish males and females aged 16-65 yrs were hospitalised with incident PAH during 1986-2001. The annual incidence of PAH was 7.1 cases per million population. On December 31, 2002, there were 165 surviving cases, giving a prevalence of PAH of 52 cases per million population. Data from the SPVU were available for 1997-2006. In 2005, the last year with a complete data set, the incidence of PAH was 7.6 cases per million population and the corresponding prevalence was 26 cases per million population. Hospitalisation data from the Scottish Morbidity Record scheme gave higher prevalences of pulmonary arterial hypertension than data from the expert centres (Scotland and France). The hospitalisation data may overestimate the true frequency of pulmonary arterial hypertension in the population, but it is also possible that the expert centres underestimate the true frequency.     

HIV-related pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare but potentially fatal complication of human immunodeficiency virus (HIV). It may occur in HIV-1 or 2 infection, irrespective of the route of transmission or the degree of immunosuppression. The improved survival of patients infected with HIV in the era of highly active antiretroviral therapy (HAART) justifies systematic screening for PAH according to an algorithm in patients with unexplained dyspnea. In all cases, right heart catheterization must be performed to establish the definitive diagnosis of pulmonary hypertension. The prevalence of PAH is about 0.5% in patients with HIV infection. A beneficial effect of HAART on the course of HIV-related PAH has not been clearly established. In contrast, PAH-specific therapies such as epoprostenol and bosentan have been demonstrated to be efficacious for short- and long-term outcomes in this context. Notably, some patients pulmonary hemodynamics and functional class normalized or near normalized with these treatments. Other PAH-specific therapies remain to be evaluated. The advent of HAART associated with the development of PAH-specific therapies has improved the prognosis of patients HIV-related PAH, with a survival rate of about 70% at 3 years.