左心疾病相关性肺动脉高压临床研究进展

肺动脉高压是一大类进展性、致残率和致死率极高的疾病。左心疾病相关性肺动脉高压最为常见,其病情更重、预后更差、病死率更高,严重影响患者生活质量。目前该类肺动脉高压的流行病学数据不清楚、临床诊断标准不统一、病理生理机制不甚明确、分类方法存在争议、靶向药物治疗缺乏证据,国内外相关研究和治疗十分有限。现概述左心疾病相关性肺动脉高压的定义及分类、流行病学、病因及发病机制、病理生理机制,并详细阐述其诊断及特异性治疗的临床研究进展。     

肺动脉高压发病机制的研究进展

肺动脉高压（Pulmonary Arteria 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,是临床众多疾病常见的并发症,也可以原因不明.虽然对肺动脉高压的研究已有100多年,但其发病机制尚未完全明了,可能与缺氧、神经体液、先天性、遗传等因素有关[1].近年来,细胞生物学和分子遗传学的飞速发展促进了对肺动脉高压发病机制的深入研究,进而带动了肺动脉高压诊断学和治疗学研究的进步.本文综述了近几年来国内外有关肺动脉高压研究进展,系统探讨了肺动脉高压发病机制以及治疗进展,为临床治疗提供一定理论依据.     

重视对肺动脉高压新知识的学习和应用

肺动脉高压（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，主要病理学改变为血管收缩、血管重构和原位血栓形成，其特征为内皮细胞和平滑肌细胞增殖所引起的阻塞性病变。近年来国外在肺动脉高压的发病机制、临床分类、诊断标准和治疗研究等方面均取得了较大进展，特别是2004年美国胸科医师学会和欧洲心脏病学会分别发表了有循证医学证据的肺动脉高压诊断与治疗指南。     

肺动脉高压的肺血管病理研究进展

近年来肺血管病理学的研究领域，尤其是肺静脉闭塞病和肺多发性毛细血管瘤病，取得了显著进展，因而美国胸科医师学院和欧洲心脏病协会于2003年制定了肺动脉高压的肺血管病理分类和诊断标准，进一步指导肺动脉高压的诊断和治疗。本文对最新的肺动脉高压病理学研究进展进行了回顾。     

肺动脉高压的研究进展

肺动脉高压是以肺血管阻力进行性增加为临床特征的一组威胁生命的疾病，近10年来，肺动脉高压的研究取得了显著的进展，涉及2003年威尼斯会议修改的肺动脉高压的命名和分类、遗传基因的发现、应用分子生物学技术探讨其发病学以及内科和外科治疗效果的改善。前列环素及其类似物和内皮素受体拮抗剂的临床研究，使肺动脉高压在治疗的安全性和有效性方面均取得了长足的进步。     

肺动脉高压治疗进展

肺动脉高压是一种进行性发展且预后不良的疾病.随着对肺动脉高压发病机制研究的不断深入,其治疗已从单纯的血管扩张剂治疗发展到以疾病的分子遗传基础为靶向的药物治疗.本文着重介绍有关肺动脉高压治疗方面的进展.     

先天性心脏病相关肺动脉高压及其诊治进展

肺动脉高压(pulmonary arterial hypertension,PAH)是先天性心脏病(congenital heart disease,CHD)最常见的合并症,在各型CHD当中,肺动脉高压的诊断和治疗贯穿术前、术中和术后,甚至伴随患者终生。文章将从流行病学、分类、筛查与诊断、血流动力学评价,以及靶向治疗带来的新机会和预后的变化等几个方面,阐述CHD相关PAH近几年的进展。     

肺动脉高压药物治疗进展

近20年来,由于肺动脉高压病理生理和分子机制研究的进展,使药物治疗有了很大的发展,病人的生存率和生存质量得以大大提高。现对肺动脉高压的药物治疗进展,介绍一些经临床试验证实有效的药物和潜在的治疗药物。     

心脏瓣膜病继发的肺动脉高压

目前已有较多关于原发性肺动脉高压和由先天性心脏病引起的继发性肺动脉高压的研究,但对心脏瓣膜病继发的肺动脉高压的全面深入研究仍较少报道,尤其是心脏瓣膜病伴重度肺动脉高压的手术治疗的风险评估和预后的预测等并不是十分清楚。本文就继发于心脏瓣膜病的肺动脉高压的发生机制、病理改变以及预后评估等方面做简要综述。     

靶向药物治疗慢性血栓栓塞性肺动脉高压的合理性及疗效探讨

慢性血栓栓塞性肺动脉高压(chronic thromboembolic pulmonary hypertension,CTEPH)是一种慢性进展性疾病,如果未经治疗,预后极差。2013年在法国尼斯召开的第五届世界肺动脉高压论坛会上将CTEPH归为第四大类肺动脉高压。由于CTEPH的症状因人而异且缺乏特异性,容易引起漏诊、误诊和延迟诊断。外科肺动脉血栓内膜剥脱术是唯一有可能治愈CTEPH的办法,但有一部分患者属于CTEPH分型中的Ⅲ型和Ⅳ型,即阻塞在亚段以下的远端或者非阻塞部位的小动脉发生血管重塑,使患者手术难度增大甚至不能进行外科手术,还有小部分患者接受外科手术后持续存在肺动脉高压。动脉性肺动脉高压和CTEPH在临床表现、病理特点和发病机制方面有交叉重叠部分,因此推动了有关靶向药物治疗CTEPH患者有效性和安全性的相关研究。     

肺动脉高压新的临床分类与组织病理学改变

肺动脉高压新的临床分类反映了当前对肺动脉高压发病机制和诊治策略的最新认识.肺动脉高压多样的组织病理改变也反映了致病因素、预后和对各种治疗的反应不同.重新评估肺动脉高压的组织病理学改变,会获取对临床诊治新的启示,有利于弥补非组织形态技术研究的不足.     

阿托伐他汀在肺动脉高压中的研究

肺动脉高压是一种慢性不可治愈的疾病,近10年来其治疗有了长足的进步,但是靶向药物治疗费用昂贵,不良反应多,亟待需要新药物的出现。阿托伐他汀以其不良反应小、药价低廉等优点将有望成为治疗肺动脉高压的有效药物。现将肺动脉高压定义、发病机制、病理生理改变、阿托伐他汀多效性等方面作如下综述。     

Primary pulmonary hypertension in adults

Primary pulmonary hypertension is an enigmatic disease found predominantly in young women, but it also affects a significant number of middle-aged and elderly males and females. Its onset, characterized by progressively worsening dyspnea, fatigue, and chest pain, is insidious. Three distinct histopathologic subtypes have been identified, and the natural history of the disease process has been well-defined. Pharmacologic treatment options have, in general, been disappointing, and it appears that heart-lung transplantation will be applied only to a small minority of young patients with primary pulmonary hypertension in the near future. We review the histopathology, evaluation, treatment, and prognosis of primary pulmonary hypertension.     

Pulmonary arterial hypertension

It is important for the general internist to maintain a high degree of suspicion for pulmonary hypertension in appropriate cases. This article reviews the definition and classification of pulmonary hypertension, epidemiology and risk factors, signs and symptoms, diagnostic approach, and treatment strategies. Multiple effective therapies now exist for pulmonary arterial hypertension, and thus early diagnosis and referral will translate into meaningful survival and better lives for our patients.     

慢性阻塞性肺疾病诊治进展

慢性阻塞性肺疾病(COPD)仍然是一个重要的公共卫生问题,也是病死率呈上升趋势的少数慢性疾病之一.近几年COPD发病机制、全身效应、急性发作和治疗等方面都有许多新的进展.目前COPD已被定义为可以预防、可以治疔的疾病,除了肺部病变的特点外,还伴有一些与患者疾病严重性相关的显著的肺外效应.本文从定义、诊断、病情评估和治疗...     

肺动脉高压治疗过程中抗凝药物应用的研究进展

肺动脉高压是一组以肺血管阻力持续增加为特征的临床综合征,其发病机制复杂,预后差。有研究表明原位血栓形成及凝血功能紊乱在肺动脉高压的发病机制中起着重要作用,是肺动脉高压发生发展的重要机制之一。但目前关于抗凝药物在肺动脉高压中的应用始终无统一的结论,本文就肺动脉高压治疗过程中应用抗凝药物的病理学和生理学基础及目前抗凝药物在肺动脉高压应用情况及最新进展展开综述。     

Assessment and treatment of pulmonary arterial hypertension: an Australian perspective in 2006

Pulmonary arterial hypertension is a group of diseases which forms a small subset of those with elevated pulmonary artery pressure (pulmonary hypertension). The recent development of selective pulmonary vasodilator has lead to a substantial resurgence of interest in what have been previously regarded as rare and incurable diseases. This review aims to describe the spectrum of pulmonary vascular diseases, the evolving understanding as to pathogenesis, the evolving evidence of efficacy for drug therapies, trying to put this into a contemporary Australian context. Several key pathogenic pathways may be involved: prostacycline, Nitric Oxide-cGMP-phosphodiesterase 5 and endothelin- all of which are exploited for therapeutic benefit by newly available drug therapies. A recently modified classification system reasserts the importance of precise diagnosis. The cardinal symptom of exertional dyspnea warrants careful evaluation in an attempt to prevent (frequently occurring) substantial delay in diagnosis. Echocardiogram is the cornerstone of screening for pulmonary arterial hypertension; however, a detailed evaluation including a carefully performed right heart catheterisation with sufficient data to allow calculation of pulmonary vascular resistance is key to accurate diagnosis. These new approaches to therapy are already substantially improving quality of life and prognosis.     

肺动脉高压的诊断和治疗进展

肺动脉高压是一种病死率很高的严重疾病,它以肺血管阻力不断升高为主要特征并最终引起右心衰竭和死亡。近年来,在肺动脉高压的诊断、治疗的研究中取得了长足的进步。规范的诊断和治疗将有助于改善肺动脉高压患者的生活质量和预后。     

Recent progress in the diagnosis and management of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension caused by non-resolving thromboembolisms of the pulmonary arteries. In Japan, in contrast to Western countries, CTEPH is more prevalent in women. A Japanese multicenter study reported that a form of CTEPH unrelated to deep vein thrombosis is associated with HLA-B?5201, suggesting that this form of CTEPH may be associated with vasculopathy. CTEPH can be cured by pulmonary endarterectomy, provided that the thrombi are surgically accessible; thus, early diagnosis is important, and all patients with exertional dyspnea should be evaluated for pulmonary hypertension. Ventilation/perfusion scans provide an excellent non-invasive means to distinguish CTEPH from pulmonary arterial hypertension. Similarly, computed tomographic pulmonary angiograms allow for the detection of thrombi and evaluation of pulmonary hemodynamics in a minimally invasive manner. Importantly, the absence of subpleural perfusion on pulmonary angiograms can suggest the presence of small vessel disease. Small vessel disease might be involved in the pathogenesis of CTEPH, and its detection is essential in preventing operative death. Although no modern therapies for pulmonary arterial hypertension have been approved for treatment of CTEPH, a recent randomized control trial of riociguat in patients with CTEPH demonstrated that riociguat significantly improved 6-min walking distance. Further investigations into treatments that target endothelial dysfunction and hyperproliferative CTEPH cells are needed. Recently, balloon pulmonary angioplasty has emerged as a promising treatment modality in Japan. A specialized medical team, including at least one expert surgeon, should make decisions regarding patients' candidacy for pulmonary endarterectomy and/or balloon pulmonary angioplasty.     

肺动脉高压研究进展

近年来有关肺动脉高压及右心功能方面的研究已成为国内外学术热点,欧洲心脏病学会(ESC)和美国心脏病学会基金会(ACCF)/美国心脏学会(AHA)相继发表了新的肺动脉高压诊断和治疗指南(共识).现简要介绍新的指南,并就肺动脉高压的基础研究、临床治疗以及肺动脉高压与右心的相关研究进展进行综述.