慢性血栓栓塞性肺动脉高压新治疗方法的进展

慢性血栓栓塞性肺动脉高压是由于肺动脉血栓未完全溶解导致的一类毛细血管前性肺动脉高压.肺动脉内膜剥脱术是目前慢性血栓栓塞性肺动脉高压的首选治疗方案.但很多患者因远端血管病变或严重合并症而无法手术,或手术后有持续性肺动脉高压,球囊肺血管成形术和靶向药物的发展为这些患者的治疗带来了新的希望.现就慢性血栓栓塞性肺动脉高压新疗法...     

注射用血塞通(冻干)对肺心病患者肺动脉高压的防治研究

目的 注射用血塞通(冻干)对肺心病患者肺动脉高压的作用,并探讨其作用机制.方法 将80例肺心病肺动脉高压患者,随机分为治疗组和对照组,各40例.观察两组临床疗效以及治疗前后临床症状积分、肺动脉平均压(mPAP)、血液流变学等指标的变化.结果 注射用血塞通(冻干)能缓解临床症状、改善血液高凝、降低肺动脉高压;两组治疗后的临床症状积分、mPAP、血液流变学均较对照组有明显的改善.结论 注射用血塞通(冻干)对肺心病肺动脉高压患者治疗具有良好的作用.     

慢性骨髓增殖性疾病并发肺动脉高压4例临床分析

目的 加强对慢性骨髓增殖性疾病(CMPD)患者合并肺动脉高压的认识,以期临床早期诊断和治疗,改善预后.方法 回顾性分析4例慢性骨髓增殖性疾病并发肺动脉高压患者的临床及实验室检查资料、治疗及转归.结果 4例患者诊断慢性骨髓增殖性疾病时年龄为44 ～ 72岁,诊断原发病到发现肺动脉高压1～26年.4例患者临床均表现为乏力、活动耐力下降,脾脏明显肿大,均有不同程度的贫血,3例患者有血小板减少.诊断肺动脉高压时肺动脉压力为58～83mm Hg,三尖瓣反流速率3.5 ～4.3 m/s.4例患者诊断CMPD后均接受正规治疗,随诊过程中发现肺动脉高压,3例患者于诊断肺动脉高压1～2年内死亡.结论 肺动脉高压是慢性骨髓增殖性疾病患者常见的心血管并发症,目前治疗方法有限,慢性骨髓增殖性疾病患者病程中出现肺动脉高压提示预后不良.     

左心疾病相关性肺动脉高压

左心疾病是引起肺动脉高压的常见病因,由其导致的肺动脉高压主要是由于左室或左房充盈压增高所致,如发展至心力衰竭则预后较差,其病死率是不伴有肺动脉高压的心力衰竭患者的2倍多。本文将对左心疾病相关性肺动脉高压的病理生理学以及诊断和治疗策略的研究现状进行阐述。     

房间隔缺损相关肺动脉高压机制及治疗进展

房间隔缺损(ASD)是常见的先天性心脏病,部分患者可能会并发肺动脉高压,对治疗和预后产生重大影响。虽然分流是先天性心脏病患者发生肺动脉高压的决定性因素,但部分患者的分流量并不足以解释肺动脉高压的严重程度。因此,阐明ASD相关肺动脉高压多方面的发病机制,对于认识肺动脉高压的发生发展过程和指导ASD的治疗具有重要意义。现就ASD相关肺动脉高压的病理生理学机制、危险因素和治疗策略的研究进展做一综述,为此类患者的诊治提供参考。     

肺动脉高压药物治疗进展

肺动脉高压(pulmonary artery hypertension,PAH)是指静息时平均肺动脉压>25 mm Hg,且肺毛细血管压或左房压<15 mm Hg [1].血管扩张剂是治疗肺动脉高压的一类重要药物,能降低肺动脉压力,改善患者血流动力学及肺通气/灌注比值,提高肺动脉高压患者的生活质量,运动耐力以及存活率.其理论基础是肺动脉高压时存在肺动脉的痉挛.当前主要的肺血管扩张剂主要有钙离子拮抗剂、前列环素类药物、内皮素受体拮抗剂、磷酸二酯酶-5抑制剂和一氧化氮.现就肺动脉高压治疗中肺血管扩张剂的应用及部分药物治疗进展进行综述.     

COPD合并肺动脉高压诊治分析

目的 探讨COPD合并肺动脉高压临床诊断,常规COPD治疗与强调戒烟和氧疗疗效对比.方法本研究随机选取我院就诊确诊为COPD合并肺动脉高压的患者90例为研究对象,随机分为对照组和实验组A、B,对照组仅针对COPD进行规范化治疗,实验组A采取常规的COPD系统化治疗外还特别强调戒烟,实验组B在实验组A治疗的基础上加上无创正压通气治疗.处理6周后,观察肺动脉压的动态变化情况和动脉血气分析情况.结果 实验组A及实验组B对改善肺动脉高压及患者动脉血气上明显高于对照组,P〈0.05.实验组B与实验组A相比,患者肺动脉压降低明显,P〈0.05.结论 规范治疗COPD对COPD合并肺动脉高压患者有很好的疗效,正压通气治疗及戒烟能明显增加患者疗效,改善患者预后.     

肿瘤治疗相关性肺动脉高压研究进展

随着诊断和治疗水平的提高,肿瘤患者生存期得以延长,心血管并发症成为肿瘤患者死亡的重要原因。肿瘤引起的肺动脉高压是肿瘤患者的心血管并发症之一,导致生活质量下降和死亡风险上升。肿瘤的发生、转移和治疗等均与肺动脉高压的发生相关,其中与化学疗法、放射疗法和靶向药物治疗相关的肺动脉高压在临床中应得到重视,本文将对肿瘤治疗相关的肺动脉高压研究进展进行综述。     

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.     

结缔组织病继发肺动脉高压的临床分析

目的 总结结缔组织病(CTD)继发肺动脉高压(PAH)患者的临床特点.方法 回顾性分析1997～ 2011年复旦大学附属华山医院收治的53例结缔组织病继发肺动脉高压患者的临床资料,对患者的疾病种类、临床表现、实验室检查以及诊治和预后情况进行统计学分析.结果 1530例结缔组织病患者共发生肺动脉高压53例,其中女46例,男7例,平均年龄(43.9±13.8)岁,病程(5.1 ±4.2)年.肺动脉高压在系统性红斑狼疮中最为常见.主要临床表现为呼吸困难(84.9％)和肢端雷诺征(56.6％).肺动脉高压患者体内的ANA、nRNP和SSA抗体的比例明显升高.53例患者中7例(13.2％)死亡,死亡主要原因为右心功能衰竭.死亡者与存活者相比,肺动脉压力显著升高,动脉氧分压(PaO2)明显降低.47例患者(88.7％)使用传统的降压药治疗肺动脉高压,新型血管扩张药使用较少.结论 不同结缔组织病间肺动脉高压的发生率有较大差异.结缔组织病继发肺动脉高压的主要表现是呼吸困难和肢端雷诺征.严重肺动脉高压将影响结缔组织病患者的生存率,肺动脉压力和PaO2的检测有利于判断预后.     

Pulmonary arterial hypertension (PAH) in connective tissue diseases

Pulmonary arterial hypertension (PAH) is characterized by progressive obliteration of the small pulmonary vascular bed as a result of vascular proliferation and remodelling of the vessel wall leading to permanently increased pulmonary vascular resistance and elevated pulmonary artery pressures, which result in right heart failure and premature death. Pathologic processes behind the complex vascular changes associated with PAH include vasoconstrictor/vasodilator imbalance, thrombosis, misguided angiogenesis and inflammation. Besides idiopathic PAH, it can also occur in association with portal hypertension, HIV infection, congenital cardiac left-to-right shunts and connective tissue diseases (CTD). Unfortunately, despite recent major improvements in PAH treatment, no current therapy can yet cure this devastating condition. This review will briefly highlight epidemiology, pathogenesis, and diagnostic and treatment options known so far for PAH occurring in connection with CTD.     

肺动脉平滑肌细胞:肺动脉高压的关键治疗靶点

肺动脉高压(PAH)是一种进展快、预后欠佳、死亡率高的心血管疾病.研究表明,肺血管重构是PAH发生发展的重要病理基础,而肺动脉平滑肌细胞的增殖和肥大是PAH肺血管重构的主要病理改变.在PAH时,肺血管平滑肌细胞由收缩表型向增殖状态的合成表型转化,主要表现为肺血管平滑肌细胞的增殖和肥大.上述病理改变最终导致肺血管管腔狭窄...     

Reversible pulmonary arterial hypertension associated with interferon-beta treatment for multiple sclerosis

Interferon (IFN) therapy has an important role in the treatment of multiple sclerosis and chronic hepatitis C infection. A few case reports have described an association between IFN therapy and the development of irreversible pulmonary arterial hypertension (PAH), and it is currently listed as a possible drug-induced cause of PAH in the most recent classification of pulmonary hypertension. A causal link between IFN use and PAH remains to be elucidated; many reports of PAH resulting from IFN occur in individuals with some other risk factor for PAH. The authors present a case involving a patient with multiple sclerosis with no known risk factors for PAH, who developed severe PAH after exposure to IFN therapy. The patient experienced significant clinical and hemodynamic improvement, with normalization of her pulmonary pressures after the initiation of combination therapy for PAH. At 28 months after diagnosis, she remains asymptomatic with no hemodynamic evidence of PAH and has been off all PAH therapy for 10 months.     

Therapie der pulmonalarteriellen Hypertonie

Current international guidelines on the treatment of pulmonary arterial hypertension (PAH) are compiled by the European Society of Cardiology and the American College of Chest Physicians. The classification of pulmonary hypertension and guidelines on diagnosis and therapy were last adopted at the 4th World Congress of PAH in Dana Point (California) in the year 2008. Based on these guidelines this article presents an overview of the current therapy recommendations for patients with PAH corresponding to group 1 of the diagnostic WHO classification of pulmonary hypertension. Here it is recommended that diagnostic and therapy should be carried out in an expert centre. The therapy forms for PAH can be classified into basic therapy (e. g. oral anticoagulants, diuretics and oxygen therapy) and specific therapy (e. g. phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostanoids). Finally, some new substances will be presented which have already progressed relatively far in the clinical development.     

Pulmonary arterial hypertension and its association with HIV infection: an overview

There has been substantial progress in our understanding of the pathogenesis and clinical consequences of infection with HIV since the virus was first identified more than 20 years ago. The details of the viral replication cycle are increasingly better understood as are the identification of host elements that both regulate viral replication and are necessary for it. Greater understanding of these events has resulted in the development of therapies for HIV infection and as a consequence there has been a dramatic improvement in overall survival in the era of HAART. With this improvement in survival has come an increasing recognition of the importance of many long-term sequelae of subclinical immune deficiency and the attendant immune activation that characterize HIV infection. One complication of chronic HIV infection for which the pathogenesis is obscure is pulmonary arterial hypertension (PAH). Cases of HIV-related PAH (PAH-HIV) have been recognized with increasing frequency in recent years. Although it is likely that PAH-HIV has an underlying etiology specifically related to HIV infection, it shares several key clinical and pathological similarities with other forms of PAH. This article outlines the features, classification and treatment of PAH, and recent theories about the underlying etiology of the disease. We will also discuss the occurrence of PAH in HIV infection and propose some hypotheses regarding pathogenesis that will be covered in more detail in the accompanying articles in this supplement.     

Significance of BMPR2 mutations in pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a debilitating disease that results from progressive remodeling and inflammation of pulmonary arteries. PAH develops gradually, is difficult to diagnose, and has a high mortality rate. Although mutation in the bone morphogenetic protein receptor 2 (BMPR2) gene has been identified as the main genetic cause of PAH, the underlying pathways involving the pathophysiology of PAH are complex and still not fully understood. Endothelial dysfunction has been observed in PAH development that results in a multitude of disturbances in the cellular processes in pulmonary vessels. Changes in the pulmonary vasculature caused by the disruption of BMPR2 signaling are observed in three main vascular components; endothelial cells, smooth muscle cells, and fibroblasts. BMPR2 also has a prominent role in maintenance of the immune system. The disruption of BMPR2 signaling pathway causes an increased degree of inflammation and decreases the ability of the immune system to resolve it. Inflammatory processes and changes in pulmonary vasculature interact with one another, resulting in the progression of chronic PAH. In this review, we highlight the various components of vascular remodeling and immune response that are caused by disruption of BMPR2 signaling, including the clinical evidence and the prospects of these components as a potential target for PAH therapy. Indeed, development of drugs to target the pathogenic pathways involved in PAH may complement existing treatment regimens and improve patient prognosis.     

What’s new in the treatment of portopulmonary hypertension?

Portopulmonary hypertension (POPH) is a complication of portal hypertension characterized by pulmonary vasoconstriction and vascular remodeling that can lead to right heart failure and death. Differentiation of POPH from other causes of pulmonary hypertension, such as volume overload or a hyperdynamic high flow state, is critical because a diagnosis of POPH has significant implications for liver transplant risk stratification, Model for End Stage Liver Disease exception points, and the use of pulmonary arterial hypertension-(PAH) specific therapy. Currently, there are 12 approved medications for the treatment of PAH in the US, and three of these were approved in 2013. This review will discuss the diagnosis, evaluation and management of POPH and the role of recently approved PAH therapies in the treatment of POPH.     

Endothelin antagonism in pulmonary arterial hypertension

The pathobiology of pulmonary arterial hypertension (PAH) reflects a multifactorial process and complex evolution that involves dysfunction of underlying cellular pathways and mediators. Among these, the endothelin system has been shown to be important in the pathogenesis of PAH. Endothelin-1 (ET-1), which is found in high levels in PAH, is a known potent vasoconstrictor with proliferative vascular remodeling properties. Left unchecked, endothelin excess, along with other derangements, may contribute to the development and perpetuation of PAH. There is now substantial evidence from clinical trials and long-term data that monotherapy with an endothelin receptor antagonist (ERA) is a beneficial, therapeutic approach in PAH. Combination therapy of an ERA with a prostanoid or phosphodiesterase-5 inhibitor, two drug classes that have different mechanisms of action, is conceptually appealing, but the evidence for its efficacy and safety are still being investigated. This review provides an overview of endothelin biology and the clinical use of ERAs for the treatment of PAH. The use of ERAs for other forms of pulmonary hypertension will not be reviewed here.