Pulmonary arterial hypertension.
Pulmonary arterial hypertension.
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DOI：
10.1186/1750-1172-8-97
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发表时间：
2013-07-06
影响因子：
3.7
通讯作者：
Sitbon O
中科院分区：
文献类型：
--
作者：
Montani D;Günther S;Dorfmüller P;Perros F;Girerd B;Garcia G;Jaïs X;Savale L;Artaud-Macari E;Price LC;Humbert M;Simonneau G;Sitbon O
肺动脉高压（PAH）是一种慢性进行性疾病，如果不治疗，可导致右心衰并最终死亡。PH的第一个分类于1973年提出。2008年，在达纳角（美国加利福尼亚州）举行的第四届PH世界研讨会修订了以前的分类。目前，PH被分为五个亚组。第1组包括患有特发性或家族性PAH的患者，伴有或不伴有种系突变。诊断为PAH的患者应系统地筛查BMPR2基因（骨形态发生蛋白受体2型）或更罕见的ACVRL1（活性受体样激酶1型）、ENG（内啡肽）或Smad8基因的潜在突变。肺静脉阻塞病和肺毛细血管血液病个体化，指定为临床1组。第2组“左心所致肺动脉高压”分为收缩期功能障碍、舒张期功能障碍和瓣膜功能障碍三个亚组。第3组“呼吸系统疾病引起的肺动脉高压”包括呼吸系统疾病的异质性亚组，如肺纤维化、慢性阻塞性肺病、肺气肿或肺间质性疾病引起的PH。第4组包括慢性血栓栓塞性肺动脉高压，没有近端或远端形式的区别。5组PH患者多因素机制不明确。有创性右心导管血流动力学评估被要求确认PH的明确诊断，显示静息平均肺动脉压(mPAP)≥25 mmHg和正常肺毛细血管楔压(PCWP)≤15 mmHg。评估PCWP可以区分毛细前和毛细后PH值（PCWP > 15 mmHg）。超声心动图是治疗潜在PH怀疑患者的重要工具。欧洲心脏病学会和欧洲呼吸学会（ESC-ERS）指南明确了超声心动图的作用，主要是根据三尖瓣反流峰值速度和收缩压（sPAP）筛选提出PH存在的评估标准。PAH的治疗包括口服抗凝和利尿剂等非特异性药物以及PAH特异性治疗。利尿剂是PH设置中最重要的治疗之一，因为右心衰导致液体潴留，肝充血，腹水和周围水肿。目前的建议是口服抗凝剂的目标是1.5-2.5之间的国际标准化比率（INR）。慢性血栓栓塞性PH患者的目标INR在2-3之间。在过去的四分之一个世纪中，对PH的病理生理机制的更好理解导致了医学治疗的发展，尽管目前还没有治愈多环芳烃的方法。一些特定的治疗药物被开发用于PAH的医疗管理，包括前列腺素类（epoprostenol, trepoprostenil, iloprost），内皮素受体拮抗剂（bosentan, ambrisentan）和磷酸二酯酶5型抑制剂（西地那非，他达拉非）。本文综述了目前关于PH流行病学、诊断方法和PH分类的研究现状。此外，还讨论了目前可用的PAH特异性治疗方法以及未来的治疗方法。
Pulmonary arterial hypertension (PAH) is a chronic and progressive disease leading to right heart failure and ultimately death if untreated. The first classification of PH was proposed in 1973. In 2008, the fourth World Symposium on PH held in Dana Point (California, USA) revised previous classifications. Currently, PH is devided into five subgroups. Group 1 includes patients suffering from idiopathic or familial PAH with or without germline mutations. Patients with a diagnosis of PAH should systematically been screened regarding to underlying mutations of BMPR2 gene (bone morphogenetic protein receptor type 2) or more rarely of ACVRL1 (activine receptor-like kinase type 1), ENG (endogline) or Smad8 genes. Pulmonary veno occusive disease and pulmonary capillary hemagiomatosis are individualized and designated as clinical group 1'. Group 2 'Pulmonary hypertension due to left heart diseases' is divided into three sub-groups: systolic dysfonction, diastolic dysfonction and valvular dysfonction. Group 3 'Pulmonary hypertension due to respiratory diseases' includes a heterogenous subgroup of respiratory diseases like PH due to pulmonary fibrosis, COPD, lung emphysema or interstitial lung disease for exemple. Group 4 includes chronic thromboembolic pulmonary hypertension without any distinction of proximal or distal forms. Group 5 regroup PH patients with unclear multifactorial mechanisms. Invasive hemodynamic assessment with right heart catheterization is requested to confirm the definite diagnosis of PH showing a resting mean pulmonary artery pressure (mPAP) of ≥ 25 mmHg and a normal pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg. The assessment of PCWP may allow the distinction between pre-capillary and post-capillary PH (PCWP > 15 mmHg). Echocardiography is an important tool in the management of patients with underlying suspicion of PH. The European Society of Cardiology and the European Respiratory Society (ESC-ERS) guidelines specify its role, essentially in the screening proposing criteria for estimating the presence of PH mainly based on tricuspid regurgitation peak velocity and systolic artery pressure (sPAP). The therapy of PAH consists of non-specific drugs including oral anticoagulation and diuretics as well as PAH specific therapy. Diuretics are one of the most important treatment in the setting of PH because right heart failure leads to fluid retention, hepatic congestion, ascites and peripheral edema. Current recommendations propose oral anticoagulation aiming for targeting an International Normalized Ratio (INR) between 1.5-2.5. Target INR for patients displaying chronic thromboembolic PH is between 2–3. Better understanding in pathophysiological mechanisms of PH over the past quarter of a century has led to the development of medical therapeutics, even though no cure for PAH exists. Several specific therapeutic agents were developed for the medical management of PAH including prostanoids (epoprostenol, trepoprostenil, iloprost), endothelin receptor antagonists (bosentan, ambrisentan) and phosphodiesterase type 5 inhibitors (sildenafil, tadalafil). This review discusses the current state of art regarding to epidemiologic aspects of PH, diagnostic approaches and the current classification of PH. In addition, currently available specific PAH therapy is discussed as well as future treatments.
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影响因子：
39.2
作者：
BARST, RJ;RUBIN, LJ;LEVY, PS
通讯作者：
LEVY, PS
影响因子：
10
作者：
Chaouat, A;Coulet, F;Humbert, M
通讯作者：
Humbert, M
影响因子：
3.9
作者：
Aldred, Micheala A.;Vijayakrishnan, Jairam;Trembath, Richard C.
通讯作者：
Trembath, Richard C.
影响因子：
8.9
作者：
Barst, Robyn J.;Oudiz, Ronald J.;Galie, Nazzareno
通讯作者：
Galie, Nazzareno
DOI：
10.1183/09031936.00010409
发表时间：
2009-11
期刊：
The European respiratory journal
影响因子：
--
作者：
Austin ED;Cogan JD;West JD;Hedges LK;Hamid R;Dawson EP;Wheeler LA;Parl FF;Loyd JE;Phillips JA 3rd
通讯作者：
Phillips JA 3rd