Jutant Etienne-Marie, Humbert Marc
Biol Aujourdhui. 2016;210(2):53-64. doi: 10.1051/jbio/2016014. Epub 2016 Sep 30.
Pulmonary hypertension (PH) is a cardio-pulmonary disorder that may involve multiple clinical conditions and can complicate the majority of cardiovascular and respiratory diseases. Its definition is an increase in mean pulmonary artery pressure (mPAP) \hbox{$\geqslant $} ⩾ 25 mmHg at rest, leading to right heart failure and ultimately death. The clinical classification of pulmonary hypertension (PH) categorizes PH into groups which share similar pathophysiological and hemodynamic characteristics and treatments. Five groups of disorders that cause PH are identified: pulmonary arterial hypertension (Group 1) which is a pre-capillary PH, defined by a normal pulmonary artery wedge pressure (PAWP) \hbox{$\leqslant $} ⩽ 15 mmH, due to remodelling of the small pulmonary arteries (<500 μm); pulmonary hypertension due to left heart disease (Group 2) which is a post-capillary PH, defined by an increased pulmonary artery wedge pressure (PAWP) >15 mmHg; pulmonary hypertension due to chronic lung disease and/or hypoxia (Group 3); chronic thrombo-embolic pulmonary hypertension (Group 4); and pulmonary hypertension due to unclear and/or multifactorial mechanisms (Group 5). PAH (PH group 1) can be treated with agents targeting three dysfunctional endothelial pathways of PAH: nitric oxide (NO) pathway, endothelin-1 pathway and prostacyclin pathway. Patients at low or intermediate risk can be treated with either initial monotherapy or initial oral combination therapy. In patients at high risk initial combination therapy including intravenous prostacyclin analogues should be considered. Patients with inadequate clinical response to maximum treatment (triple therapy with an intravenous prostacyclin) should be assessed for lung transplantation. Despite progresses, PAH remains a fatal disease with a 3-year survival rate of 58%. Treatment of group 2, group 3 and group 5 PH is the treatment of the causal disease and PAH therapeutics are not recommended. Treatment of group 4 PH is pulmonary endarteriectomy if patients are eligible, otherwise balloon pulmonary angioplasty and/or medical therapy can be considered.
肺动脉高压（PH）是一种心肺疾病，可能涉及多种临床情况，并且会使大多数心血管和呼吸系统疾病复杂化。其定义为静息时平均肺动脉压（mPAP）≥25 mmHg，导致右心衰竭并最终死亡。肺动脉高压（PH）的临床分类将其分为具有相似病理生理和血流动力学特征及治疗方法的几组。已确定导致PH的五组疾病：肺动脉高压（第1组），这是一种毛细血管前性PH，由正常肺动脉楔压（PAWP）≤15 mmHg定义，是由于小肺动脉（<500μm）重塑所致；左心疾病所致肺动脉高压（第2组），这是一种毛细血管后性PH，由肺动脉楔压（PAWP）>15 mmHg升高定义；慢性肺病和/或缺氧所致肺动脉高压（第3组）；慢性血栓栓塞性肺动脉高压（第4组）；以及机制不明和/或多因素所致肺动脉高压（第5组）。第1组肺动脉高压（PAH）可使用针对PAH三个功能失调的内皮途径的药物进行治疗：一氧化氮（NO）途径、内皮素-1途径和前列环素途径。低风险或中风险患者可采用初始单药治疗或初始口服联合治疗。对于高风险患者，应考虑初始联合治疗，包括静脉用前列环素类似物。对最大治疗（静脉用前列环素三联疗法）临床反应不佳的患者应评估是否适合肺移植。尽管取得了进展，但PAH仍然是一种致命疾病，3年生存率为58%。第2组、第3组和第5组PH的治疗是对病因性疾病的治疗，不推荐使用PAH治疗药物。第4组PH的治疗，如果患者符合条件则进行肺动脉内膜剥脱术，否则可考虑球囊肺动脉成形术和/或药物治疗。
