Division of Pulmonary, Sleep and Critical Care Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, RI 02903, USA.
Prog Cardiovasc Dis. 2012 Sep-Oct;55(2):89-103. doi: 10.1016/j.pcad.2012.07.002.
Major advances have been made in the treatment of World Health Organization Group 1 pulmonary arterial hypertension (PAH). Since the mid-1990s, nine medications have become available in the United States to target three key pathophysiologic derangements in PAH - the prostacyclin, endothelin, and nitric oxide pathways. As a group, these agents have led to improvements in functional capacity, symptoms, hemodynamics, and survival. Most patients with mild to moderate PAH are started on orally active agents such as endothelin receptor antagonists or phosphodiesterase inhibitors. Patients with more severe disease, particularly those with evidence of right heart failure, should be treated with continuous prostacyclin infusion or a combination of a prostacyclin and oral therapy. Each medication has unique properties and clinical considerations, and the selection of an appropriate therapy must be tailored to the individual patient. None of the currently available WHO Group 1 PAH therapies are curative, however, and it is the hope that new therapies in development may halt or reverse disease progression. This review will discuss the major therapeutic classes of presently available medications and their role in managing the patient with PAH. We will also review data supporting the use of combination therapy, adjuvant background therapy, and new agents currently under investigation.
在治疗世界卫生组织第 1 组肺动脉高压 (PAH) 方面已经取得了重大进展。自 20 世纪 90 年代中期以来，美国已有九种药物可用于靶向 PAH 的三个关键病理生理异常 - 前列环素、内皮素和一氧化氮途径。作为一个整体，这些药物已导致功能能力、症状、血液动力学和生存方面的改善。大多数轻度至中度 PAH 患者开始服用口服活性药物，如内皮素受体拮抗剂或磷酸二酯酶抑制剂。病情更严重的患者，特别是有右心衰竭证据的患者，应使用持续的前列环素输注或前列环素和口服治疗的联合治疗。每种药物都有其独特的特性和临床注意事项，必须根据个体患者的情况选择合适的治疗方法。然而，目前可用的所有 1 组 PAH 治疗方法都不是治愈性的，人们希望正在开发的新疗法可能会阻止或逆转疾病进展。这篇综述将讨论目前可用药物的主要治疗类别及其在管理 PAH 患者中的作用。我们还将回顾支持联合治疗、辅助背景治疗和目前正在研究的新药物的数据。
