Takaoka Shanon, Faul John L, Doyle Ramona
Division of Pulmonary/Critical Care Medicine, Stanford University Medical Center, Stanford, California 94305, USA.
Semin Cardiothorac Vasc Anesth. 2007 Jun;11(2):137-48. doi: 10.1177/1089253207301356.
Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature, characterized by relentless deterioration and death. Patients with PAH are known to be at increased risk for anesthetic complications and surgical morbidity and mortality. However, outcomes in patients have improved with the recent development of new drug therapies. The 3 major drug classes for treatment of PAH are prostanoids, endothelin-receptor antagonists, and phosphodiesterase-5 inhibitors. In this review, the authors provide an overview of each drug class, its mechanism of action, indications, and current supportive literature. Surgical and interventional treatments of PAH, including atrial septostomy, pulmonary thromboendarterectomy, and transplantation, are briefly reviewed, and the rationale, indications, and selection criteria for each are discussed. Although available medical and surgical therapies for PAH have improved patient outcomes, acute decompensated right heart failure (RHF) remains a common and challenging complication of PAH. The authors review this topic and provide an outline of the general pathophysiology of RHF and an approach to its management.
肺动脉高压（PAH）是一种肺血管的进行性疾病，其特征是病情持续恶化并导致死亡。已知PAH患者发生麻醉并发症以及手术并发症和死亡率增加的风险更高。然而，随着新药疗法的近期发展，患者的预后已有所改善。治疗PAH的三大类药物是前列环素类、内皮素受体拮抗剂和磷酸二酯酶-5抑制剂。在本综述中，作者概述了每类药物、其作用机制、适应证以及当前的支持性文献。简要回顾了PAH的手术和介入治疗，包括房间隔造口术、肺动脉血栓内膜切除术和移植，并讨论了每种治疗的基本原理、适应证和选择标准。尽管现有的PAH药物和手术疗法改善了患者的预后，但急性失代偿性右心衰竭（RHF）仍然是PAH常见且具有挑战性的并发症。作者对该主题进行了综述，并概述了RHF的一般病理生理学及其管理方法。
