Badesch David B, Abman Steve H, Ahearn Gregory S, Barst Robyn J, McCrory Douglas C, Simonneau Gerald, McLaughlin Vallerie V
University of Colorado Health Sciences Center, Denver, CO 80262, USA.
Chest. 2004 Jul;126(1 Suppl):35S-62S. doi: 10.1378/chest.126.1_suppl.35S.
Pulmonary arterial hypertension (PAH) is often difficult to diagnose and challenging to treat. Untreated, it is characterized by a progressive increase in pulmonary vascular resistance leading to right ventricular failure and death. The past decade has seen remarkable improvements in therapy, driven largely by the conduct of randomized controlled trials. Still, the selection of most appropriate therapy is complex, and requires familiarity with the disease process, evidence from treatment trials, complicated drug delivery systems, dosing regimens, side effects, and complications. This chapter will provide evidence-based treatment recommendations for physicians involved in the care of these complex patients. Due to the complexity of the diagnostic evaluation required, and the treatment options available, it is strongly recommended that consideration be given to referral of patients with PAH to a specialized center.
肺动脉高压（PAH）常常难以诊断且治疗颇具挑战性。若不进行治疗，其特征为肺血管阻力逐渐增加，进而导致右心室衰竭和死亡。在过去十年中，治疗方面取得了显著进展，这在很大程度上得益于随机对照试验的开展。然而，选择最合适的治疗方法仍很复杂，需要熟悉疾病过程、治疗试验的证据、复杂的给药系统、给药方案、副作用及并发症。本章将为参与这些复杂患者护理的医生提供基于证据的治疗建议。鉴于所需诊断评估及可用治疗方案的复杂性，强烈建议将PAH患者转诊至专科中心。
