Department of Pharmacy and Therapeutics, University of Pittsburgh School of Pharmacy, UPMC Presbyterian Hospital, 727 Salk Hall, 3501 Terrace Street, Pittsburgh, PA, 15261, USA.
University of Michigan, Ann Arbor, MI, USA.
Curr Cardiol Rep. 2019 Nov 22;21(11):141. doi: 10.1007/s11886-019-1235-4.
Pulmonary arterial hypertension (PAH) leads to progressive increases in pulmonary vascular resistance (PVR), right heart failure, and death if left untreated. This review will summarize and discuss recent updates in the classification and management of patients with PAH.
PAH requires careful hemodynamic assessment and is defined by a mean pulmonary artery pressure > 20 mmHg with normal left-sided filling pressures and a PVR ≥ 3 Wood units. Most patients with PAH require targeted pharmacotherapy based on multiparametric risk stratification. Significant improvements in clinical outcome have been realized through the approval of 14 unique pharmacotherapeutic options. The latest clinical recommendations provide the updated hemodynamic definition and clinical classification as well as evidence-based treatment recommendations. An important change is the focus on initial upfront combination therapy for most patients with PAH. Structured follow-up and escalation of treatment for those not achieving low-risk status is paramount.
肺动脉高压（PAH）可导致肺血管阻力（PVR）进行性增加，如果不进行治疗，可导致右心衰竭和死亡。本综述将总结和讨论 PAH 患者分类和管理的最新进展。
PAH 需要仔细的血流动力学评估，定义为平均肺动脉压＞20mmHg，同时左心充盈压正常，PVR≥3 伍德单位。大多数 PAH 患者需要基于多参数风险分层的靶向药物治疗。通过批准 14 种独特的药物治疗方案，临床结局得到了显著改善。最新的临床建议提供了更新的血流动力学定义和临床分类以及基于证据的治疗建议。一个重要的变化是关注大多数 PAH 患者的初始联合治疗。对于那些未达到低危状态的患者，进行结构化的随访和治疗升级至关重要。
