Hôpital Antoine Béclère, Université Paris-Sud 11, Clamart, France.
Eur Respir Rev. 2010 Dec;19(118):272-8. doi: 10.1183/09059180.00008210.
Major advances have occurred in the treatment of pulmonary arterial hypertension (PAH) over the past decade. The advent of PAH-specific pharmacological treatments has offered hope to patients with a debilitating, progressive disease and a poor prognosis. Combined drug treatment offers improved benefits over monotherapy, and current treatment guidelines for PAH recommend a sequential add-on approach to combination therapy for patients in New York Heart Association (NYHA)/World Health Organization functional class (WHO FC) II-IV. Goal-oriented therapy determines the timing of treatment escalation by inadequate response to known prognostic indicators. Close monitoring of patients aids the early identification of inadequate response, so that treatment can be escalated promptly and before the patient's condition deteriorates further. Existing treatment goals are based on baseline values of prognostic indicators, but it is vital to identify risk factors that are both relevant during treatment and that can be assessed during follow-up appointments. Data from different PAH aetiologies indicate that NYHA/WHO FC is the most appropriate prognostic marker, with 6-min walk distance and several haemodynamic parameters representing alternatives. Future refinement of goal-oriented therapy could include the use of multiple prognostic markers, while additional, large clinical trials will answer questions concerning choice and combination of treatment goals.
在过去的十年中,肺动脉高压 (PAH) 的治疗取得了重大进展。肺动脉高压特异性药物治疗的出现为患有这种使人衰弱、进行性疾病和预后不良的患者带来了希望。联合药物治疗比单一疗法提供了更好的益处,目前的 PAH 治疗指南建议对纽约心脏协会 (NYHA)/世界卫生组织功能分类 (WHO FC) II-IV 级的患者采用序贯加用方法进行联合治疗。以目标为导向的治疗通过对已知预后指标的反应不足来确定治疗升级的时机。密切监测患者有助于及早发现治疗反应不足,以便及时升级治疗,防止病情进一步恶化。现有的治疗目标是基于预后指标的基线值,但确定在治疗期间相关且可在随访期间评估的危险因素至关重要。来自不同 PAH 病因的数据表明,NYHA/WHO FC 是最合适的预后标志物,6 分钟步行距离和几个血流动力学参数是替代标志物。以目标为导向的治疗的进一步改进可能包括使用多个预后标志物,而更多的大型临床试验将回答关于治疗目标选择和联合的问题。