Dept of Experimental, Diagnostic and Specialty Medicine (DIMES), Alma Mater Studiorum, University of Bologna, Bologna, Italy.
Pulmonary and Critical Care Division, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Eur Respir J. 2019 Jan 24;53(1). doi: 10.1183/13993003.01889-2018. Print 2019 Jan.
Pulmonary arterial hypertension (PAH) remains a severe clinical condition despite the availability over the past 15 years of multiple drugs interfering with the endothelin, nitric oxide and prostacyclin pathways. The recent progress observed in medical therapy of PAH is not, therefore, related to the discovery of new pathways, but to the development of new strategies for combination therapy and on escalation of treatments based on systematic assessment of clinical response. The current treatment strategy is based on the severity of the newly diagnosed PAH patient as assessed by a multiparametric risk stratification approach. Clinical, exercise, right ventricular function and haemodynamic parameters are combined to define a low-, intermediate- or high-risk status according to the expected 1-year mortality. The current treatment algorithm provides the most appropriate initial strategy, including monotherapy, or double or triple combination therapy. Further treatment escalation is required in case low-risk status is not achieved in planned follow-up assessments. Lung transplantation may be required in most advanced cases on maximal medical therapy.
尽管在过去的 15 年中已经有多种药物可以干预内皮素、一氧化氮和前列环素途径，但肺动脉高压 (PAH) 仍然是一种严重的临床情况。因此，PAH 治疗方面的最新进展并不是由于新途径的发现，而是由于联合治疗的新策略的发展以及基于临床反应的系统评估来升级治疗。目前的治疗策略基于新诊断的 PAH 患者的严重程度，通过多参数风险分层方法进行评估。根据预期的 1 年死亡率，将临床、运动、右心室功能和血流动力学参数相结合，以确定低、中或高风险状态。目前的治疗算法提供了最合适的初始策略，包括单药治疗、双药或三药联合治疗。如果在计划的随访评估中未达到低风险状态，则需要进一步升级治疗。在大多数晚期病例中，肺移植可能是最大程度药物治疗的必要手段。
