Adamali Huzaifa, Gaine Sean P, Rubin Lewis J
Department of Respiratory Medicine, Mater Misericordiae University Hospital, University College Dublin, Dublin 7, Ireland.
Semin Respir Crit Care Med. 2009 Aug;30(4):484-92. doi: 10.1055/s-0029-1233317. Epub 2009 Jul 24.
Pulmonary arterial hypertension (PAH) is a sinister condition that is often diagnosed late and is associated with a progressive clinical deterioration and premature death. However, over the past 10 years the field has seen a dramatic increase in the number of effective therapies underpinned by advances in our understanding of the pathobiology of this illness. Identification of dysfunctional signaling pathways in the pulmonary circulation provided novel therapeutic targets with the goal of reducing pulmonary vascular resistance and prevention of death from right ventricular failure. Treatment algorithms have now become more complex, with therapeutic options defined by pulmonary artery vasoreactivity, functional class, and response to treatment. Combination therapies are increasingly considered as clinical trials demonstrating the efficacy of this approach are emerging. Monitoring and measuring the response to treatment in clinical trials and clinical practice are evolving from the historical dependence on functional class and 6-minute walk test to include estimates of the delay in the time to clinical worsening and the response of emerging biomarkers.
肺动脉高压（PAH）是一种凶险的疾病，常常在晚期才被诊断出来，并且与临床症状的进行性恶化和过早死亡相关。然而，在过去10年里，随着我们对这种疾病病理生物学认识的进步，该领域出现了大量有效的治疗方法。肺循环中功能失调信号通路的识别提供了新的治疗靶点，目标是降低肺血管阻力并预防右心室衰竭导致的死亡。现在治疗方案变得更加复杂，治疗选择由肺动脉血管反应性、功能分级和对治疗的反应来确定。随着证明这种方法有效性的临床试验不断涌现，联合治疗越来越受到关注。在临床试验和临床实践中，监测和衡量对治疗的反应正从以往对功能分级和6分钟步行试验的依赖，发展到包括对临床恶化时间延迟的估计以及新兴生物标志物的反应。
