Mathai Stephen C, Ghofrani Hossein-Ardeschir, Mayer Eckhard, Pepke-Zaba Joanna, Nikkho Sylvia, Simonneau Gérald
Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA
University of Giessen and Marburg Lung Center, Giessen, Germany, member of the German Center for Lung Research (DZL).
Eur Respir J. 2016 Aug;48(2):526-37. doi: 10.1183/13993003.01626-2015. Epub 2016 Apr 13.
Patients with chronic thromboembolic pulmonary hypertension (CTEPH) experience debilitating symptoms that have a negative impact on their quality of life (QoL) in terms of physical capability, psychological wellbeing and social relationships. The use of QoL measurement tools is important in the assessment of treatment efficacy and in guiding treatment decisions. However, despite the importance of QoL, particularly to the patient, it remains under-reported in clinical studies of CTEPH therapy. CTEPH is unique in pulmonary hypertension in that it is potentially curable by surgery; however, a proportion of patients either have residual PH following surgery or are not operable. Although some patients with CTEPH have been treated off-label with pulmonary arterial hypertension-specific therapies, there have been few randomised controlled trials of these therapies in patients with CTEPH. Moreover, in these trials QoL outcomes are variably assessed, and there is little consistency in the tools used. Here we review the assessment of QoL in patients with CTEPH and the tools that have been used. We also discuss the effect of surgical intervention and medical therapies on QoL. We conclude that further studies of QoL in patients with CTEPH are needed to further validate the optimal QoL tools.
慢性血栓栓塞性肺动脉高压（CTEPH）患者会出现使人虚弱的症状，这些症状在身体能力、心理健康和社会关系方面对其生活质量（QoL）产生负面影响。生活质量测量工具的使用对于评估治疗效果和指导治疗决策很重要。然而，尽管生活质量很重要，尤其是对患者而言，但在CTEPH治疗的临床研究中，它的报告仍然不足。CTEPH在肺动脉高压中是独特的，因为它有可能通过手术治愈；然而，一部分患者术后仍有残余肺动脉高压，或者无法进行手术。尽管一些CTEPH患者接受了肺动脉高压特异性疗法的超说明书治疗，但针对这些疗法在CTEPH患者中的随机对照试验很少。此外，在这些试验中，生活质量结果的评估各不相同，所使用的工具也几乎没有一致性。在此，我们回顾了CTEPH患者生活质量的评估以及所使用的工具。我们还讨论了手术干预和药物治疗对生活质量的影响。我们得出结论，需要对CTEPH患者的生活质量进行进一步研究，以进一步验证最佳的生活质量工具。
