title: Quality of life in patients with chronic thromboembolic pulmonary hypertension.
abstract: 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.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA smathai4@jhmi.edu.
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Mathai
authors.affiliation: University of Giessen and Marburg Lung Center, Giessen, Germany, member of the German Center for Lung Research (DZL).
authors.forename: Hossein-Ardeschir
authors.initials: HA
authors.lastname: Ghofrani
authors.affiliation: Kerckhoff Heart and Lung Center, Bad Nauheim, Germany.
authors.forename: Eckhard
authors.initials: E
authors.lastname: Mayer
authors.affiliation: Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, UK.
authors.forename: Joanna
authors.initials: J
authors.lastname: Pepke-Zaba
authors.affiliation: Global Clinical Development, Bayer Pharma AG, Berlin, Germany.
authors.forename: Sylvia
authors.initials: S
authors.lastname: Nikkho
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Service de Pneumologie, Hôpital Bicêtre, Université Paris-Sud, Laboratoire d'Excellence en Recherche sur le Médicament et Innovation Thérapeutique, and INSERM Unité 999, Le Kremlin-Bicêtre, France.
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
mesh_terms: D004691:Endarterectomy; D005081:Exercise Therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000503:physiopathology / Q000523:psychology*; D007398:Interpersonal Relations; D017063:Outcome Assessment, Health Care; D000071066:Patient Reported Outcome Measures; D011594:Psychometrics; D011655:Pulmonary Embolism / Q000503:physiopathology / Q000523:psychology; D011788:Quality of Life; D016032:Randomized Controlled Trials as Topic; D012107:Research Design; D012919:Social Behavior; D013923:Thromboembolism / Q000503:physiopathology / Q000523:psychology*