PT  - JOURNAL ARTICLE
AU  - Stephen C. Mathai
AU  - Hossein-Ardeschir Ghofrani
AU  - Eckhard Mayer
AU  - Joanna Pepke-Zaba
AU  - Sylvia Nikkho
AU  - Gérald Simonneau
TI  - Quality of life in patients with chronic thromboembolic pulmonary hypertension
AID  - 10.1183/13993003.01626-2015
DP  - 2016 Apr 13
TA  - European Respiratory Journal
PG  - ERJ-01626-2015
4099  - http://erj.ersjournals.com/content/early/2016/04/13/13993003.01626-2015.short
4100  - http://erj.ersjournals.com/content/early/2016/04/13/13993003.01626-2015.full
AB  - 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.HRQoL is rarely measured in CTEPH trials: further studies are needed as well as validation of appropriate HRQoL tools http://ow.ly/ZrkL1