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Quality of life


improving QoL in patients with PAH. Besides the assessment of therapy success in clinical trials, QoL is increasingly considered in the daily management of PAH. One important example is ‘shared decision making’,26 which reflects the interaction between patient and physician in order to develop a personalised, individually tailored management strategy. Individual patient needs may differ significantly. It is the physicians’ duty to recognise and support them. In addition, the goals of physicians and patients may not necessarily be the same: physicians are trained to identify prognostic relevant parameters and try to improve them, leading sometimes to the ‘treatment’ of haemodynamic or laboratory parameters instead of treating the patient. Shared decision making may even lead to the acceptance of a worsening of such parameters or to the decision against further therapy escalation if it is reasonable from the patient’s QoL point of view. This approach is well known within oncology and is being increasingly adapted in patients with severe cardiovascular diseases. A specific issue within this process is end of life care. This issue is discussed in the actual PH guidelines and it is recognised that open and sensitive communication with patients may allow advanced planning and discussion of their fears, concerns and wishes, which is essential for good care in all patients.13


In addition,


non-pharmacological interventions may serve an important purpose in providing the emotional support that has been shown to be fundamental to the wellbeing of patients with PAH and their families.27


Conclusions 18


What role functional endpoints play in the daily life of PAH patients is an important question. Based on the discussed considerations, we may conclude that several functional parameters (including 6MWD) are associated with parameters reflecting patients’ daily QoL. The assessment of QoL in PAH patients is of great value because QoL has proved to be of prognostic relevance and it helps to determine minimal important clinical differences of functional tests. This facilitates the interpretation of their results and provides a connection to the daily life of patients. Clinically meaningful endpoints representing


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patients’ daily QoL, such as TTCW, are now used as primary endpoints of clinical trials, allowing real-life conclusions in clinical studies to be drawn. l


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12. Galie N et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015;373(9):834–44.


13. Galie N et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for


European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 2016;37(1): 67–119.


14. Sitbon O et al; GRIPHON investigators. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 2015;373(26): 2522–33.


15. McKenna SP et al. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR): a measure of health-related quality of life and quality of life for patients with pulmonary hypertension. Qual Life Res 2006;15:103–15.


16. Chua R et al. Comparison and validation of three measures of quality of life in patients with pulmonary hypertension. Intern Med J 2006;36:705–10.


17. Yorke J et al. emPHasis-10: development of a health-related quality of life measure in pulmonary hypertension. Eur Respir J 2014;43:1106–13.


18. Kingman M et al. Living with pulmonary hypertension: unique insights from an international ethnographic study. BMJ Open 2014;4:e004735-2013-004735.


19. Halank M et al. Exercise capacity affects quality of life in patients with pulmonary hypertension. Lung 2013;191:337–43.


20. Talwar A et al. Dyspnea, depression and health related quality of life in pulmonary arterial hypertension patients. J Exerc Rehabil 2015;11(5):259–65.


21. Strange G et al. Bosentan therapy in patients with pulmonary arterial hypertension: the relationship between improvements in 6 minute walk distance and quality of life. Respirology 2008;13:674–82.


22. Pepke-Zaba J et al. Sildenafil improves health-related quality of life in patients with pulmonary arterial hypertension. Chest 2008;133:183–9.


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24. McCabe C et al. Patient-reported outcomes assessed by the CAMPHOR questionnaire predict clinical deterioration in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Chest 2013;144:522–30.


25. Arena R et al. The effect of exercise training on the pulmonary arterial system in patients with pulmonary hypertension. Prog Cardiovasc Dis 2015;57(5):480–8.


26. Howard LS, Ferrari P, Mehta S. Physicians’ and patients’ expectations of therapies for pulmonary arterial hypertension: where do they meet? Eur Respir Rev 2014;23(134):458–68.


27. Delcroix M, Howard L. Pulmonary arterial hypertension: the burden of disease and impact on quality of life. Eur Respir Rev 2015;24(138): 621–9.


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