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Quality of life

that QoL, as assessed by the CAMPHOR questionnaire, had been able to predict clinical deterioration.20


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 6MWT) are associated with parameters reflecting patients’ daily QoL. The assessment of QoL in PAH patients is of great value because QoL 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 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

References 1. McLaughlin VV et al. End points and clinical trial design in pulmonary arterial hypertension. J Am Coll Cardiol 2009;54(Suppl. 1):S97–107.


2. Miyamoto S et al. Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension. Comparison with cardiopulmonary exercise

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6. Gabler NB et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation 2012;126:349–56.

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8. Barst RJ et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;169:441–7.

9. Pulido T et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. New Engl J Med 2013;369:809–18.

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11. Galie N et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009;30:394–403. 12. 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.

13. 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.

14. 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.

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

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

17. 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.

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19. Cenedese E et al. Measurement of quality of life in pulmonary hypertension and its significance. Eur Respir J 2006;28:808–15.

20. 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.

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