Abstract

Individuals with pulmonary arterial hypertension (PAH) experience severely impaired quality of life. A disease-specific patient reported outcome measure for PAH (the Cambridge Pulmonary Hypertension Outcome Review--CAMPHOR) has recently been developed and validated in the UK, USA and Canada. It has demonstrated reliability and validity in PAH populations in these countries. The aim of this study was to assess the reliability and validity of the CAMPHOR in an Australian and New Zealand (NZ) PAH population. Semistructured interviews were conducted with a cohort of 15 PAH patients (aged 68.9±10.0 years; 11 women) to determine the relevance of the CAMPHOR and ensure the terminology and language used was understandable and appropriate for our PAH population. The test-retest reliability, internal consistency and construct validity of the CAMPHOR were then examined in an Australian and NZ PAH population (n=61, aged 56.9±14.5 years; 48 women). Data from the patient interviews confirmed that the CAMPHOR is appropriate for use in our PAH population. The three CAMPHOR scales (symptoms, activity limitations and quality of life) had excellent test-retest reliability (correlation coefficients (r(s))=0.86-0.94, P<0.01) and internal consistency (Cronbach's alpha coefficients=0.89-0.92). The CAMPHOR also demonstrated the ability to distinguish between individuals with PAH who differed according to World Health Organisation functional class. We have shown the CAMPHOR to be valid and reliable in an Australian and NZ PAH population and recommend its use in clinical practice.

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