Adaptation and validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Netherlands

Neth Heart J Adaptation and validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Netherlands M. Wapenaar 0 1 2 3 J. Twiss 0 1 2 3 M. Wagenaar 0 1 2 3 P. Seijkens 0 1 2 3 L. van den Toorn 0 1 2 3 J. Stepanous 0 1 2 3 A. Heaney 0 1 2 3 A. van den Bosch 0 1 2 3 K. A. Boomars 0 1 2 3 0 Department of Pulmonary Medicine, VU University Medical Center , Amsterdam , The Netherlands 1 Galen Research Ltd , Manchester , UK 2 K. A. Boomars 3 Department of Cardiology, Erasmus Medical University Center , Rotterdam , The Netherlands Background The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the first disease-specific instrument for pulmonary arterial hypertension (PAH) to assess patient-perceived symptoms, activity limitations and quality of life. To be able to use this questionnaire in the Netherlands, the aim of the study was to translate and validate this instrument for the Dutch-speaking population. Methods First the CAMPHOR was translated into Dutch (by means of a bilingual and a lay panel) and field-tested by means of cognitive debriefing interviews with ten PAH patients. For psychometric evaluation, 80 patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH) were asked to complete the CAMPHOR twice over a two-week period. To test for construct validity, participants also completed the Nottingham Health Profile (NHP). Results The Dutch version of the CAMPHOR showed high internal consistency for all scales (Cronbach's alpha 0.89-0.91) and excellent reproducibility over two weeks (reliability coefficients 0.87-0.91). Concurrent validity showed that the CAMPHOR scales correlated as expected with the NHP scales. The CAMPHOR was able to distinguish between patient groups based on self-reported general health status, disease severity and NYHA classification demonstrating evidence of known group validity. The CAMPHOR activity limitations scale correlated moderately with the distance walked during the 6-minute walk test (r = -0.47, p < 0.01) and the symptoms scale with the Borg dyspnoea score (r = 0.51, p < 0.01). Conclusion The Dutch version of the CAMPHOR is a reliable and valid measure of quality of life and health status in patients with PAH and CTEPH is recommended for use in routine care and in clinical research. Pulmonary arterial hypertension; Chronic thromboembolic pulmonary hypertension; CAMPHOR health questionnaire; Health-related quality of life - Published online: 19 May 2016 © The Author(s) 2016. This article is available at SpringerLink with Open Access Department of Pulmonary Medicine, Erasmus Medical University Center, Rotterdam, The Netherlands Background Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary vasculature, leading to increased pulmonary vascular resistance ultimately resulting in right heart failure and premature death [ 1 ]. PAH can affect persons of all ages, and females are more affected than males [ 1, 2 ]. Symptoms include breathlessness, fatigue, chest pain, palpitations, ankle oedema and syncope [1]. Unfortunately, it is not possible to cure the disease with the currently available treatments. The aim of therapy is to lengthen survival time, to ameliorate symptoms, to improve quality of life and to reduce the number of hospitalisations [ 3 ]. Chronic thromboembolic hypertension (CTEPH) is a form of precapillary PH. Patients with non-operable CTEPH suffer from the same symptoms as patients with IPAH idiopathic pulmonary arterial hypertension, HPAP heritable pulmonary arterial hypertension, PVOD pulmonary veno-occlusive disease, CTEPH chronic thromboembolic pulmonary hypertension, ERA endothelin receptor antagonist, PDE-5 inhibitor phosphodiesterase-5 inhibitor PAH and despite treatment with specific PAH medication have a poor life expectancy [ 4, 5 ]. In spite of the current treatment options, health-related quality of life (HRQL) is impaired in most patients suffering from PAH [ 6–9 ]. HRQL should be measured with an appropriate questionnaire [10]. Generic HRQL measures employed in PAH populations are of limited value in the assessment of PAH, since these do not take into account all aspects of the disease and its treatment [ 11–14 ]. Therefore, a disease-specific outcome measure for patients with PAH has been developed, the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) [15]. The questionnaire comprises three scales that assess overall symptoms (25 items), activity limitations (15 items) and quality of life (15 items). This tool is designed for use in clinical practice as well as clinical trials. This questionnaire has been used as an outcome parameter in studies concerning PAH over the last years [ 16–18 ]. The CAMPHOR health questionnaire Patients (n = 76) has been translated and validated in several languages for several countries [ 19–23 ]. This paper describes the adaptation and the validation of CAMPHOR for Dutch-speaking population in the Netherlands. (...truncated)