Comparison of cardiac and pulmonary-specific quality-of-life measures in pulmonary arterial hypertension

Dept of Medicine, University of California, San Francisco, CA 94143-0924, USA.
European Respiratory Journal (Impact Factor: 7.64). 08/2011; 38(3):608-16. DOI: 10.1183/09031936.00161410
Source: PubMed


Studies systematically comparing the performance of health-related quality-of-life (HRQoL) instruments in pulmonary arterial hypertension (PAH) are lacking. We sought to address this by comparing cardiac and respiratory-specific measures of HRQoL in PAH. We prospectively assessed HRQoL in 128 patients with catheterisation-confirmed PAH at baseline and at 6, 12 and post-24 month follow-up visits. Cardiac-specific HRQoL was assessed using the Minnesota Living with Heart Failure Questionnaire (LHFQ); respiratory-specific HRQoL was assessed using the Airways Questionnaire 20 (AQ20); and general health status was assessed using the 36-item Short Form physical component summary (SF-36 PCS). The LHFQ and AQ20 were highly intercorrelated. Both demonstrated strong internal consistency and converged with the SF-36 PCS. Both discriminated patients based on World Health Organization (WHO) functional class, 6-min walking distance (6MWD) and Borg dyspnoea index (BDI), with the exception of a potential floor effect associated with low 6MWD. The LHFQ was more responsive than the AQ20 to changes over time in WHO functional class, 6MWD and BDI. In multivariate analyses, the LHFQ and AQ20 were each longitudinal predictors of general health status, independent of functional class, 6MWD and BDI. In conclusion, both cardiac-specific and respiratory-specific measures appropriately assess HRQoL in most patients with PAH. Overall, the LHFQ demonstrates stronger performance characteristics than the AQ20.

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    • "However, to complement such trial endpoints, rigorous measurement of HRQoL related to PAH is also recommended to ensure treatments and interventions improve not just objective functional capacity, but also the day-to-day well-being of patients. To date, HRQoL measurement in trials has typically relied on generic measures which may not fully evaluate the specific impacts experienced by PAH patients [10,11]. Few disease-specific measures of quality of life (QoL) in PAH exist and are limited by the lack of explicitly defined responsiveness and clinical utility [12]. "
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    ABSTRACT: The Living with Pulmonary Hypertension questionnaire (LPH) was adapted from the Minnesota Living with Heart Failure Questionnaire for use in patients with pulmonary arterial hypertension (PAH). Study objectives were to confirm the face and content validity, to assess the structure and psychometric properties, and provide guidance for the interpretation of the LPH. A qualitative interview study was conducted with PAH patients in the US (n=12), Germany (n=14) and France (n=12) to evaluate the face and content validity of the LPH. Psychometric validation was performed using blinded data from a double blind, Phase III, clinical trial (n=196). Validation analyses were performed on baseline and week 12 (visit 6/last visit) data and included evaluation of: item response distributions, quality of completion, construct validity, reliability, clinical validity and responsiveness. Analyses to provide an estimation of the Minimal Important Difference (MID) for the LPH scores were performed. Cognitive debriefing interviews with 38 PAH patients indicated that the most commonly reported PAH symptoms and impacts are covered by LPH items. Patients found the LPH questionnaire relevant and comprehensive to their experience. Some suggestions were made to enhance the face validity of the LPH. The content validity of the questionnaire was supported. Results of the psychometric validation analyses (n=190) indicated that the LPH Emotional and Physical scores met the criteria for convergent and discriminant validity; for the total score all but two items met the test for item convergent validity. Internal consistency reliability was demonstrated by Cronbach's alpha values of >0.70 for all LPH scores. The LPH Physical and Total scores discriminated between World Health Organisation (WHO) Functional classes and 6 Minute walk test distances, indicating clinical validity and were also responsive to change in clinical severity, as measured by change in WHO functional class and Borg CR 10 Scale. Further investigation is required to confirm the responsiveness of the Emotional score. Estimation of MID using distribution-based methods indicated a change of 3 points for the sub-scales and 7 for the total score to be clinically meaningful. The LPH is a valid and reliable instrument that meets FDA criteria.
    Health and Quality of Life Outcomes 10/2013; 11(1):161. DOI:10.1186/1477-7525-11-161 · 2.12 Impact Factor
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    • "Other researchers have investigated the functioning of the SF-36 physical (PCS) and mental (MCS) component summaries in PH patients [28]. Chen et al reported low levels of end effects for the MCS and PCS scales. "
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    ABSTRACT: The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and the Medical Outcomes Study Short Form 36 (SF-36) are widely used to assess patient-reported outcome in individuals with pulmonary hypertension (PH). The aim of the study was to compare the psychometric properties of the two measures. Participants were recruited from specialist PH centres in Australia and New Zealand. Participants completed the CAMPHOR and SF-36 at two time points two weeks apart. The SF-36 is a generic health status questionnaire consisting of 36 items split into 8 sections. The CAMPHOR is a PH-specific measure consisting of 3 scales; symptoms, activity limitations and needs-based QoL. The questionnaires were assessed for distributional properties (floor and ceiling effects), internal consistency (Cronbach's alpha), test-retest reliability and construct validity (scores by World Health Organisation functional classification). The sample comprised 65 participants (mean (SD) age = 57.2 (14.5) years; n(%) male = 14 (21.5%)). Most of the patients were in WHO class 2 (27.7%) and 3 (61.5%). High ceiling effects were observed for the SF-36 bodily pain, social functioning and role emotional domains. Test-retest reliability was poor for six of the eight SF-36 domains, indicating high levels of random measurement error. Three of the SF-36 domains did not distinguish between WHO classes. In contrast, all CAMPHOR scales exhibited good distributional properties, test retest reliability and distinguished between WHO functional classes. The CAMPHOR exhibited superior psychometric properties, compared with the SF-36, in the assessment of PH patient-reported outcome.
    BMC Pulmonary Medicine 07/2013; 13(1):45. DOI:10.1186/1471-2466-13-45 · 2.40 Impact Factor
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    • "Some trials have applied a modified version of the Minnesota Living with Heart Failure (MLWHF) questionnaire [13,14]. Although this has been shown to have a reasonable performance [15,16] it was not designed for patients with PH. "
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    ABSTRACT: Background Individuals with precapillary pulmonary hypertension (PH) experience severely impaired quality of life. A disease-specific outcome measure for PH, the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) was developed and validated in the UK and subsequently adapted for use in additional countries. The aim of this study was to translate and assess the reliability and validity of the CAMPHOR for German-speaking populations. Methods Three main adaptation stages involved; translation (employing bilingual and lay panels), cognitive debriefing interviews with patients and validation (assessment of the adaptation’s psychometric properties). The psychometric evaluation included 107 patients with precapillary PH (60 females; age mean (standard deviation) 60 (15) years) from 3 centres in Austria, Germany and Switzerland. Results No major problems were found with the translation process with most items easily rendered into acceptable German. Participants in the cognitive debriefing interviews found the questionnaires relevant, comprehensive and easy to complete. Psychometric analyses showed that the adaptation was successful. The three CAMPHOR scales (symptoms, activity limitations and quality of life) had excellent test-retest reliability correlations (Symptoms = 0.91; Activity limitations = 0.91; QoL = 0.90) and internal consistency (Symptoms = 0.94; Activity limitations = 0.93; QoL = 0.94). Predicted correlations with the Nottingham Health Profile provided evidence of the construct validity of the CAMPHOR scales. The CAMPHOR adaptation also showed known group validity in its ability to distinguish between participants based on perceived general health, perceived disease severity, oxygen use and NYHA classification. Conclusions The CAMPHOR has been shown to be valid and reliable in the German population and is recommend for use in clinical practice.
    Health and Quality of Life Outcomes 09/2012; 10(1):110. DOI:10.1186/1477-7525-10-110 · 2.12 Impact Factor
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