Chest
Original ResearchPulmonary Vascular DiseasePatient-Reported Outcomes Assessed by the CAMPHOR Questionnaire Predict Clinical Deterioration in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension
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Materials and Methods
This study was a retrospective analysis of data covering 2004 and 2012 obtained from the Pulmonary Vascular Disease Unit database at Papworth Hospital, corresponding to 8 years of CAMPHOR use in our practice. All patients provided written informed consent in completing questionnaires. Data collection for this study was under the approval of the local research ethics committee (Huntingdon), which approved the use of CAMPHOR in a PH population from 2004 (IRB reference H02/805).
Results
One hundred five patients with IPAH and 138 patients with CTEPH were screened, and 87 and 112, respectively, enrolled in the study. Patients were excluded for different reasons in the IPAH and CTEPH groups (Fig 1). Baseline demographics, CAMPHOR scores, and clinical data are shown in Table 1. Mean age in the IPAH group was lower, and pulmonary hemodynamics were more severe. Total CAMPHOR scores and all subscales were higher in the IPAH group, consistent with worse self-assessment of symptoms.
Discussion
The current clinical application of CAMPHOR focuses on the evaluation of patient perceptions in PH. We have shown, we believe for the first time, a novel use for the CAMPHOR questionnaire obtained at diagnosis in its ability to predict CD in patients with IPAH and CTEPH. Similar to NYHA class and 6MWD, with the univariable model, both total CAMPHOR score and its subscales appear to predict risk of CD in IPAH and CTEPH. Following adjustment for 6MWD and NYHA class, some predictive effect
Conclusions
Several international prognostic stratification tools now exist in PAH that may predict survival accurately, but these still rely on invasive tests.21, 23, 24 Increasing attention is now turning toward the physical and emotional implications of living with an incurable disease, and the IPAH and CTEPH populations should not be regarded as exceptional.28 The relatively limited response to PAH-targeted treatments with persistent high mortality rates makes the clinical incorporation of patient
Acknowledgments
Author contributions: Dr Pepke-Zaba is the overall guarantor of the manuscript.
Dr McCabe: contributed to the creation of the figures and was the principal writer of the manuscript.
Ms Bennett: contributed to the statistical design and analyses, creation of tables and figures, and writing of the manuscript.
Ms Doughty: contributed to the data gathering from the patient database and analysis of baseline patient characteristics and manuscript preparation.
Dr MacKenzie Ross: contributed to the data
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Funding/Support: This research was supported by the National Institute for Health Research (NIHR) Cambridge Biomedical Research Centre. Dr Sharples was supported by the Medical Research Council [Programme No. U015232027] and Ms Bennett holds a fellowship in clinical trials methodology from the National Institute for Health Research.
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