Chest
Volume 144, Issue 2, August 2013, Pages 522-530
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Original Research
Pulmonary Vascular Disease
Patient-Reported Outcomes Assessed by the CAMPHOR Questionnaire Predict Clinical Deterioration in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

https://doi.org/10.1378/chest.12-2443Get rights and content

Background

The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is a disease-specific assessment tool used for the evaluation and follow-up of patients with pulmonary hypertension (PH). We describe a novel use for this questionnaire in its potential to predict clinical deterioration (CD) in two patient cohorts with subtypes of PH, idiopathic pulmonary arterial hypertension (IPAH), and chronic thromboembolic pulmonary hypertension (CTEPH) during an 8-year period.

Methods

We retrospectively analyzed CAMPHOR scores obtained at baseline and at follow-up visits in patients under the care of our unit over an 8-year period to assess CD and survival, as well as 6-min walk distance (6MWD) and New York Heart Association (NYHA) class.

Results

Using Cox regression, we demonstrated a significant predictive effect of CD from total CAMPHOR scores at study enrollment in IPAH and CTEPH (hazard ratios, 1.03 [95% CI, 1.01-1.05] and 1.04 [95% CI, 1.02-1.06] per unit score increase, respectively), as well as from CAMPHOR subscales as independent predictors. This predictive effect is diluted after adjusting for the prognostic effect of 6MWD and NYHA class. Repeated CAMPHOR assessment over time appears not to add predictive value of CD to that obtained at diagnosis, although it still informs physicians of important changes in self-reported symptoms.

Conclusions

When emphasis is placed on the evaluation of patient perceptions, CAMPHOR may represent an alternative method of estimating the likelihood of CD.

Section snippets

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.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.

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