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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 HypertensionSelf-Reported Outcomes in Pulmonary Hypertension

Colm McCabe, MBBS; Maxine Bennett, MSc; Natalie Doughty, MSc; Robert MacKenzie Ross, MBBChir; Linda Sharples, PhD; Joanna Pepke-Zaba, PhD
Author and Funding Information

From the Pulmonary Vascular Disease Unit (Drs McCabe, MacKenzie Ross, and Pepke-Zaba and Ms Doughty) Papworth Hospital NHS Trust; and the MRC Biostatistics Unit (Ms Bennett and Dr Sharples), Institute of Public Health, Cambridge, England.

Correspondence to: Joanna Pepke-Zaba, PhD, Pulmonary Vascular Disease Unit, Papworth Hospital NHS Trust, Cambridge, CB23 3RE, England; e-mail: joanna.pepkezaba@papworth.nhs.uk


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.


Chest. 2013;144(2):522-530. doi:10.1378/chest.12-2443
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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.

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