title: Patient-reported outcomes assessed by the CAMPHOR questionnaire predict clinical deterioration in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.
abstract: 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.
authors: Pulmonary Vascular Disease Unit, Papworth Hospital NHS Trust, Cambridge, England.
authors: Colm
authors: C
authors: McCabe
authors: MRC Biostatistics Unit, Institute of Public Health, Cambridge, England.
authors: Maxine
authors: M
authors: Bennett
authors: Pulmonary Vascular Disease Unit, Papworth Hospital NHS Trust, Cambridge, England.
authors: Natalie
authors: N
authors: Doughty
authors: Pulmonary Vascular Disease Unit, Papworth Hospital NHS Trust, Cambridge, England.
authors: Robert
authors: R
authors: MacKenzie Ross
authors: MRC Biostatistics Unit, Institute of Public Health, Cambridge, England.
authors: Linda
authors: L
authors: Sharples
authors: Pulmonary Vascular Disease Unit, Papworth Hospital NHS Trust, Cambridge, England. Electronic address: joanna.pepkezaba@papworth.nhs.uk.
authors: Joanna
authors: J
authors: Pepke-Zaba
mesh_terms: D002908:Chronic Disease; D018450:Disease Progression; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D013923:Thromboembolism / Q000150:complications*; D016138:Walking / Q000502:physiology