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
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
References (28)
- et al.
An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry
Chest
(2012) - et al.
Surgical management and outcome of patients with chronic thromboembolic pulmonary hypertension: results from an international prospective registry
J Thorac Cardiovasc Surg
(2011) - et al.
Factors that prognosticate mortality in idiopathic pulmonary arterial hypertension: a systematic review of the literature
Respir Med
(2010) - et al.
United States validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)
J Heart Lung Transplant
(2008) - et al.
Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial
Lancet
(2008) - et al.
Depressive symptoms in pulmonary arterial hypertension: prevalence and association with functional status
Psychosomatics
(2010) - et al.
Survival after pulmonary thromboendarterectomy: effect of residual pulmonary hypertension
J Thorac Cardiovasc Surg
(2011) - et al.
Pulmonary arterial hypertension
Circulation
(2006) - et al.
A meta-analysis of randomized controlled trials in pulmonary arterial hypertension
Eur Heart J
(2009) - et al.
The Bosentan Patient Registry: long-term survival in pulmonary arterial hypertension
Intern Med J
(2011)
The value of tools to assess pulmonary arterial hypertension
Eur Respir Rev
Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT)
Eur Heart J
Measurement of quality of life in pulmonary hypertension and its significance
Eur Respir J
Chronic obstructive pulmonary disease patient well-being and its relationship with clinical and patient-reported outcomes: a real-life observational study
Respiration
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2021, Journal of Heart and Lung TransplantationCitation Excerpt :Because PROs emphasize the patient's perception of symptoms, use of a PRO tool can provide critical information in conjunction with a telemedical evaluation. McCabe et al45 retrospectively applied the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) tool on patients with idiopathic PAH (IPAH) and CTEPH to assess the predictability of clinical decline. At study enrollment, total CAMPHOR scores demonstrated a significant predictive effect on clinical decline in patients with IPAH and CTEPH (hazard ratios: 1.03 [95% CI: 1.01–1.05] and 1.04 [95% CI: 1.02–1.06], respectively, per unit score increase).
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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