Patient-Reported Outcomes Assessed by the CAMPHOR Questionnaire Predict Clinical Deterioration in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

doi:10.1378/chest.12-2443

Chest

Volume 144, Issue 2, August 2013, Pages 522-530

https://doi.org/10.1378/chest.12-2443 Get 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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Clinical Utility of Patient-Reported Outcome Instruments in the Management of Pulmonary Hypertension: A Systematic Review
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Despite the greater sensitivity and specificity of disease-specific patient-reported outcome measures (PROM) to detect clinical change, only recently have such instruments been developed for pulmonary hypertension (PH), specifically pulmonary arterial hypertension (PAH) and chronic thromboembolic disease (CTEPH). Although these valuable tools are now being incorporated into clinical studies of PH, they have not yet reached widespread integration into routine clinical care.
In this systematic review, the authors assess the psychometric properties of PROM developed for PH, compare PROM with other clinical outcomes in PH, and address the utility of PROM in clinical care.
The authors performed a systematic search of papers published between January 1, 2006, and October 1, 2022, using the MEDLINE database to identify PROM developed and validated for PH. The identified PROM were found to have been developed only in groups with PAH and CTEPH. The authors evaluated the identified instruments according to established psychometric criteria. An additional search was performed to identify randomized controlled trials (RCTs) utilizing these PROM for comparison with clinical outcomes.
From 527 papers retrieved, a total of 35 PROM were identified. Of these, 5 disease-specific instruments were included in the final analysis. While both CAMPHOR (Cambridge Pulmonary Hypertension Outcome Review) and emPHasis-10 performed well in patients with PAH and CTEPH with regard to their psychometric properties, emPHasis-10 demonstrated superior feasibility for use in clinical practice due to its concise format. The Pulmonary Arterial Hypertension–Symptoms and Impacts Questionnaire performed well in the authors’ analysis, though additional data is needed regarding interpretability and feasibility.
EmPHasis-10 demonstrated strong psychometric properties and the greatest feasibility for clinical use. Further study assessing the integration of PROM into routine clinical care for PH is needed.
Patient-Reported Outcomes Instruments: The Important Balance Between Feasibility and Analysis
2024, JACC: Heart Failure
Considerations When Selecting Patient-Reported Outcome Measures for Assessment of Health-Related Quality of Life in Patients With Pulmonary Hypertension: A Narrative Review
2022, Chest
Citation Excerpt :
All four PROMs were content validated in samples of patients with PAH,16-19,22 whereas validation samples for CAMPHOR, PAH-SYMPACT, and emPHasis-10 also included patients with CTEPH.16,18,23 Table 416-18,22,24-55 summarizes findings related to the evaluation of psychometric properties for PH-specific PROMs. Detailed descriptions of findings for each PROM are provided in e-Appendix 3.
It is well established that pulmonary hypertension (PH) places a substantial burden on patients’ health-related quality of life (HRQoL). As more effective treatments have been developed for this condition, evaluating treatment benefit based on experiences reported by patients regarding their well-being and physical, social, and emotional functioning has increased. A review of the published literature and clinical trials in PH was conducted to identify and evaluate patient-reported outcome measures (PROMs) that assess PH-specific HRQoL for use in clinical studies. The Cambridge Pulmonary Hypertension Outcome Review, emPHasis-10, Living with Pulmonary Hypertension Questionnaire, and Pulmonary Arterial Hypertension—Symptoms and Impact were selected for in-depth evaluation with respect to their content validity, psychometric properties, interpretation guidelines, conceptual coverage, and administrative feasibility. Recommendations for clinical study end point strategies are provided. The review identified many strengths for each of the PROMs. Content development for all PROMs followed best practices, and any weaknesses in assessment of measurement properties were from a scarcity of available data. Although conceptual coverage and patient burden varied greatly across the PROMs, each provided a unique strength relative to the others, and no one PROM was recommended as most appropriate across all contexts of use. Optimal end point selection for assessing PH-specific HRQoL thus requires consideration of the purpose and situation in which the assessment will be conducted. These recommendations should be considered as a snapshot of a quickly evolving landscape that should be updated as new information emerges.
The longitudinal use of EmPHasis-10 and CAMPHOR questionnaire health-related quality of life scores in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension
2021, Respiratory Medicine
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Since the EmPHasis-10 score consists of only 10 questions, it might be easier applicable in daily clinical practice than the CAMPHOR questionnaire [18]. Both the EmPHasis-10 and CAMPHOR questionnaire are shown to be of prognostic value [16,21–23]. The aim of this study is to investigate the longitudinal validity and responsiveness of the CAMPHOR and EmPHasis-10 questionnaires and to establish the minimal clinically important differences (MCID) for both questionnaires.
Health-related quality of life (HRQoL) is impaired in patients with pulmonary hypertension (PH). The EmPHasis-10 and CAMPHOR questionnaires are developed to evaluate HRQoL specifically in patients with PH. Data on the longitudinal use of both questionnaires are still limited. We evaluated the longitudinal value of both questionnaires and established minimal clinically important differences (MCID).
Sixty-one treatment naïve pulmonary arterial hypertension or chronic thromboembolic patients were prospectively included. Patients were treated according to the current ESC/ERS guidelines. We compared EmPHasis-10 and CAMPHOR scores between baseline, 6 and 12 months of follow-up and evaluated the correlation between these scores and a 5-scale symptom severity score, 5-scale overall health score, NYHA-classification, 6 min walk test distance (6MWD), NT-proBNP and echocardiographic parameters.
After one year of treatment a significant reduction in EmPHasis-10 score and CAMPHOR QoL and symptoms domain score was observed. Moderate to good correlations were observed between the questionnaires and the overall-health and symptom severity score and 6MWD. No relevant correlations were seen between the questionnaires and NT-pro-BNP and echocardiographic parameters. EmPHasis-10 scores showed strong correlations with all CAMPHOR domains. The MCID for the EmPHasis-10 questionnaire was −8. The MCIDs for the CAMPHOR domains were: activity −3, symptoms −4, QoL −3.
The EmPHasis-10 and CAMPHOR questionnaires are valid tools for the longitudinal measurement of HRQoL in patients with PH. The much shorter EmPHasis-10 correlates well with the CAMPHOR domain scores and with the clinical endpoints and it may be easier to use in daily practice.
Risk assessment in patients with pulmonary arterial hypertension in the era of COVID 19 pandemic and the telehealth revolution: State of the art review
2021, Journal of Heart and Lung Transplantation
Citation 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).
Patients affected by pulmonary arterial hypertension (PAH) benefit from intensive, continuous clinical monitoring to guide escalation of treatments that carry the potential to improve survival and quality of life. During the coronavirus disease 2019 pandemic, the need for physical distancing has fueled the expeditious expansion of various telehealth modalities, which may apply in a unique manner to individuals with PAH. Performance of objective risk assessments in patients with PAH remotely via telemedical visits and other telehealth mechanisms is unprecedented and not yet rigorously validated. The uniquely high risk for rapid deterioration in patients with PAH demands a high degree of sensitivity to detect changes in functional assessments. In this review, several telehealth modalities for potential utilization in risk assessment and treatment titration in patients with PAH are explored, yet additional study is needed for their validation with the pre-pandemic care paradigm.

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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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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

Background

Methods

Results

Conclusions

Section snippets

Materials and Methods

Results

Discussion

Conclusions

Acknowledgments

Chest

J Thorac Cardiovasc Surg

Respir Med

J Heart Lung Transplant

Lancet

Psychosomatics

J Thorac Cardiovasc Surg

Pulmonary arterial hypertension

Circulation

A meta-analysis of randomized controlled trials in pulmonary arterial hypertension

Eur Heart J

The Bosentan Patient Registry: long-term survival in pulmonary arterial hypertension

Intern Med J

The value of tools to assess pulmonary arterial hypertension

Eur Respir Rev

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

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