Cambridge Pulmonary Hypertension Outcome Review Research Articles

Psychometric performance of the CAMPHOR and SF-36 in pulmonary hypertension

BackgroundThe Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and the Medical Outcomes Study Short Form 36 (SF-36) are widely used to assess patient-reported outcome in individuals with pulmonary hypertension (PH). The aim of the study was to compare the psychometric properties of the two measures.MethodsParticipants were recruited from specialist PH centres in Australia and New Zealand. Participants completed the CAMPHOR and SF-36 at two time points two weeks apart. The SF-36 is a generic health status questionnaire consisting of 36 items split into 8 sections. The CAMPHOR is a PH-specific measure consisting of 3 scales; symptoms, activity limitations and needs-based QoL. The questionnaires were assessed for distributional properties (floor and ceiling effects), internal consistency (Cronbach's alpha), test-retest reliability and construct validity (scores by World Health Organisation functional classification).ResultsThe sample comprised 65 participants (mean (SD) age = 57.2 (14.5) years; n(%) male = 14 (21.5%)). Most of the patients were in WHO class 2 (27.7%) and 3 (61.5%). High ceiling effects were observed for the SF-36 bodily pain, social functioning and role emotional domains. Test-retest reliability was poor for six of the eight SF-36 domains, indicating high levels of random measurement error. Three of the SF-36 domains did not distinguish between WHO classes. In contrast, all CAMPHOR scales exhibited good distributional properties, test retest reliability and distinguished between WHO functional classes.ConclusionsThe CAMPHOR exhibited superior psychometric properties, compared with the SF-36, in the assessment of PH patient-reported outcome.

Functional and Quality of Life Improvements in Treated Patients with Chronic Thromboembolic Pulmonary Hypertension

Purpose Chronic thromboembolic hypertension (CTEPH) is a form of pulmonary hypertension (PH) best treated surgically, and evidence in support of targeted therapy in inoperable patients is weak. This study aimed to demonstrate the effects of targeted therapy in patients with CTEPH in whom pulmonary endarterectomy (PEA) was not feasible. Methods and Materials A retrospective audit of CTEPH patients in the Newcastle PH Clinic was conducted. Six-minute walk distance (6MWD), Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) scores, and pulmonary hemodyamics were collected. Changes in 6MWD and CAMPHOR six months after intervention (surgery or starting of targeted therapies) were evaluated. Patients were stratified as inoperable patients (distal disease, patient preference, and comborid conditions), patients with residual PH post-PEA, or those with no PH post-PEA. Means were compared using student’s t-test; p-values Results Of 160 patients diagnosed with CTEPH, serial CAMPHOR and 6MWD data have been collected on the last 82, of which 37 were inoperable and on targeted therapy (Group A), 12 underwent PEA with residual PH (Group B), and 33 underwent PEA with no residual PH (Group C). Baselines were similar, except 6MWD and age between Group A and Group C (mean 241m vs 325m, p=0.003 and 64.4 vs 54.5 years, p=0.002). Mean 6MWD increased 49 m in Group A, 67 m in Group B, and 109 m in Group C (p Conclusions In inoperable CTEPH, targeted therapy can improve 6MWD and WHO functional class, but perhaps not quality of life. In contrast, patients undergoing PEA with normalization of pulmonary hemodynamics have improved 6MWD, WHO functional class, and quality of life.

Benefits of Intensive Treadmill Exercise Training on Cardiorespiratory Function and Quality of Life in Patients With Pulmonary Hypertension

Pulmonary hypertension (PH) restricts the ability to engage in physical activity and decreases longevity. We examined the impact of aerobic exercise training on function and quality of life in patients with World Health Organization group 1 PH. Patients were randomized to a 10-week education only (EDU) or education/exercise combined (EXE) group. The exercise program consisted of 24-30 sessions of treadmill walking for 30-45 min per session at 70% to 80% of heart rate reserve. Outcome variables included changes in 6-min walk test (6MWT) distance, time to exercise intolerance, peak work rate (WR) from a cardiopulmonary treadmill test, and quality-of-life measures, including the Short Form Health Survey, version 2 (SF-36v2) and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). Data are presented as mean SD. Twenty-three women (age, 54 11 years; BMI, 31 7 kg/m 2 ) were randomized to the EDU (n 5 13) or EXE (n 5 10) groups. Following 10 weeks of intervention, patients in the EXE group demonstrated an improvement in 6MWT distance (56 45 m; P 5 .002), increased time to exercise intolerance (1.9 1.3 min; P 5 .001), and peak WR (26 23 W; P 5 .004). Additionally, the EXE group scored significantly ( P , .050) better on six of the eight scales on SF-36v2, and fi ve of the six scales on CAMPHOR. In contrast, no significant improvement was observed for any of the outcome measures following EDU. No adverse events were noted in either group. Ten weeks of brisk treadmill walking improved 6MWT distance, cardiorespiratory function, and patient-reported quality of life in female patients with group 1 PH.

Rapid Transition from Inhaled Iloprost to Inhaled Treprostinil in Patients with Pulmonary Arterial Hypertension

BackgroundInhaled treprostinil is a prostacyclin analog approved for the treatment of pulmonary arterial hypertension (PAH) that may provide a more convenient treatment option for patients receiving inhaled iloprost while maintaining the clinical benefit of inhaled prostacyclin therapy.AimsIn this open-label safety study, 73 PAH patients were enrolled with primarily World Health Organization Class II (56%) or III (42%) symptoms. At baseline, most patients (93%) were receiving 5 μg of iloprost per dose but 38% of patients reported a dosing frequency below the labeled rate of 6–9 times daily. Patients initiated inhaled treprostinil at 3 breaths four times daily (qid) at the immediate next scheduled iloprost dose. The primary objective was to assess the safety of rapid transition from iloprost to inhaled treprostinil; clinical status and quality of life were also assessed.ResultsMost patients (84%) achieved the target treprostinil dose of 9 breaths qid and remained on study until transition to commercial therapy (89%). The most frequent adverse events (AEs) were cough (74%), headache (44%), and nausea (30%), and five patients prematurely discontinued study drug due to AE (n = 3), disease progression (n = 1), or death (n = 1). At week 12, the time spent on daily treatment activities was reduced compared to baseline, with a mean total savings of 1.4 h per day. Improvements were also observed at week 12 for 6-min walk distance (+16.0; P < 0.001), N-terminal pro-B-type natriuretic peptide (−74 pg/mL; P = 0.001), and the Cambridge Pulmonary Hypertension Outcome Review (all domains P < 0.001).ConclusionsPulmonary arterial hypertension patients can be safely transitioned from inhaled iloprost to inhaled treprostinil while maintaining clinical status.

The Polish adaptation of the CAMbridge Pulmonary Hypertension Outcome Review (CAMPHOR).

Pulmonary hypertension (PH) results in severely impaired quality of life (QoL) in people with this condition. The CAMbridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the only questionnaire providing a disease-specific measurement of symptoms, functioning and QoL in PH patients. It has already been adapted for use in several countries. The aim of this study was to adapt and validate CAMPHOR for the Polish-speaking population. Two panels (bilingual and lay) were conducted to translate CAMPHOR into Polish. This new version was then tested by cognitive debriefing interviews with 15 patients. Finally, a postal validation survey was conducted with 56 patients on two occasions 2 weeks apart to assess its psychometric properties. No problems were experienced in producing a Polish translation of CAMPHOR. Interviewees responded well to the Polish CAMPHOR, finding it relevant, comprehensible and easy to complete. For all three CAMPHOR scales (Symptoms, Activity, QoL), The Cronbach alpha coefficients were above 0.8 at both time points, indicating high internal consistency. Test-retest reliability for the three scales achieved a value above 0.80. Predicted correlations with the Nottingham Health Profile provided evidence of the construct validity of CAMPHOR scales. The Polish CAMPHOR could distinguish between patients who differed according to their perceived general health and perceived disease severity. No significant differences in scores were found between participants grouped by gender or age. The Polish version of CAMPHOR demonstrated good psychometric properties and is recommended for use in clinical practice.

PCV92 Comparison of the Psychometric Properties of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and the SF-36 in Patients with Pulmonary Hypertension

PCV92 Comparison of the Psychometric Properties of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and the SF-36 in Patients with Pulmonary Hypertension

Adaptation and validation of the Cambridge pulmonary hypertension outcome review for Sweden

The Cambridge pulmonary hypertension outcome review (CAMPHOR) is the first pulmonary hypertension-specific instrument for the assessment of the patient's perceived symptoms, activity limitations and quality of life (QoL). To produce and validate a Swedish language version of the CAMPHOR. Bilingual (n = 5) and lay panels (n = 5) were conducted to translate the CAMPHOR into Swedish. This new questionnaire was then field-tested with 14 patients and finally, it underwent psychometric evaluation by means of a postal validation study involving 38 patients with pulmonary hypertension (PH). Few problems were experienced in translating the CAMPHOR into Swedish. The field-test participants found the scales relevant, comprehensible and easy to complete. Psychometric analyses showed that the Swedish adaptation was successful. The Swedish CAMPHOR scales had good internal consistency. Cronbach's alpha coefficients were 0.92 for the symptoms scale, 0.92 for activity limitations and 0.95 for the quality of life. Predicted correlations with the Nottingham Health Profile provided evidence of the construct validity of the scales. The Swedish scales also indicated known groups validity. The Swedish version of the CAMPHOR is a reliable and valid measure of the impact of pulmonary hypertension on the lives of affected patients. It is recommended for use in clinical studies and routine practice in pulmonary hypertension patients.

The German adaptation of the Cambridge pulmonary hypertension outcome review (CAMPHOR)

BackgroundIndividuals with precapillary pulmonary hypertension (PH) experience severely impaired quality of life. A disease-specific outcome measure for PH, the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) was developed and validated in the UK and subsequently adapted for use in additional countries. The aim of this study was to translate and assess the reliability and validity of the CAMPHOR for German-speaking populations.MethodsThree main adaptation stages involved; translation (employing bilingual and lay panels), cognitive debriefing interviews with patients and validation (assessment of the adaptation’s psychometric properties). The psychometric evaluation included 107 patients with precapillary PH (60 females; age mean (standard deviation) 60 (15) years) from 3 centres in Austria, Germany and Switzerland.ResultsNo major problems were found with the translation process with most items easily rendered into acceptable German. Participants in the cognitive debriefing interviews found the questionnaires relevant, comprehensive and easy to complete. Psychometric analyses showed that the adaptation was successful. The three CAMPHOR scales (symptoms, activity limitations and quality of life) had excellent test-retest reliability correlations (Symptoms = 0.91; Activity limitations = 0.91; QoL = 0.90) and internal consistency (Symptoms = 0.94; Activity limitations = 0.93; QoL = 0.94). Predicted correlations with the Nottingham Health Profile provided evidence of the construct validity of the CAMPHOR scales. The CAMPHOR adaptation also showed known group validity in its ability to distinguish between participants based on perceived general health, perceived disease severity, oxygen use and NYHA classification.ConclusionsThe CAMPHOR has been shown to be valid and reliable in the German population and is recommend for use in clinical practice.

Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: Results from a cross-sectional patient survey

Pulmonary arterial hypertension (PAH) is a complex disease with variable clinical manifestations; nevertheless, morbidity and mortality associated with PAH are considerable. This study examined quality of life (QOL) in PAH patients and assessed use of palliative care (PC) for addressing QOL issues and what barriers might exist regarding early PC implementation for patients with PAH. An Internet-based survey was distributed to Pulmonary Hypertension Association patient-related listservs. Symptom burden and QOL were assessed using Linear Analog Self Assessment (LASA) QOL items and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). Of 774 eligible patients with active e-mail addresses, 315 returned surveys (41% overall response), and 276 (88%) contained analyzable responses. Responders (mean age, 48.9 years ± 16.0) were predominantly white (85%), female (86%), and with idiopathic PAH (42%). Profound deficiency in overall QOL (40%), fatigue (57%), physical well-being (56%), social activity (49%), emotional well-being (49%), and pain (38%) were reported. Most patients believed their PAH physician had excellent understanding of PAH progression/plan of care (92%), but less were satisfied with care regarding QOL management (77%). Few patients considered PC (8%), or had pain management (4%) or PC involved (1%). Most common reasons were beliefs that patients were doing well/not sick (63%) or that PC had not been suggested (22%). PAH may result in symptoms or QOL impairment persisting despite optimal PAH therapy. However, PC awareness or use by PAH patients and providers is low. Opportunities may exist to integrate PC into care for PAH patients.

Predictors of Health-Related Quality of Life in Patients With Idiopathic Pulmonary Arterial Hypertension

Idiopathic pulmonary arterial hypertension is a debilitating disease that leads to right ventricular heart failure and death. There is limited research on how this disease affects patients. The purpose of this study was to describe differences in health status, health-related quality of life, and psychological states among functional classes along with predictors of health-related quality of life. A convenience sample of 104 participants with idiopathic pulmonary arterial hypertension was recruited. Participants completed a sociodemographic and clinical data form; Medical Outcomes Study Short-Form 36, US Cambridge Pulmonary Hypertension Outcome Review, and Profile of Mood States surveys. The mean age was 51.9 (SD, 16.6) years, and 81 (78%) were female. Patients with functional class IV had significantly worse self-reported physical health status, health-related quality of life, and psychological distress than the other functional classes. Predictors of health-related quality of life were role-emotional (Medical Outcomes Study Short-Form 36), symptoms and activity (US Cambridge Pulmonary Hypertension Outcome Review), depression (Profile of Mood States), employment, and diuretic and oxygen use, accounting for 86% of the variance. These factors need to be monitored and assessed with patients with idiopathic pulmonary arterial hypertension especially as functional class increases. Effective symptom management is necessary to reduce the negative impact on health-related quality of life. A case study is presented to illustrate these points.

Cluster analysis of symptoms in pulmonary arterial hypertension: a pilot study

Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressures leading to right heart failure and death. The aim of this study was to use cluster analysis to describe the symptom profile in PAH and differences in the health outcomes of health status, health-related quality of life (HRQoL) and psychological states in the cluster groups. A cross-sectional descriptive design was used. A convenience sample completed a socio-demographic and clinical data form, a PAH Symptom Severity and Interference Scale, the Medical Outcomes Study Short Form (SF-36), the United States Cambridge Pulmonary Hypertension Outcome Review (US CAMPHOR) and the Short Form of the Profile of Mood States (POMS). Of the 151 participants, the mean age was 53.5 ± 15.1 with the majority female (n = 128, 85%). Fifty-eight (41%) were disabled and 67 (44%) were Functional Class IV. The most prevalent symptoms were shortness of breath with exertion (n = 149, 99%) and fatigue (n = 144, 93%). Three clusters emerged: Cluster 1 diffuse symptoms (n = 93), Cluster 2 severe cardiopulmonary symptoms (n = 32) and Cluster 3 moderate cardiopulmonary symptoms (n = 26). Overall, on the SF-36 the participants had poor general health, reduced physical function, role physical, vitality, and a low composite score for physical health. On the POMS the sample had limited vigor and increased fatigue. Cluster 2 Severe Cardiopulmonary Symptoms had worse SF-36, US CAMPHOR and POMS scores than the other cluster groups. Patients with PAH are experiencing a constellation of symptoms that are interfering with their life and emerging clusters were present.

S21 Objective and patient reported outcomes of long term management of patients with chronic thromboembolic pulmonary hypertension (CTEPH): a single centre experience

ObjectiveTo determine long-term objective and Patient Reported Outcomes at a single centre in patients with CTEPH (distal or residual post Pulmonary endarterectomy).MethodA retrospective study of all incident patients with CTEPH...

S22 Improved symptoms and quality of life after pulmonary endarterectomy (PEA) in patients with chronic thromboembolic disease (CTED) and borderline pulmonary hypertension (PH)

Background Chronic thromboembolic pulmonary hypertension (CTEPH) is defined by a mean pulmonary artery pressure (mPAP) =25 mm Hg at right heart catheterisation in patients with multiple chronic/organised occlusive thrombi in the pulmonary arteries. PEA is potentially curable and is the treatment of choice for patients with CTEPH. There is a small group of patients with CTED and exertional dyspnoea but with a resting mPAP that does not fulfil criteria for PH in whom PEA may be considered. The outcome of PEA in these patients has not been previously assessed. Objective To assess the post PEA functional and haemodynamic outcomes in patients with symptomatic CTED and borderline PH. Methods Retrospective data were collected on patients with baseline mPAP of =25 mm Hg who underwent PEA at the UK and Ireland national referral centre between 2002 and 2010. Patients were reassessed 3 and 12 months after surgery. Right heart catheterisation was performed at baseline and 3 months post PEA. Results 558 patients underwent PEA at Papworth Hospital between 2002 and 2010. From those, 16 had symptomatic operable CTED and mPAP of =25 mm Hg. Mean age was 45±17 and 69% were female. All survived surgery and are currently alive at follow-up. The median length of stay was 13 days (IQR 6). Results are displayed in the Abstract S22 table 1 as mean ± SD. WHO class data are expressed as percentages. CAMPHOR (Cambridge Pulmonary Hypertension Outcome Review) is a disease specific quality of life questionnaire and scores are expressed as median. Conclusion In this small series of patients with CTED and borderline PH, there was a significant symptomatic, haemodynamic and functional benefit from PEA at 1-year. Further research is required to assess the prognostic benefit in this population. We would like to acknowledge the national pulmonary hypertension centres in the UK and Ireland, and support by the Cambridge NIHR Comprehensive Biomedical Research Centre.

Measures of systemic sclerosis (scleroderma): Health Assessment Questionnaire (HAQ) and Scleroderma HAQ (SHAQ), Physician‐ and Patient‐Rated Global Assessments, Symptom Burden Index (SBI), University of California, Los Angeles, Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT) 2.0, Baseline Dyspnea Index (BDI) and Transition Dyspnea Index (TDI) (Mahler's Index), Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), and Raynaud's Condition Score (RCS)

Measures of systemic sclerosis (scleroderma): Health Assessment Questionnaire (HAQ) and Scleroderma HAQ (SHAQ), Physician‐ and Patient‐Rated Global Assessments, Symptom Burden Index (SBI), University of California, Los Angeles, Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT) 2.0, Baseline Dyspnea Index (BDI) and Transition Dyspnea Index (TDI) (Mahler's Index), Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), and Raynaud's Condition Score (RCS)

Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Australian and New Zealand population

Individuals with pulmonary arterial hypertension (PAH) experience severely impaired quality of life. A disease-specific patient reported outcome measure for PAH (the Cambridge Pulmonary Hypertension Outcome Review--CAMPHOR) has recently been developed and validated in the UK, USA and Canada. It has demonstrated reliability and validity in PAH populations in these countries. The aim of this study was to assess the reliability and validity of the CAMPHOR in an Australian and New Zealand (NZ) PAH population. Semistructured interviews were conducted with a cohort of 15 PAH patients (aged 68.9±10.0 years; 11 women) to determine the relevance of the CAMPHOR and ensure the terminology and language used was understandable and appropriate for our PAH population. The test-retest reliability, internal consistency and construct validity of the CAMPHOR were then examined in an Australian and NZ PAH population (n=61, aged 56.9±14.5 years; 48 women). Data from the patient interviews confirmed that the CAMPHOR is appropriate for use in our PAH population. The three CAMPHOR scales (symptoms, activity limitations and quality of life) had excellent test-retest reliability (correlation coefficients (r(s))=0.86-0.94, P<0.01) and internal consistency (Cronbach's alpha coefficients=0.89-0.92). The CAMPHOR also demonstrated the ability to distinguish between individuals with PAH who differed according to World Health Organisation functional class. We have shown the CAMPHOR to be valid and reliable in an Australian and NZ PAH population and recommend its use in clinical practice.

Use of non-invasive haemodynamic measurements to detect treatment response in precapillary pulmonary hypertension

BackgroundHaemodynamic measurements may be superior to the 6-min walk distance (6MWD) as outcome measures in pulmonary hypertension (PH) as they are directly linked to the mechanisms of disease and are...

303 A Pilot Observational Study of the Correlation between Depression and Quality of Life Measured by the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)

303 A Pilot Observational Study of the Correlation between Depression and Quality of Life Measured by the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)

305 Adaptation and Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for Sweden

305 Adaptation and Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for Sweden

Sleep Quality, Depression, and Quality of Life in Patients with Pulmonary Hypertension

Pulmonary hypertension is a disabling disease characterized by progressive functional worsening, right heart failure, and death. Although pulmonary hypertension has been associated with poor quality of life, sleep quality has not been investigated in pulmonary hypertension patients. This was a cross-sectional study in which patients (N=40) were asked to complete standardized questionnaires to assess sleep quality [using Pittsburgh Sleep Quality Index (PSQI)], insomnia, sleepiness, dyspnea, depression, restless leg syndrome, and quality of life [using Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR)] during routine office visits. Baseline hemodynamics, pulmonary function tests, exercise capacity, and transthoracic echocardiogram were analyzed. Pulmonary hypertension functional class was World Health Organization class II [20 (50%)], III [18 (45%)], and IV [2 (5%)], and 29 (72.5%) had poor sleep quality. PSQI score was associated with CAMPHOR symptoms score (R=0.61, P<0.001), CAMPHOR activities score (R=0.38, P=0.016), CAMPHOR quality-of-life score (R=0.45, P=0.004), depression (R=0.42, P=0.007), and dyspnea (R=0.36, P=0.02). Sleep quality was not associated with age, gender, other comorbidities, pulmonary hypertension etiology, baseline hemodynamics, pulmonary function testing, echocardiographic parameters, or exercise capacity. Poor sleep quality is common in patients with pulmonary hypertension and correlates with depression, dyspnea, and poor quality of life. Improving sleep quality in patients with pulmonary hypertension may improve quality of life.

Exercise Training in Patients with Pulmonary Arterial Hypertension: A Case Report

To describe the benefits of a feasible, outpatient exercise training program on exercise tolerance and health-related quality of life (HRQL) in individuals with pulmonary arterial hypertension (PAH). Case report on two subjects recruited from a tertiary care pulmonary hypertension clinic. Subject 1 was a 50-year-old male with idiopathic PAH. Subject 2 was a 54-year-old female with a 20+ year history of scleroderma and 6-year history of PAH. Both subjects underwent exercise training 3 times per week for 6 weeks using a cycle ergometer at workloads progressing from 50% to 80% of peak workload. Outcomes were assessed using cardiopulmonary exercise testing, six-minute walk test (6MWT), and HRQL using the Chronic Respiratory Disease Questionnaire (CRQ) and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). Both subjects made substantial improvements in oxygen consumption and workload at anaerobic threshold (improvements of 3.8 and 4.2 mL.kg(-1).min,(-1) 26 and 18 W, respectively) and 6MWT distance (from 496 to 586m and 416 to 517m, respectively). Only Subject 1 made substantial improvements in peak oxygen consumption (from 16.0 to 18.3 mL.kg(-1).min(-1)and from 15.0 to 15.6 mL.kg(-1).min,(-1) respectively) and peak work rate (from 112 to 130W and 66 to 69W, respectively). Both subjects demonstrated improved HRQL. No adverse events were noted. A short and practical exercise training program can improve measures of workload, aerobic capacity, and HRQL in individuals with PAH with no adverse effects shown in these two case studies.