Cambridge Pulmonary Hypertension Outcome Review Research Articles

The optimal cut-off value for the one-minute sit-to-stand test to determine functional impairment in patients with pulmonary hypertension

Abstract Introduction Pulmonary hypertension (PH) often leads to reduced functional capacity, commonly assessed by the six-minute walk test (6MWT). The 1-min sit-to-stand test (1-min STST) is a shorter test with smaller space requirements. Despite its potential utility, to our knowledge no studies have determined the optimal cut-off value for the 1-min STST in patients with PH. Aim This study aimed to assess the optimal cut-off value for the 1-min STST by utilizing three established 6MWT thresholds (165m, 320m, and 440m) recommended in current European PH guidelines published by Humbert et al. (1). Methods A receiver operating characteristics (ROC) curve analysis was conducted to identify a threshold for the 1-min STST, aligning with the three 6MWT cut-offs. Clinical parameters and health-related quality of life (HRQoL) assessed through the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire were evaluated. Results A total number of 114 patients with PH (mean age 66±14 years, 57% female) were included in the analysis. Patients performed a mean number of 17±7 repetitions in the 1-min STST and achieved a mean 6MWT distance of 351±137m. The <165m 6MWT threshold revealed the highest area under the curve (AUC=0.90; 95%-CI 0.84-0.97). The optimal cut-off for the 1-min STST was 14.5 repetitions, exhibiting 69% sensitivity and 100% specificity. Patients below this threshold demonstrated significantly worse clinical parameters and reported worse HRQoL (p<0.001), as shown in Table 1. Conclusion A 1-min STST cut-off of 14.5 repetitions identified PH patients with functional impairment, suggesting its potential utility for risk stratification.

Impact of preoperative body mass index on long-term survival, quality of life, and functional outcomes after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: Results from the UK National Cohort

BackgroundPrevious studies have demonstrated the safety of pulmonary endarterectomy (PEA) in all body mass index (BMI) strata. However, long-term survival and patient-reported quality of life outcome measures by BMI strata remain unknown. We sought to examine the impact of preoperative BMI on long-term survival, quality of life, and functional outcomes for patients undergoing PEA for chronic thromboembolic pulmonary hypertension (CTEPH). MethodsRetrospective review of 2004 patients from the UK National Cohort between 2007 and 2021 undergoing PEA for CTEPH (mPAP>20 mmHg and PVR>160 dynes). Patients were stratified into BMI<20, 20-29, 30-39, 40-49, and 50+. All-cause mortality was the primary outcome measure. Secondary outcome measures were 3-6 month postoperative hemodynamics, 6-minute walk distance (6MWD), New York Heart Association (NYHA) class, and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) scores. Kruskal-Wallis, ANOVA, and Kaplan Meier Estimate were used for comparisons. ResultsPostoperative hemodynamics and 6MWD at 3-6 months were similar across BMI strata. However, patients with BMI 50+ reported the highest incidence of post-operative NYHA III/IV limitation (53.3%, p<0.001) and the highest residual symptom burden by CAMPHOR (p<0.001). Overall 5-year survival was lowest in patients with BMI 50+ (70.2%) and BMI<20 (73.4%), whilst highest in BMI 30-39 (88.2%, p=0.008). 10-year Kaplan-Meier Estimates predicted lowest survival in BMI 50+ and BMI<20. ConclusionPEA remains safe and effective for all patients regardless of BMI. Despite similar hemodynamic outcomes, patients with BMI 50+ are at greatest risk of long-term all-cause mortality and patients with BMI 50+ experience residual symptomatic limitation.

The one-minute sit-to-stand-test performance is associated with health-related quality of life in patients with pulmonary hypertension.

Patients with pulmonary hypertension (PH) have an impaired functional capacity and poor health-related quality of life (HRQoL). The one-minute sit-to-stand test (1-min STST) can be used for the assessment of functional capacity. Our aim was to evaluate the 1-min STST performance and its association with patient-reported HRQoL in patients with PH. We prospectively assessed functional capacity in 98 PH patients (mean age 66 ± 15 years, 55% female) using the 1-min STST. Patients had to stand up and sit down from a chair as many times as possible within one minute. Patients' HRQoL was evaluated with the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire, which consists of the three subcategories symptoms, activities and quality of life (QoL). We observed a significant correlation of the 1-min STST performance with all HRQoL subcategories assessed with the CAMPHOR questionnaire: A lower number of 1-min STST repetitions correlated with more symptoms (rs = -.398, p < .001), worse functioning (rs = -.551, p < .001) and a decreased QoL (rs = -.407, p < .001). Furthermore, in the multivariable linear regression analysis, adjusted for age, sex, body mass index (BMI) and mean pulmonary artery pressure (mPAP), lower 1-min STST performance was an independent predictor for worse symptoms (est. β = -0.112, p = .003), activities (est. β = -0.198, p < .001) and QoL (est. β = -0.130, p < .001) assessed with the CAMPHOR questionnaire. Our results indicate that regardless of age, sex, BMI and mPAP the 1-min STST performance is associated with all CAMPHOR HRQoL subcategories in patients with PH. Therefore, the 1-min STST performance might be a new option to assess functional capacity correlated to HRQoL in patients with PH.

Change in health-related quality of life at early follow-up in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.

Symptoms associated with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) impact patient's health-related quality of life (HRQoL). Studies on change and if a minimal clinically important difference (MCID) in HRQoL is reached within a year after diagnosis are lacking. The aim was to investigate the change in HRQoL as well as the proportion of patients that reached MCID at an early postdiagnosis visit. The study included adult patients from the Swedish PAH &CTEPH registry,diagnosed 2008-2021, with Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) at time of diagnosis and a follow-up. Data were analyzed as total population and dichotomized for sex, age (<65 vs. ≥65 years), time of diagnosis (≤2015 vs. >2015) and pulmonary hypertension (PH)subgroups. Data reported as median, interquartile range (IQR), and proportions (%). There were 151 patients (PAH = 119, CTEPH = 32) with an available CAMPHOR score at diagnosis and follow-up. CAMPHOR total sum was 31 (IQR: 21-43) and 25 (14-36); (p < 0.001) at diagnosis and follow-up, respectively. At follow-up, 56% had reached MCID in total sum, while for domains activity, symptoms, and QoL 27%, 33%, and 39% reached MCID, respectively. These results were independent of PH subgroup, diagnosis before or after 2015 and sex. Age below 65 years was related to improvements in activity and worsening of symptoms. In conclusion on a group level, improvements in CAMPHOR total sum as well as all domains were seen in the first year after diagnosis, however, only slightly more than half of the patients reached MCID for CAMPHOR total sum.

The one-minute sit-to-stand test (1-min STST) and its relationship to quality of life (QoL) in patients with pulmonary hypertension (PH)

Abstract Introduction Patients with pulmonary hypertension (PH) have a decreased functional capacity and poor quality of life (QoL). The one-minute sit-to-stand test (1-min STST) can be potentially used for functional capacity assessment, but there is no data to date on its relationship to QoL in PH patients. Aims Our aim was to find out if 1-min STST performance correlates to patient-reported QoL. Methods 106 PH patients (mean age 66 ± 15 years, 57% female) were prospectively assessed for cardiorespiratory fitness using the 1-min STST. The patients had to stand-up and sit-down from a chair as many times as possible within one minute. Patients QoL was evaluated with the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire. Results Mean number of 1-min STST repetitions was 16 ± 6.5 and mean CAMPHOR score was 26 ± 19. Patients with worse 1-min STST performance reported significantly worse QoL concerning the total score and all the sub-scores of the CAMPHOR questionnaire: They had a higher total score (r = -0.533, p &amp;lt; 0.001) and regarding sub-scores more symptoms: (r = -0.457, p &amp;lt; 0.001), worse functioning (r = -0.524, p &amp;lt; 0.001) and decreased QoL (r = -0.505, p &amp;lt; 0.001). Also, in multivariable linear regression analysis, adjusted for age, sex and BMI, lower 1-min STST performance was an independent predictor for worse QoL (β⁠ = -1.5, p &amp;lt; 0.001). Conclusion Our results indicate that regardless of age, sex and BMI the 1-min STST performance is associated with QoL in patients with PH.

Clinical Utility of Patient-Reported Outcome Instruments in the Management of Pulmonary Hypertension: A Systematic Review

BackgroundDespite 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. ObjectivesIn 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. MethodsThe 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. ResultsFrom 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. ConclusionsEmPHasis-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 Long-Term Outcome of Balloon Pulmonary Angioplasty for Inoperable CTEPH.

Balloon pulmonary angioplasty (BPA) is a promising interventional treatment for inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Evidence in favor of BPA is growing, but long-term data remain scarce. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is validated for the assessment of patients with pulmonary hypertension within three domains: symptoms, activity, and quality of life (QoL). The aim of the present study was to evaluate the long-term effects of BPA on these domains in patients with inoperable CTEPH. Between March 2014 and August 2019, technically inoperable patients with target lesions for BPA were included in this prospective, observational study. CAMPHOR scores were compared between baseline (before the first BPA) and 6 months after the last intervention and also for scores assessed at annual follow-ups. A total of 152 patients had completed a full series of BPA interventions and a 28 (interquartile range [IQR]: 26-32) week follow-up. Further follow-up assessments including the CAMPHOR score were performed 96 (IQR: 70-117) weeks, 178 (IQR: 156-200) weeks, and 250 (IQR: 237-275) weeks after the last intervention. From baseline to the last follow-up, CAMPHOR scores for symptoms, activity, and QoL improved from 9 (IQR: 6-14) to 3 (IQR: 0-9) (p < 0.001), 8 (IQR: 5-12) to 4 (IQR: 2-8) (p < 0.001), and 5 (IQR: 2-9) to 1 (IQR: 0-5) (p < 0.001). BPA leads to long-lasting, significant improvement of symptoms, physical capacity, and QoL in inoperable CTEPH patients.

Quality of life and functional status after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension: A Swedish single-center study.

Little is known about long-term quality of life (QOL) and functional status after pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH). We investigated QOL and functional status late after PEA. All patients who underwent PEA for CTEPH 1993-2020 at one Swedish center were included. Baseline characteristics and data from right heart catheterization, 6-min walk test, and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) were obtained from patient charts and national registers. The RAND 36-Item Health Survey was sent by post, and Karnofsky Performance Status (KPS) was evaluated by telephone. A total of 110 patients were included. The survey was completed by 49/66 (74%) patients who were alive in 2020. In all domains except for bodily pain, QOL was slightly lower than that of an age-matched reference population. The KPS score was obtained from 42/49 (86%) patients; of these, 31 patients (74%) had a KPS score of ≥80% (able to carry on normal activity). All 42 patients were able to live at home and care for personal needs. The median postoperative CAMPHOR scores were: 4 for symptoms, 4 for activity, and 2.5 for QOL. We observed that QOL after PEA approached the expected QOL in a reference population and that CAMPHOR scores were comparable to those of a large UK cohort after PEA. Functional status improved when assessed late after PEA. Three-quarters of the study population were able to conduct normal activities at late follow-up. Our findings suggest that many patients enjoy satisfactory QOL and high functional status late after PEA.

Physical activity and its clinical correlates in chronic thromboembolic pulmonary hypertension.

Limited data are available on physical activity (PhA) levels in chronic thromboembolic pulmonary hypertension (CTEPH) patients, as well as on the clinical utility of PhA measurements using questionnaires and accelerometers. We aimed to study PhA levels of CTEPH patients and their clinical correlates, and to compare PhA levels measured by the International Physical Activity Questionnaire (IPAQ) with measures from accelerometers. This is a cross‐sectional study (n = 50). PhA levels were measured using accelerometers and questionnaires (IPAQ). Clinical parameters evaluated were walked distance on the 6‐min‐walking test (6MWT), pulmonary vascular resistance, N‐terminal brain natriuretic peptide and quality of life (HRQoL) (Cambridge Pulmonary Hypertension Outcome Review questionnaire). Time spent in sedentary behavior was lower in self‐reported measurement (279 ± 165 min/day) compared with accelerometry (446 ± 117 min/day, p < 0.000). Accelerometer‐derived data showed that CTEPH patients spent 60% of the recorded time in sedentary behaviors and 2% in moderate‐to‐vigorous PhA (MVPA). Correlation analysis showed that MVPA was significantly correlated with 6MWT (p = 0.023) and symptom domain of HRQoL (p = 0.044). Self‐reported MVPA was significantly higher than the one registered by the accelerometer (411 ± 569 vs. 131 ± 108 min/week, p = 0.027). Bland−Altman analysis indicated poor agreement between the two methods. Our results showed that CTEPH patients spend most of their days in sedentary behaviors and only a small amount of time in MVPA. Only MVPA was associated with HRQoL and CTEPH severity. In addition, we showed a poor agreement between self‐reported and accelerometer‐derived PhA in CTEPH patients, with the former overestimating the overall PhA.

Quality of life of patients with pulmonary arterial hypertension: a meta-analysis.

Pulmonary arterial hypertension (PAH) is a rare condition, with an incidence of 15-50 cases per million annually. Available studies demonstrate that despite the longer survival of PAH patients, their quality of life (QoL) deteriorates as the condition progresses. Consequently, the goals of PAH therapy have expanded from increasing survival to improving health-related quality of life. The objective of this systematic review and meta-analysis was to summarize the available evidence about the level of QoL in patients with PAH. A systematic search was performed using the Cochrane guidelines for conducting meta-analysis following the PRISMA statement. The meta-analysis includes findings from 11 studies evaluating the QoL of PAH patients at baseline and at follow-up (12 weeks) using the Short Form (36) Health Survey (SF-36), the Minnesota Living with Heart Failure Questionnaire (MLHFQ) and the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). The mean physical component score (SF-36) for the group was 37.2 points (95% CI: 33.24-41.16) and the heterogeneity coefficient was I²=97.71% (p < 0.001). The mean mental component score (SF-36) was 46.38 (95% CI: 44.21-48.56) and the heterogeneity coefficient was I²=87.92% (p < 0.001). The result indicates improved QoL 12 weeks after the intervention, though three papers did not fully confirm this. The greatest improvement in QoL was found in patients treated with bosentan and iloprost and the smallest improvement in QoL was found in patients treated with epoprostenol sodium. The heterogeneity coefficient was I²=91.36%, p < 0.001 for CAMPHOR and I²=97.65%, p < 0.001 for MLHFQ. PAH patients tend to have a poor QoL, mainly in the physical functioning domain, less so in the psychological functioning domain. QoL may be improved by therapeutic interventions, mainly pharmaceutical ones. Patients with PAH also tend to suffer from depression, anxiety, stress, or sleep disorders. All these factors are significantly correlated with poorer QoL.

Serial right heart catheter assessment between balloon pulmonary angioplasty sessions identify procedural factors that influence response to treatment

Balloon pulmonary angioplasty (BPA) is delivered as a series of treatments for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) however, there is little published data on the procedural determinants of outcome.Pre- and post-BPA clinical and hemodynamic data, as well as serial hemodynamic and procedural data at each BPA session were evaluated to determine patient and procedure-related factors that influence hemodynamic response.Per procedure data from 210 procedures in 84 patients and per patient data from 182 procedures in 63 patients with completed treatment and 3-month follow-up were analyzed. A median of 3 (range 1-6) BPA procedures treating a median of 2 segments per procedure (range 1-3) were performed per patient with a median interval between procedures of 42 (range 5-491) days. Clinical outcome correlated with hemodynamic change (pulmonary vascular resistance [ΔPVR] vs Cambridge Pulmonary Hypertension Outcome Review [CAMPHOR] symptom score: p < 0.001, Pearson's r = 0.48, n = 49). Responders to BPA had more severe disease at baseline and 37.5 % of non-responders were post-PEA. There was a dose-response relationship between per procedure and total number of segments treated and hemodynamic improvement (ΔPVR: 1 segment: -0.9%, 2: -14.5%, 3 or more: -16.1%, p < 0.001). Treating totally occluded vessels had a greater hemodynamic effect (mean pulmonary artery pressure [ΔmPAP]: sessions with occlusion: -8.0%, without occlusion treated: -3.2%, p < 0.05) without an increased complication rate.The magnitude of clinical benefit is related to the hemodynamic effect of BPA which in turn is related to the number of segments treated and lesion severity. Patients who were post-PEA were less likely to respond to BPA.

Physical activity and its clinical correlates in chronic thromboembolic pulmonary hypertension

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Fundação para a Ciência (FCT) Coordenação de Aperfeiçoamento de Pessoal de Nível (CAPES) Background Limited data is available on physical activity (PA) levels in chronic thromboembolic pulmonary hypertension (CTEPH) patients, as well as on the impact of using different tools to assess PA such as questionnaires and accelerometers. Purpose We aimed to quantify PA levels of CTEPH patients and study its clinical correlates, as well as to compare PA levels measured by the International Physical Activity Questionnaire (IPAQ) with measures from accelerometers. Methods This is a cross-sectional study (n = 50). Physical activity levels were measured using accelerometers and questionnaire (IPAQ). Clinical parameters evaluated were walked distance on the 6-minute-walking test (6MWT), pulmonary vascular resistance, N-terminal brain natriuretic peptide and quality of life (HRQoL) using the Cambridge Pulmonary Hypertension Outcome Review questionnaire. Results Accelerometer-derived data showed that CTEPH patients spent 60% of the recorded time in sedentary behaviours and only 2% in moderate-to-vigorous PA (MVPA). MVPA was mildly correlated with 6MWT (r = 0.359; p= 0.023) and symptom domain of HRQoL (r=-0.371; p = 0.044) but not with NT-proBNP, pulmonary vascular resistance or functional domain of HRQoL. Time spent in sedentary behaviour was lower in self-reported measurement (279 ± 165min/day) compared to accelerometry (446 ± 117min/day, p &amp;lt; 0.001). Self-reported MVPA was significantly higher than the one registered by the accelerometer (411 ± 569 vs. 131 ± 108 min/week, p = 0.027). Bland-Altman analysis indicated poor agreement between the two methods. Conclusions Our results showed that CTEPH patients spend most of their days in sedentary behaviors and only a small amount of time in MVPA. MVPA was associated with symptoms domain of HRQoL and submaximal functional capacity. In addition, we showed a poor agreement between self-reported and accelerometer-derived PA in CTEPH patients, with the former overestimating the overall PA activity.

A minimal clinically important difference measured by the Cambridge Pulmonary Hypertension Outcome Review for patients with idiopathic pulmonary arterial hypertension.

Several patient-reported outcome measures have been developed to assess health status in pulmonary arterial hypertension. The required change in instrument scores needed, to be seen as meaningful to the individual, however remain unknown. We sought to identify minimal clinically important differences in the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and to validate these against objective markers of functional capacity. Minimal clinically important differences were established from a discovery cohort (n = 129) of consecutive incident cases of idiopathic pulmonary arterial hypertension with CAMPHOR scores recorded at treatment-naïve baseline and 4–12 months following pulmonary arterial hypertension therapy. An independent validation cohort (n = 87) was used to verify minimal clinically important differences. Concurrent measures of functional capacity relative to CAMPHOR scores were collected. Minimal clinically important differences were derived using anchor- and distributional-based approaches. In the discovery cohort, mean (SD) was 54.4 (16.4) years and 64% were female. Most patients (63%) were treated with sequential pulmonary arterial hypertension therapy. Baseline CAMPHOR scores were: Symptoms, 12 (7); Activity, 12 (7) and quality of life, 10 (7). Pulmonary arterial hypertension treatment resulted in significant improvements in CAMPHOR scores (p < 0.05). CAMPHOR minimal clinically important differences averaged across methods for health status improvement were: Symptoms, –4 points; Activity, –4 points and quality of life –3 points. CAMPHOR Activity score change ≥minimal clinically important difference was associated with significantly greater improvement in six-minute walk distance, in both discovery and validation populations. In conclusion, CAMPHOR scores are responsive to pulmonary arterial hypertension treatment. Minimal clinically important differences in pulmonary hypertension-specific scales may provide useful insights into treatment response in future clinical trials.

CAMPHOR score: patient-reported outcomes are improved by pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension.

Pulmonary endarterectomy (PEA) is the recommended treatment for eligible patients with chronic thromboembolic pulmonary hypertension (CTEPH). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) score is an internationally validated patient-reported outcome (PRO) measure for CTEPH. It assesses three domains: activity, quality of life (QoL) and symptoms. We assessed PROs in patients with CTEPH undergoing PEA. This retrospective observational study of consecutive CTEPH patients undergoing PEA at the UK national PEA centre between 2006 and 2017 assessed change in CAMPHOR score from baseline (pre-PEA) until up to 5 years post-PEA. CAMPHOR scores were compared between 1) those with and without clinically significant residual pulmonary hypertension and 2) those undergoing PEA and propensity-matched CTEPH patients who were not operated on. The minimally clinically important difference (MCID) was calculated using an anchor-based method. Out of 1324 CTEPH patients who underwent PEA, 1053 (80%) had a CAMPHOR score recorded pre-PEA, 934 (71%) had a score recorded within a year of PEA and 784 (60%) had both. There were significant improvements between pre- and post-PEA in all three CAMPHOR domains (median±interquartile range activity -5±7, QoL -4±8, symptoms -7±8; all p<0.0001). Improvements in CAMPHOR score were greater and more sustained in those without clinically significant residual pulmonary hypertension. CTEPH patients undergoing PEA had better CAMPHOR scores than those not operated on. The MCID in CAMPHOR score was -3±5 for activity, -4±7 for QoL and -6±7 for symptoms. PROs are markedly improved by PEA in patients with CTEPH, more so in those without clinically significant residual pulmonary hypertension.

Adaptation and validation of the quality of life assessment of the Cambridge pulmonary hypertension outcome review (CAMPHOR) for Brazil

BackgroundPulmonary Hypertension (PH) impacts negatively on patients’ health-related quality of life (HRQoL). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) was the first PH-specific and validated instrument for use in different languages worldwide. This report describes the adaptation and psychometric validation of the CAMPHOR into Brazilian Portuguese language.MethodsThe translation and validation process included a bilingual and lay panel translation; cognitive debriefing interviews; psychometric testing in two repeated times assessing internal consistency, reproducibility and validity of the questionnaire. The Nottingham Health Profile (NHP) questionnaire was used as a comparator to test for convergent validity.ResultsThe translation captured the same concepts as the English questionnaire and produced a comprehensive instrument in a Brazilian-Portuguese version expressing common, natural language. The psychometric evaluation involved 102 patients (48.8 ± 14.5 years, 80,4% female]. Cronbach’s alpha coefficients were above 0.9 on all three CAMPHOR scales. There was excellent test-retest reliability (coefficients above 0.85 on all scales). CAMPHOR Symptoms scale and Activities scale correlated highly with Physical Mobility section and CAMPHOR QoL scale was strongly associated with the Emotional Reactions and Social Isolation sections of NHP. There was a significant association between gender and perceived general health (p < 0.05). There were significant differences in CAMPHOR scale scores between patients who differed according to their perceived disease severity and general health.ConclusionsThe present CAMPHOR version demonstrated good psychometric properties and provides a reliable instrument for assessing HRQL and QoL in Brazilian PH patients, addressing patients’ perspective of their illness in a comprehensive way.

Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: the UK experience

ObjectiveInoperable chronic thromboembolic pulmonary hypertension (CTEPH) managed medically has a poor prognosis. Balloon pulmonary angioplasty (BPA) offers a new treatment for inoperable patients. The national BPA service for the UK...

The Effects of a 10-wk Outpatient Pulmonary Rehabilitation Program on Exercise Performance, Muscle Strength, Soluble Biomarkers, and Quality of Life in Patients With Pulmonary Hypertension.

Pulmonary arterial hypertension (PAH) is characterized by right ventricular failure, leading to exertional dyspnea, skeletal muscle weakness, and poor quality of life (QOL). Apart from treatment with PAH-specific drugs, guidelines recommend pulmonary rehabilitation (PR). Clinical PR programs have shown improvement in functional capacity and QOL. However, little is known about the effectiveness of an outpatient PR program. The aim of our study was to assess effectiveness of a multidisciplinary outpatient PR program. Patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH), who were in a stable condition on optimized drug therapy, followed a 10-wk outpatient program in a rehabilitation center. The PR program was designed to improve exercise capacity and health status by means of low load cycling, walking, and muscle training twice a week combined with psychological counseling. QOL was measured by the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) questionnaire. Twenty-one patients (13 women) with PAH (n = 16) or CTEPH (n = 5) completed the study. All patients were in New York Heart Association (NYHA) functional class III, and their mean age was 45 ± 16 yr. After PR, the mean cycling endurance time increased by 4.4 min (P < .001), 6-min walk distance by 12.2 m (P < .05), and maximum inspiratory pressure by 5.8 cm H2O (P = .01). Skeletal muscle function increased significantly. The CAMPHOR questionnaire demonstrated significant decrease in symptoms and improvement in QOL. Soluble biomarkers did not show any change before and after PR. Outpatient PR could be an effective instrument to improve exercise capacity and health status in patients with PAH or CTEPH.

4283Exercise right heart catheterization before and after balloon pulmonary angioplasty in inoperable patients with chronic thromboembolic pulmonary hypertension

Abstract Background Balloon pulmonary angioplasty (BPA) is an evolving treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). The main indicator for success is improvement in pulmonary hemodynamics, but outcome data are heterogeneous. Purpose The aim of the present study was to evaluate pulmonary hemodynamics not only at rest, but also during exercise before and 6 months after BPA. Methods We report a prospective series of 64 consecutive patients with inoperable CTEPH who were treated interventionally with BPA. All patients underwent standardized assessment prior to the first BPA and 6 months after the last intervention. Assessment included WHO FC, Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), 6-minute walking distance (6MWD), serum levels of the N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP), and exercise RHC. Results The mean number of sessions per patient was 5.6 (± 1.3) and the mean number of pulmonary segments targeted in all interventions was 11 (± 3). BPA treatment led to improvements in pulmonary hemodynamics and exercise capacity (6MWD: 416±94 m vs. 463±96 m; p&lt;0.0001) except for CO and CI during RHC at rest; these parameters showed improvements only during exercise RHC. MPAP at rest showed a reduction from 41±9 to 31±9 mmHg (p&lt;0.0001) and PVR at rest decreased from 6.8±2.3 WU to 4.3±1.9 WU (p&lt;0.0001). Further decreases were observed for systolic pulmonary arterial pressure, TPG, PVR, and TPR. Cardiac output (7.0±2.0 L/min vs. 8.3±2.0 L/min; p&lt;0.0001) and cardiac index during exercise RHC (3.8±1.1 L/min/m2 vs. 4.4±1.1 L/min/m2; p&lt;0.0001) improved significantly. Median NT-proBNP concentrations decreased from 741 ng/L (IQR 192–1425 ng/L) to 139 ng/L (IQR 60–266 ng/L) during BPA treatment (p&lt;0.0001). Results from the CAMPHOR questionnaire showed significant improvements in symptoms (11±5.8 vs. 5.5±4.9, p&lt;0.0001), activity limitations (9.2±5.6 vs. 5.2±4.5, p&lt;0.0001), and quality of life (6.4±5.7 vs. 3.5±3.7, p&lt;0.0001). Conclusion Significant improvements in pulmonary hemodynamics at rest and during exercise were observed 6 months after BPA. Exercise right heart catheterization offers a more discriminating evaluation of the changes in pulmonary hemodynamics after BPA.

ADAPTATION AND VALIDATION OF THE CAMBRIDGE PULMONARY HYPERTENSION OUTCOME REVIEW (CAMPHOR) FOR CROATIA.

SUMMARYPulmonary hypertension (PH) is a chronic disease which severely impairs quality of life (QoL). The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) is the first disease-specific tool to assess patient-reported symptoms, functioning and QoL in PH patients. The aim of this study was to adapt and validate the CAMPHOR for use in Croatia. The adaptation process involved three stages: translation (bilingual and lay panel), cognitive debriefing interviews with patients and psychometric validation. For the latter stage, a postal survey was conducted with 50 patients to examine the reliability and validity of the adapted scale. All three scales of the Croatian CAMPHOR demonstrated excellent internal consistency (Symptoms = 0.93; Activity limitations = 0.94; QoL = 0.92) and test-retest reliability correlations (Symptoms = 0.90; Activity limitations = 0.95; QoL = 0.90). Predicted correlations with the SF-36 scales provided evidence for construct validity of the CAMPHOR scales. Evidence for known group validity was shown by the ability of the scales to distinguish between participants based on patient-perceived general health and disease severity. The Croatian version of the CAMPHOR is a valid and reliable tool for use in clinical routine and clinical research.

Cross-cultural adaptation of the Cambridge Pulmonary Hypertension Outcome Review for use in patients with pulmonary hypertension in Colombia.

ABSTRACT Objective:To conduct a cross-cultural adaptation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) as an instrument to evaluate the perception of symptoms, functional limitation, and health-related quality of life (HRQoL) in subjects diagnosed with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) in Colombia. Methods:The adaptation process involved 3 phases: translation, cognitive debriefing interviews, and a validation survey. To evaluate the psychometric properties, we recruited individuals ≥ 18 years of age who had been diagnosed with PAH or CTEPH to take part in the latter two stages of the adaptation process. All individuals were being followed on an outpatient basis by the pulmonary hypertension programs at Hospital Universitario San Ignacio, Fundación Clínica Shaio,and Clínicos IPS, all located in the city of Bogotá, Colombia. Results:A Spanish-language version of the CAMPHOR was developed for use in Colombia. The internal consistency was excellent for the symptoms, functioning, and quality of life scales (Cronbach’s alpha coefficients of 0.92, 0.87, and 0.93, respectively). Test-retest reliability was above 0.70. The evaluation of the convergent validity and known group validity of the CAMPHOR scales confirmed that there were moderate and strong correlations with the related constructs of the Medical Outcomes Study 36-item Short-Form Health Survey, version 2, as well as showing their capacity to discriminate disease severity. Conclusions:The Spanish-language version of the CAMPHOR developed for use in Colombia was the result of a translation and cultural adaptation process that allows us to consider it equivalent to the original version, having shown good psychometric properties in the study sample. Therefore, its use to assess the impact of interventions on the HRQoL of patients with PAH or CTEPH is recommended, in research and clinical practice.