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Background
Individuals 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 trans...
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BACKGROUND: 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 cou...
Citations
... For the evaluation of HRQoL in PH, patients receive a validated PH specific survey, the so-called Cambridge Pulmonary Hypertension Outcome Review (CAM-PHOR) for a thorough assessment of disease specific HRQoL [12]. Previous studies have shown significant positive correlations between the distance walked in the 6MWT and HRQoL [13][14][15]. ...
... The CAMPHOR questionnaire was developed by Galen Research, Enterprise House in 2006 [12]. It has been validated in patients with PH and shows good reliability and validity [12,14]. The survey contains 65 questions in total, 25 related to symptoms (concerning the frequency and severity of symptoms), 15 related to activities of daily living (the degree to which activities are limited by breathlessness and patients have to rely on outside help to perform the activities of daily living) and 25 related to general quality of life (QoL) (aspects of social and psychological function affected by the disease). ...
Introduction
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.
Aims
Our aim was to evaluate the 1-min STST performance and its association with patient-reported HRQoL in patients with PH.
Methods
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).
Results
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.
Conclusion
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.
... The incidence and prevalence of patients diagnosed with group 4 PH or chronic thromboembolic PH (CTEPH) is 2-6 and 26-38 cases/million adults, with the increasing number over recent years potentially being related to improved diagnostics [1]. Typical symptoms include exertional dyspnea, along with impaired exercise performance and reduced quality of 2 of 12 life [4][5][6]. Due to pathophysiological changes such as fibrotic scars in more proximal pulmonary arteries and vascular remodeling or intimal thickening (known as distal microvasculopathy), pulmonary vascular resistance (PVR) increases, resulting in an elevated pulmonary artery pressure (PAP) [7,8]. ...
Background:
Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (CTEPH) with accessible lesions. Breathing pure oxygen (hyperoxia) during right heart catheterization (RHC) allows for the calculation of the right-to-left shunt fraction (Qs/Qt). In the absence of intracardiac shunt, Qs/Qt can be used as a marker of ventilation-perfusion mismatch in patients with CTEPH. This study involved investigating Qs/Qt after PEA and its relation to other disease-specific outcomes.
Study design and methods:
This study is a retrospective study that focuses on patients with operable CTEPH who had Qs/Qt assessment during RHC before and 1 year after PEA. Additionally, 6 min walking distance (6MWD), WHO functional class (WHO-FC), and NT-proBNP were assessed to calculate a four-strata risk score.
Results:
Overall, 16 patients (6 females) with a median age of 66 years (quartiles 55; 74) were included. After PEA, an improvement in mean pulmonary artery pressure (38 [32; 41] to 24 [18; 28] mmHg), pulmonary vascular resistance (5.7 [4.0; 6.8] to 2.5 [1.4; 3.8] WU), oxygen saturation (92 [88; 93]% to 94 [93; 95]%), WHO-FC, and risk score was observed (all p < 0.05). No improvement in median Qs/Qt could be detected (13.7 [10.0; 17.5]% to 13.0 [11.2; 15.6]%, p = 0.679). A total of 7 patients with improved Qs/Qt had a significant reduction in risk score compared to those without improved Qs/Qt.
Conclusion:
PEA did not alter Qs/Qt assessed after 1 year in operable CTEPH despite an improvement in hemodynamics and risk score, potentially indicating a persistent microvasculopathy. In patients whose shunt fraction improved with PEA, the reduced shunt was associated with an improvement in risk score.
... The cardinal symptoms of PVD are dyspnea on exertion with impaired exercise performance, daily activity, and quality of life. [2][3][4][5] Disease progression causes gas exchange disturbances associated with hypoxemia, especially during exercise and sleep. 6,7 The pharmaceutical treatment of PVD according to guidelines is mainly based on medication targeting 3 biochemical pathways, which counteract pulmonary vasoconstriction and/or promote pulmonary vasodilatation. ...
Main pulmonary vascular diseases (PVD) with precapillary pulmonary hypertension (PH) are pulmonary arterial and chronic thromboembolic PH. Guidelines recommend supplemental oxygen therapy (SOT) for severely hypoxemic patients with PH, but evidence is scarce. The authors performed a systematic review and where possible meta-analyses on the effects of SOT on hemodynamics and exercise performance in patients with PVD. In PVD, short-term SOT significantly improved mean pulmonary artery pressure and exercise performance. There is growing evidence on the benefit of long-term SOT for selected patients with PVD regarding exercise capacity and maybe even survival.
... The leading symptom of PVD is dyspnea on exertion, leading to impaired exercise performance, reduced daily activity, and diminished quality of life (Cenedese et al., 2006;Cima et al., 2012;Ulrich et al., 2013;Saxer et al., 2019). With disease progression, worsening hemodynamics may lead to gas exchange impairment and hypoxemia, particularly during exercise and sleep (Ulrich et al., 2008. ...
Ulrich, Silvia, Mona Lichtblau, Simon R. Schneider, Stéphanie Saxer, and Konrad E. Bloch, Clinician's corner: counseling patients with pulmonary vascular disease traveling to high altitude. High Alt Med Biol 00:000-000, 2022.-Pulmonary vascular diseases (PVDs) with precapillary pulmonary hypertension (PH), such as pulmonary arterial or chronic thromboembolic PH, impair exercise performance and survival in patients. Vasodilators and other treatments improve quality of life and prognosis to an extent in patients who have PVDs as chronic disorders. Obviously, patients with PVD wish to participate in usual daily activities, including travel to popular settlements and mountainous regions located at high altitude. However, the pulmonary hemodynamic impairment due to PVD leads to blood and tissue hypoxia, particularly during exercise and sleep. It is thus of concern that alveolar hypoxia at higher altitude may exacerbate patients' symptoms and lead to decompensation. Current PH guidelines discourage high-altitude exposure for fear of altitude-related adverse health effects. However, several recent well-designed prospective and randomized trials show that despite altitude-induced hypoxemia, pulmonary hemodynamic changes and impairment of exercise performance in patients with PVD are similar to the responses in healthy people or in patients with mild chronic obstructive pulmonary disease. The vast majority of patients with PVD can tolerate short-term exposure to moderate altitudes up to 2,500 m. For the roughly 10% of patients with stable disease who develop severe hypoxemia when ascending to 2,500 m, they respond well to low-level supplemental oxygen support. The best low-altitude predictors for adverse health effects at high altitude are the known clinical risk factors for PVD such as symptoms, functional class, exercise capacity, and exertional oxygen desaturation, whereas hypoxia altitude simulation testing is of little additive value. In any case, patients should be instructed that altitude-related adverse health effects may be difficult to predict and that in case of worsening symptoms, immediate accompanied descent to lower altitude and oxygen therapy are required. Patients with severe hypoxemia near sea level may safely visit high-altitude regions up to 1,500-2,000 m while continuing oxygen therapy and avoiding strenuous exercise. All PH patients should be counseled before any high-altitude sojourn by doctors with experience in PVD and high-altitude medicine and have an action plan for the occurrence of severe hypoxemia and other altitude-related conditions such as acute mountain sickness.
... In the absence of relevant lung diseases, the two major forms of precapillary pulmonary hypertension (PH) are pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH/CTEPH). PH is a relatively rare condition with potentially drastic limitations in prognosis and quality of life (1)(2)(3). PH is diagnosed and hemodynamically classified by right heart catheterization (RHC) as mean pulmonary artery pressure (mPAP) >20 mmHg with a pulmonary artery wedge pressure (PAWP) ≤15 mmHg (3)(4)(5). ...
Background: Exact and simultaneous measurements of mean pulmonary artery pressure (mPAP) and cardiac output (CO) are crucial to calculate pulmonary vascular resistance (PVR), which is essential to define pulmonary hypertension (PH). Simultaneous measurements of mPAP and CO are not feasible using the direct Fick (DF) method, due to the necessity to sample blood from the catheter-tip. We evaluated a modified DF method, which allows simultaneous measurement of mPAP and CO without needing repetitive blood samples.
Methods: Twenty-four patients with pulmonary arterial or chronic thromboembolic PH had repetitive measurements of CO at rest and end-exercise during three phases of a crossover trial. CO was assessed by the original DF method using oxygen uptake, measured by a metabolic unit, and arterial and mixed venous oxygen saturations from co-oximetry of respective blood gases served as reference. These CO measurements were then compared with a modified DF method using pulse oximetry at the catheter- and fingertip.
Results: The bias among CO measurements by the two DF methods at rest was −0.26 L/min with limits of agreement of ±1.66 L/min. The percentage error was 28.6%. At the end-exercise, the bias between methods was 0.29 L/min with limits of agreement of ±1.54 L/min and percentage error of 16.1%.
Conclusion: Direct Fick using a catheter- and fingertip pulse oximetry (DFp) is a practicable and reliable method for assessing CO in patients with PH. This method has the advantage of allowing simultaneous measurement of PAP and CO, and frequent repetitive measurements are needed during exercise.
Clinical Trial Registration: https://clinicaltrials.gov/ct2/show/NCT02755259 , identifier: NCT02755259.
... Major forms are pulmonary arterial and chronic thromboembolic PH, hereafter summarized as PH (1). The leading symptom in PH is dyspnoea on exertion leading to exercise limitation, reduced daily activity and impaired quality of life (2)(3)(4). Exertional and sleep-related hypoxaemia (5) are further consequences of PH that may impair daytime performance (5)(6)(7). ...
Aims: To test the acute hemodynamic effect of acetazolamide in patients with pulmonary hypertension (PH) under ambient air and hypoxia.
Methods: Patients with pulmonary arterial or chronic thromboembolic PH (PAH/CTEPH) undergoing right heart catheterization were included in this randomized, placebo-controlled, double-blinded, crossover trial. The main outcome, pulmonary vascular resistance (PVR), further hemodynamics, blood- and cerebral oxygenation were measured 1 h after intravenous administration of 500 mg acetazolamide or placebo-saline on ambient air (normoxia) and at the end of breathing hypoxic gas (F I O 2 0.15, hypoxia) for 15 min.
Results: 24 PH-patients, 71% men, mean ± SD age 59 ± 14 years, BMI 28 ± 5 kg/m ² , PVR 4.7 ± 2.1 WU participated. Mean PVR after acetazolamide vs. placebo was 5.5 ± 3.0 vs. 5.3 ± 3.0 WU; mean difference (95% CI) 0.2 (−0.2–0.6, p = 0.341). Heart rate was higher after acetazolamide (79 ± 12 vs. 77 ± 11 bpm, p = 0.026), pH was lower (7.40 ± 0.02 vs. 7.42 ± 0.03, p = 0.002) but PaCO 2 and PaO 2 remained unchanged while cerebral tissue oxygenation increased (71 ± 6 vs. 69 ± 6%, p = 0.017). In acute hypoxia, acetazolamide decreased PVR by 0.4 WU (0.0–0.9, p = 0.046) while PaO 2 and PaCO 2 were not changed. No adverse effects occurred.
Conclusions: In patients with PAH/CTEPH, i.v. acetazolamide did not change pulmonary hemodynamics compared to placebo after 1 hour in normoxia but it reduced PVR after subsequent acute exposure to hypoxia. Our findings in normoxia do not suggest a direct acute pulmonary vasodilator effect of acetazolamide. The reduction of PVR during hypoxia requires further corroboration. Whether acetazolamide improves PH when given over a prolonged period by stimulating ventilation, increasing oxygenation, and/or altering vascular inflammation and remodeling remains to be investigated.
... Whilst this study has a number of strengths, we acknowledge that although patient characteristics were similar to those of well-published PAH cohorts, data from this study originate from a single pulmonary hypertension centre and may therefore be subject to bias. CAMPHOR has also received criticism for being more time intensive than other available pulmonary hypertension QOL measures, however remains the most validated with adaption for use in, but not limited to, the: United States, 39 Australia/New Zealand, 40 Portugal, 41 Germany, 42 Netherlands, 43 Poland, 44 Brazil, 45 Croatia, 46 French/English Canada 47 and Columbia. 48 This provides plentiful opportunity for the external validation of MCID estimates to assess their robustness which is unafforded by other measures at present. ...
Several patient-reported outcome measures have been developed to assess health status in pulmonary arterial hypertension (PAH). 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 (MCIDs) in the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) and to validate these against objective markers of functional capacity. MCIDs were established from a discovery cohort (n = 129) of consecutive incident cases of idiopathic PAH with CAMPHOR scores recorded at treatment-naïve baseline and 6 â 9 months following PAH therapy. An independent validation cohort (n = 87) was used to verify MCIDs. Concurrent measures of functional capacity relative to CAMPHOR scores were collected. MCIDs were derived using anchor- and distributional-based approaches. In the discovery cohort, mean (SD) age was 54.4 (16.4) years and 64% were female. Most patients (63%) were treated with sequential PAH therapy. Baseline CAMPHOR scores were: Symptoms, 12 (7); Activity, 12 (7) and QoL, 10 (7). PAH treatment resulted in significant improvements in CAMPHOR scores (p < 0.05). CAMPHOR MCIDs averaged across methods for health status improvement were: Symptoms, -4 points; Activity, -4 points and QoL -3 points. CAMPHOR Activity score change >= MCID with PAH treatment was associated with significantly greater improvement in 6-minute walk distance, in both discovery and validation cohorts. In conclusion, CAMPHOR scores are responsive to improvements in functional capacity with PAH treatment. MCIDs in PAH scales may provide useful insights into treatment response in future clinical trials.
... The measure was developed and validated in a unique PH reference center in United Kingdom and has been adapted for use in many countries, providing a valuable tool to reflect the actual patient experience in each country [13][14][15][16][17][18][19][20]. However, a validated version of the CAMPHOR for use in Brazil has been previously unavailable. ...
Background:
Pulmonary 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.
Methods:
The 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.
Results:
The 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.
Conclusions:
The 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.
... Atomic Merz-Kollman (MK), Mulliken and natural population atomic (NPA) charges were studied in both R(+) and S(-) forms of camphor in gas phase and aqueous solution by using the B3LYP/6-311++G** method because the dihedral angles and the solvation energies have evidenced different behaviours between both enantiomers in solution. Besides, it is very important to undestand the conexion existent between the only acceptor H bonds group (O) in both R(+) and S(-) forms and the different properties attributed from long time to camphor [3,7,8,[11][12][13][14][15][17][18][19][20][21][23][24][25][26][27][28][29]. Hence, atomic Merz-Kollman (MK), Mulliken and natural population atomic (NPA) charges were studied together with the molecular electrostatic potentials (MEP) and bond orders (BO) for both forms of camphor in both media by using the the B3LYP/6-311++G** method. ...
... The knowledge of the energies gap and of some typical descriptors in the two R(+) and S(-) forms of camphor are essential to predict their reactivities and behaviours in different media taking into account the diverse medicinal and biological properties attributed to camphor [3,7,8,[11][12][13][14][15][17][18][19][20][21][23][24][25][26][27][28][29]. Hence, the frontier orbitals and the chemical potential (μ), electronegativity (χ), global hardness (η), global softness (S), global electrophilicity index (ω) and nucleophilicity indexes () descriptors were calculated for the two R(+) and S(-) forms of camphor in gas phase and aqueous solution by using the hybrid B3LYP/6-311++G** method [42][43][44][45]. ...
B3LYP/6-311++G** calculations were performed to study structures and vibrational properties of Cis S(-) and R(+) forms of camphor. Comparisons between calculated geometrical parameters of both forms of Camphor in gas phase and aqueous solution show very good concordances with the experimental ones corresponding to (+)-3-bromocamphor. NBO calculations predict only *, * and n* interactions althougth the expected n* transitions due to ketone groups C=O were no predicted. Gap and electrophilicity index (ω) values of both forms of camphor are close to the value observed in antiviral thymidine. Such observations could be explained by the proximities between the acceptor groups H bonds (C=O) and the CH3 groups present in both camphor and thymidine species. Reasonable concordances were found among the predicted 1H- and 13C-NMR, UV-visible, ECD, IR and Raman spectra with the corresponding experimental ones. Complete vibrational assignments and scaled force constants for both forms camphor are reported for first time
... La escala CAMPHOR ha demostrado ser una herramienta que genera resultados válidos, confiables y sensibles para la evaluación de la CVRS basal y en respuesta a intervenciones en sujetos con HAP e HPTEC, (15,17,18) por lo cual cuenta con múltiples procesos de adaptación lingüística y cultural, alcanzando en todas sus versiones adecuadas propiedades psicométricas similares a las de la original. (19)(20)(21)(22)(23)(24)(25)(26) La escala CAMPHOR ha sido adaptada y validada para su uso en Estados Unidos, (19) Canadá, (20) Australia/ Nueva Zelanda, (21) Alemania/Suiza/Austria, (22) Suecia, (23) Portugal, (24) Países Bajos (25) y recientemente en España, (26) alcanzando en todas sus versiones adecuadas características psicométricas. ...
... La escala CAMPHOR ha demostrado ser una herramienta que genera resultados válidos, confiables y sensibles para la evaluación de la CVRS basal y en respuesta a intervenciones en sujetos con HAP e HPTEC, (15,17,18) por lo cual cuenta con múltiples procesos de adaptación lingüística y cultural, alcanzando en todas sus versiones adecuadas propiedades psicométricas similares a las de la original. (19)(20)(21)(22)(23)(24)(25)(26) La escala CAMPHOR ha sido adaptada y validada para su uso en Estados Unidos, (19) Canadá, (20) Australia/ Nueva Zelanda, (21) Alemania/Suiza/Austria, (22) Suecia, (23) Portugal, (24) Países Bajos (25) y recientemente en España, (26) alcanzando en todas sus versiones adecuadas características psicométricas. ...
... (30) Al ser el CAMPHOR la primera escala desarrollada específicamente para evaluar la CVRS en HP, ha sido la más utilizada en estudios clínicos y cuenta con varias publicaciones que describen en detalle el proceso de adaptación cultural a diferentes países. (19)(20)(21)(22)(23)(24)(25)(26) Es importante señalar que en las adaptaciones realizadas se alcanzaron características psicométricas adecuadas y similares a la versión original. ...
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.
