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Published in: BMC Pulmonary Medicine 1/2021

Open Access 01-12-2021 | Pulmonary Hypertension | Research article

Clinical evaluation of pulmonary hypertension using patient-reported outcomes: a cross-sectional study

Authors: Miguel Ángel Amor-García, Sara Ibáñez-García, Xandra García-González, Teresa Mombiela, Cristina Villanueva-Bueno, Ana Herranz-Alonso, María Sanjurjo-Sáez

Published in: BMC Pulmonary Medicine | Issue 1/2021

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Abstract

Background

Patients with pulmonary hypertension (PH) have progressive and disabling symptoms, as well as a burden of treatments and a difficult clinical evaluation that make health-related quality of life a particularly relevant endpoint in this disease. The objective of the study was to evaluate patient-reported outcomes of patients receiving specific treatment for PH in a tertiary hospital using a specific questionnaire (Cambridge Pulmonary Hypertension Outcome Review-CAMPHOR) in the pharmacy consultation.

Methods

A cross-sectional, observational, descriptive study was conducted. It included all patients receiving specific treatment for PH in a tertiary hospital in Madrid, Spain. The inclusion period comprised between August to December 2019. CAMPHOR questionnaires containing three domains: symptoms, activities and quality of life were completed by the patients at the pharmacy consultation. Demographic and clinical variables, including WHO Functional Class (WHO FC), PH-specific tests and hemodynamic parameters, were recorded. Non-parametric analyses to assess relations between variables and CAMPHOR domains were performed.

Results

Thirty-six patients consented to participate in the study and completed the questionnaire. Median scores for symptoms, activities, and quality of life domains were 5.5 (2.5–10), 8.0 (4.5–10.5) and 3.5 (1–7.5), respectively. Statistically significant differences were found in the three domains when comparing by WHO FC, in the activities domain for 6-m walking test and in the quality of life domain for patients who had emergency visits or hospitalizations in the last year.

Conclusions

The CAMPHOR questionnaire could be useful as a complementary test to achieve an integrated evaluation of PH patients, who could complete it easily during their routine pharmacy visits.
Literature
1.
go back to reference Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913.CrossRef Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913.CrossRef
2.
go back to reference Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.CrossRef Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.CrossRef
3.
go back to reference Kylhammar D, Kjellström B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018;39(47):4175–81.CrossRef Kylhammar D, Kjellström B, Hjalmarsson C, et al. A comprehensive risk stratification at early follow-up determines prognosis in pulmonary arterial hypertension. Eur Heart J. 2018;39(47):4175–81.CrossRef
4.
go back to reference Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3):1702606.CrossRef Hoeper MM, Pittrow D, Opitz C, et al. Risk assessment in pulmonary arterial hypertension. Eur Respir J. 2018;51(3):1702606.CrossRef
5.
go back to reference Reis A, Santos M, Vicente M, et al. Health-related quality of life in pulmonary hypertension and its clinical correlates: a cross-sectional study. Biomed Res Int. 2018;2018:3924517.CrossRef Reis A, Santos M, Vicente M, et al. Health-related quality of life in pulmonary hypertension and its clinical correlates: a cross-sectional study. Biomed Res Int. 2018;2018:3924517.CrossRef
6.
go back to reference Dunlap B, Weyer G. Pulmonary hypertension: diagnosis and treatment. Am Fam Phys. 2016;94(6):463–9. Dunlap B, Weyer G. Pulmonary hypertension: diagnosis and treatment. Am Fam Phys. 2016;94(6):463–9.
7.
go back to reference Chin KM, Gomberg-Maitland M, Channick RN, et al. Psychometric validation of the pulmonary arterial hypertension-symptoms and impact (PAH-SYMPACT) Questionnaire: results of the SYMPHONY trial. Chest. 2018;154(4):848–61.CrossRef Chin KM, Gomberg-Maitland M, Channick RN, et al. Psychometric validation of the pulmonary arterial hypertension-symptoms and impact (PAH-SYMPACT) Questionnaire: results of the SYMPHONY trial. Chest. 2018;154(4):848–61.CrossRef
8.
go back to reference Gu S, Hu H, Dong H. Systematic review of health-related quality of life in patients with pulmonary arterial hypertension. Pharmacoeconomics. 2016;34(8):751–70.CrossRef Gu S, Hu H, Dong H. Systematic review of health-related quality of life in patients with pulmonary arterial hypertension. Pharmacoeconomics. 2016;34(8):751–70.CrossRef
9.
go back to reference Kondo T, Okumura N, Adachi S, Murohara T. <Editors’ Choice> pulmonary hypertension: diagnosis, management, and treatment. Nagoya J Med Sci. 2019;81(1):19–30.PubMedPubMedCentral Kondo T, Okumura N, Adachi S, Murohara T. <Editors’ Choice> pulmonary hypertension: diagnosis, management, and treatment. Nagoya J Med Sci. 2019;81(1):19–30.PubMedPubMedCentral
10.
go back to reference Thenappan T, Ormiston ML, Ryan JJ, Archer SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ. 2018;360:j5492.CrossRef Thenappan T, Ormiston ML, Ryan JJ, Archer SL. Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ. 2018;360:j5492.CrossRef
11.
go back to reference Delcroix M, Howard L. Pulmonary arterial hypertension: the burden of disease and impact on quality of life. Eur Respir Rev. 2015;24(138):621–9.CrossRef Delcroix M, Howard L. Pulmonary arterial hypertension: the burden of disease and impact on quality of life. Eur Respir Rev. 2015;24(138):621–9.CrossRef
12.
go back to reference Chen H, Taichman DB, Doyle RL. Health-related quality of life and patient-reported outcomes in pulmonary arterial hypertension. Proc Am Thorac Soc. 2008;5(5):623–30.CrossRef Chen H, Taichman DB, Doyle RL. Health-related quality of life and patient-reported outcomes in pulmonary arterial hypertension. Proc Am Thorac Soc. 2008;5(5):623–30.CrossRef
13.
go back to reference McCollister D, Shaffer S, Badesch DB, et al. Development of the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT®) questionnaire: a new patient-reported outcome instrument for PAH. Respir Res. 2016;17(1):72.CrossRef McCollister D, Shaffer S, Badesch DB, et al. Development of the Pulmonary Arterial Hypertension-Symptoms and Impact (PAH-SYMPACT®) questionnaire: a new patient-reported outcome instrument for PAH. Respir Res. 2016;17(1):72.CrossRef
14.
go back to reference McKenna SP, Doughty N, Meads DM, Doward LC, Pepke-Zaba J. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR): a measure of health-related quality of life and quality of life for patients with pulmonary hypertension. Qual Life Res. 2006;15(1):103–15.CrossRef McKenna SP, Doughty N, Meads DM, Doward LC, Pepke-Zaba J. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR): a measure of health-related quality of life and quality of life for patients with pulmonary hypertension. Qual Life Res. 2006;15(1):103–15.CrossRef
15.
go back to reference Meads DM, McKenna SP, Doughty N, et al. The responsiveness and validity of the CAMPHOR Utility Index. Eur Respir J. 2008;32(6):1513–9.CrossRef Meads DM, McKenna SP, Doughty N, et al. The responsiveness and validity of the CAMPHOR Utility Index. Eur Respir J. 2008;32(6):1513–9.CrossRef
16.
go back to reference Twiss J, McKenna S, Ganderton L, et al. Psychometric performance of the CAMPHOR and SF-36 in pulmonary hypertension. BMC Pulm Med. 2013;13:45.CrossRef Twiss J, McKenna S, Ganderton L, et al. Psychometric performance of the CAMPHOR and SF-36 in pulmonary hypertension. BMC Pulm Med. 2013;13:45.CrossRef
17.
go back to reference Aguirre-Camacho A, Stepanous J, Blanco-Donoso LM, et al. Adaptation and Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for Use in Spain. Rev Esp Cardiol (Engl Ed). 2017;70(6):467–73.CrossRef Aguirre-Camacho A, Stepanous J, Blanco-Donoso LM, et al. Adaptation and Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for Use in Spain. Rev Esp Cardiol (Engl Ed). 2017;70(6):467–73.CrossRef
18.
go back to reference McCabe C, Bennett M, Doughty N, et al. Patient-reported outcomes assessed by the CAMPHOR questionnaire predict clinical deterioration in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Chest. 2013;144(2):522–30.CrossRef McCabe C, Bennett M, Doughty N, et al. Patient-reported outcomes assessed by the CAMPHOR questionnaire predict clinical deterioration in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Chest. 2013;144(2):522–30.CrossRef
19.
go back to reference Small M, Piercy J, Pike J, et al. Incremental burden of disease in patients diagnosed with pulmonary arterial hypertension receiving monotherapy and combination vasodilator therapy. Adv Ther. 2014;31(2):168–79.CrossRef Small M, Piercy J, Pike J, et al. Incremental burden of disease in patients diagnosed with pulmonary arterial hypertension receiving monotherapy and combination vasodilator therapy. Adv Ther. 2014;31(2):168–79.CrossRef
20.
go back to reference Benza RL, Gomberg-Maitland M, Elliott CG, et al. Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies. Chest. 2019; 156(2):323‐337. Benza RL, Gomberg-Maitland M, Elliott CG, et al. Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies. Chest. 2019; 156(2):323‐337.
21.
go back to reference Swetz KM, Shanafelt TD, Drozdowicz LB, et al. Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: results from a cross-sectional patient survey. J Heart Lung Transplant. 2012;31(10):1102–8.CrossRef Swetz KM, Shanafelt TD, Drozdowicz LB, et al. Symptom burden, quality of life, and attitudes toward palliative care in patients with pulmonary arterial hypertension: results from a cross-sectional patient survey. J Heart Lung Transplant. 2012;31(10):1102–8.CrossRef
22.
go back to reference Halank M, Einsle F, Lehman S, et al. Exercise capacity affects quality of life in patients with pulmonary hypertension. Lung. 2013;191(4):337–43.CrossRef Halank M, Einsle F, Lehman S, et al. Exercise capacity affects quality of life in patients with pulmonary hypertension. Lung. 2013;191(4):337–43.CrossRef
23.
go back to reference Amedro P, Basquin A, Gressin V, et al. Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study. Cardiol Young. 2016;26(7):1250–9.CrossRef Amedro P, Basquin A, Gressin V, et al. Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study. Cardiol Young. 2016;26(7):1250–9.CrossRef
Metadata
Title
Clinical evaluation of pulmonary hypertension using patient-reported outcomes: a cross-sectional study
Authors
Miguel Ángel Amor-García
Sara Ibáñez-García
Xandra García-González
Teresa Mombiela
Cristina Villanueva-Bueno
Ana Herranz-Alonso
María Sanjurjo-Sáez
Publication date
01-12-2021
Publisher
BioMed Central
Published in
BMC Pulmonary Medicine / Issue 1/2021
Electronic ISSN: 1471-2466
DOI
https://doi.org/10.1186/s12890-021-01416-7

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