Skip to main content
Top
Published in: PharmacoEconomics 8/2016

01-08-2016 | Systematic Review

Systematic Review of Health-Related Quality of Life in Patients with Pulmonary Arterial Hypertension

Authors: Shuyan Gu, Huimei Hu, Hengjin Dong

Published in: PharmacoEconomics | Issue 8/2016

Login to get access

Abstract

Background

The increasing survival of patients with pulmonary arterial hypertension (PAH) has shifted attention towards the disease burden that PAH imposes on patients and healthcare systems. Most studies emphasize epidemiology and medications, while large observational studies reporting on the health-related quality of life (HRQOL) of patients with PAH are lacking.

Objective

Our objective was to study the HRQOL of patients with PAH and to summarize the factors that influence it.

Methods

We conducted systematic literature searches in English (PubMed, Web of Knowledge, ScienceDirect and OVID) and Chinese (China National Knowledge Infrastructure, Wanfang Data, Chongqing VIP and SinoMed) databases to identify studies published from 2000 to 2015 assessing the HRQOL of patients with PAH. Search results were independently reviewed and extracted by two reviewers.

Results

Of 3392 records identified in the initial search, 20 eligible papers (19 English, 1 Chinese) were finally included. Studies used a range of instruments; the generic 36-item Short Form Survey (SF-36) was the most widely used, and the disease-specific Cambridge Pulmonary Hypertension Outcome Survey (CAMPHOR) was the second mostly widely used. Mean HRQOL scores assessed via the SF-36 (physical component summary [PCS] 25.4–80.1; mental component summary [MCS] 33.2–76.0) and CAMPHOR (symptom scores 3.1–17; total HRQOL 2.8–12.6; activity scores 3.8–18.1) varied across studies, reporting decreased HRQOL in patients. Mental health (depression, anxiety, stress), physical health (exercise capacity, symptoms) and medical therapies were reported to affect HRQOL.

Conclusion

We found that PAH places a substantial burden on patients, particularly in terms of HRQOL; however, the paucity of large observational studies in this area requires the attention of researchers, especially in China.
Appendix
Available only for authorised users
Literature
1.
go back to reference Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004;351:1425–36.CrossRefPubMed Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004;351:1425–36.CrossRefPubMed
4.
go back to reference Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007;131:1917–28.CrossRefPubMed Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007;131:1917–28.CrossRefPubMed
5.
go back to reference Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–54.CrossRefPubMed Simonneau G, Robbins IM, Beghetti M, Channick RN, Delcroix M, Denton CP, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–54.CrossRefPubMed
6.
go back to reference McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573–619.CrossRefPubMed McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573–619.CrossRefPubMed
7.
go back to reference Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43:5S–12S.CrossRefPubMed Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43:5S–12S.CrossRefPubMed
9.
go back to reference Jing ZC, Xu XQ, Han ZY, Wu Y, Deng KW, Wang H, et al. Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest. 2007;132:373–9.CrossRefPubMed Jing ZC, Xu XQ, Han ZY, Wu Y, Deng KW, Wang H, et al. Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension. Chest. 2007;132:373–9.CrossRefPubMed
10.
go back to reference Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137:376–87.CrossRefPubMed Badesch DB, Raskob GE, Elliott CG, Krichman AM, Farber HW, Frost AE, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest. 2010;137:376–87.CrossRefPubMed
11.
go back to reference McDonough A, Matura LA, Carroll DL. Symptom experience of pulmonary arterial hypertension patients. Clin Nurs Res. 2011;20:120–34.CrossRefPubMed McDonough A, Matura LA, Carroll DL. Symptom experience of pulmonary arterial hypertension patients. Clin Nurs Res. 2011;20:120–34.CrossRefPubMed
12.
go back to reference Flattery MP, Pinson JM, Savage L, Salyer J. Living with pulmonary artery hypertension: patients’ experiences. Heart Lung. 2005;34:99–107.CrossRefPubMed Flattery MP, Pinson JM, Savage L, Salyer J. Living with pulmonary artery hypertension: patients’ experiences. Heart Lung. 2005;34:99–107.CrossRefPubMed
13.
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;24:621–9.CrossRefPubMed Delcroix M, Howard L. Pulmonary arterial hypertension: the burden of disease and impact on quality of life. Eur Respir Rev. 2015;24:621–9.CrossRefPubMed
14.
go back to reference Buys R, Avila A, Cornelissen VA. Exercise training improves physical fitness in patients with pulmonary arterial hypertension: a systematic review and meta-analysis of controlled trials. BMC Pulm Med. 2015;15:40.CrossRefPubMedPubMedCentral Buys R, Avila A, Cornelissen VA. Exercise training improves physical fitness in patients with pulmonary arterial hypertension: a systematic review and meta-analysis of controlled trials. BMC Pulm Med. 2015;15:40.CrossRefPubMedPubMedCentral
15.
go back to reference Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023–30.CrossRefPubMed Humbert M, Sitbon O, Chaouat A, Bertocchi M, Habib G, Gressin V, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med. 2006;173:1023–30.CrossRefPubMed
16.
go back to reference Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30:104–9.CrossRefPubMed Peacock AJ, Murphy NF, McMurray JJ, Caballero L, Stewart S. An epidemiological study of pulmonary arterial hypertension. Eur Respir J. 2007;30:104–9.CrossRefPubMed
17.
go back to reference Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USA-based registry for pulmonary arterial hypertension: 1982–2006. Eur Respir J. 2007;30:1103–10.CrossRefPubMed Thenappan T, Shah SJ, Rich S, Gomberg-Maitland M. A USA-based registry for pulmonary arterial hypertension: 1982–2006. Eur Respir J. 2007;30:1103–10.CrossRefPubMed
18.
go back to reference Mikhail GW, Prasad SK, Li W, Rogers P, Chester AH, Bayne S, et al. Clinical and haemodynamic effects of sildenafil in pulmonary hypertension: acute and mid-term effects. Eur Heart J. 2004;25:431–6.CrossRefPubMed Mikhail GW, Prasad SK, Li W, Rogers P, Chester AH, Bayne S, et al. Clinical and haemodynamic effects of sildenafil in pulmonary hypertension: acute and mid-term effects. Eur Heart J. 2004;25:431–6.CrossRefPubMed
19.
go back to reference Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353:2148–57.CrossRefPubMed Galie N, Ghofrani HA, Torbicki A, Barst RJ, Rubin LJ, Badesch D, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353:2148–57.CrossRefPubMed
20.
go back to reference Barst RJ, Langleben D, Badesch D, Frost A, Lawrence EC, Shapiro S, et al. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol. 2006;47:2049–56.CrossRefPubMed Barst RJ, Langleben D, Badesch D, Frost A, Lawrence EC, Shapiro S, et al. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol. 2006;47:2049–56.CrossRefPubMed
21.
go back to reference Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142:448–56.CrossRefPubMed Benza RL, Miller DP, Barst RJ, Badesch DB, Frost AE, McGoon MD. An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest. 2012;142:448–56.CrossRefPubMed
22.
go back to reference McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galie N, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. 2005;25:244–9.CrossRefPubMed McLaughlin VV, Sitbon O, Badesch DB, Barst RJ, Black C, Galie N, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J. 2005;25:244–9.CrossRefPubMed
23.
go back to reference Galie N, Badesch D, Oudiz R, Simonneau G, McGoon MD, Keogh AM, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2005;46:529–35.CrossRefPubMed Galie N, Badesch D, Oudiz R, Simonneau G, McGoon MD, Keogh AM, et al. Ambrisentan therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2005;46:529–35.CrossRefPubMed
24.
go back to reference Galie N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. 2009;30:394–403.CrossRefPubMedPubMedCentral Galie N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. 2009;30:394–403.CrossRefPubMedPubMedCentral
25.
go back to reference Cha KS, Cho KI, Seo JS, Choi JH, Park YH, Yang DH, et al. Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study). Am J Cardiol. 2013;112:1834–9.CrossRefPubMed Cha KS, Cho KI, Seo JS, Choi JH, Park YH, Yang DH, et al. Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study). Am J Cardiol. 2013;112:1834–9.CrossRefPubMed
26.
go back to reference Duffels M, Vis JC, van Loon R, Nieuwkerk PT, van Dijk A, Hoendermis ES, et al. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down’s syndrome. Am J Cardiol. 2009;103:1309–15.CrossRefPubMed Duffels M, Vis JC, van Loon R, Nieuwkerk PT, van Dijk A, Hoendermis ES, et al. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down’s syndrome. Am J Cardiol. 2009;103:1309–15.CrossRefPubMed
27.
go back to reference Vis JC, Duffels MG, Mulder P, de Bruin-Bon RH, Bouma BJ, Berger RM, et al. Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol. 2013;164:64–9.CrossRefPubMed Vis JC, Duffels MG, Mulder P, de Bruin-Bon RH, Bouma BJ, Berger RM, et al. Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol. 2013;164:64–9.CrossRefPubMed
28.
go back to reference Chen YF, Jowett S, Barton P, Malottki K, Hyde C, Gibbs JS, et al. Clinical and cost-effectiveness of epoprostenol, iloprost, bosentan, sitaxentan and sildenafil for pulmonary arterial hypertension within their licensed indications: a systematic review and economic evaluation. Health Technol Assess. 2009;13:1–320.CrossRefPubMed Chen YF, Jowett S, Barton P, Malottki K, Hyde C, Gibbs JS, et al. Clinical and cost-effectiveness of epoprostenol, iloprost, bosentan, sitaxentan and sildenafil for pulmonary arterial hypertension within their licensed indications: a systematic review and economic evaluation. Health Technol Assess. 2009;13:1–320.CrossRefPubMed
29.
go back to reference Mazzanti G, Albini A, Palazzini M, Monti E, Bachetti C, Rinaldi A, et al. A randomized open label study comparing first-line treatment with bosentan or sildenafil in Pulmonary Arterial Hypertension (PAH). Eur Heart J. 2013;341:186–7. Mazzanti G, Albini A, Palazzini M, Monti E, Bachetti C, Rinaldi A, et al. A randomized open label study comparing first-line treatment with bosentan or sildenafil in Pulmonary Arterial Hypertension (PAH). Eur Heart J. 2013;341:186–7.
30.
go back to reference Crackett RM, Hall A, Day MB, Desoyza JR, Small T, Fisher AJ, et al. Treatment outcomes in pulmonary arterial hypertension (PHA) associated with systemic sclerosis (Sscl.) in matched groups using either bosentan or sildenafil monotherapy. J Heart Lung Transpl. 2013;32S:S301.CrossRef Crackett RM, Hall A, Day MB, Desoyza JR, Small T, Fisher AJ, et al. Treatment outcomes in pulmonary arterial hypertension (PHA) associated with systemic sclerosis (Sscl.) in matched groups using either bosentan or sildenafil monotherapy. J Heart Lung Transpl. 2013;32S:S301.CrossRef
31.
go back to reference Matura LA, Carroll DL. Human responses to pulmonary arterial hypertension: review of the literature. J Cardiovasc Nurs. 2010;25:420–7.CrossRefPubMed Matura LA, Carroll DL. Human responses to pulmonary arterial hypertension: review of the literature. J Cardiovasc Nurs. 2010;25:420–7.CrossRefPubMed
32.
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 Thoracic Soc. 2008;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 Thoracic Soc. 2008;5:623–30.CrossRef
33.
go back to reference Rival G, Lacasse Y, Martin S, Bonnet S, Provencher S. Effect of pulmonary arterial hypertension-specific therapies on health-related quality of life: a systematic review. Chest. 2014;146:686–708.CrossRefPubMed Rival G, Lacasse Y, Martin S, Bonnet S, Provencher S. Effect of pulmonary arterial hypertension-specific therapies on health-related quality of life: a systematic review. Chest. 2014;146:686–708.CrossRefPubMed
34.
go back to reference Zeng X, Zhang Y, Kwong JS, Zhang C, Li S, Sun F, et al. The methodological quality assessment tools for preclinical and clinical studies, systematic review and meta-analysis, and clinical practice guideline: a systematic review. J Evid Based Med. 2015;8:2–10.CrossRefPubMed Zeng X, Zhang Y, Kwong JS, Zhang C, Li S, Sun F, et al. The methodological quality assessment tools for preclinical and clinical studies, systematic review and meta-analysis, and clinical practice guideline: a systematic review. J Evid Based Med. 2015;8:2–10.CrossRefPubMed
35.
go back to reference Zeng X, Liu H, Chen X, Leng W. Meta-analysis series 4: the methodological quality assessment tools for observational study. Chinese J Evid Based Cardiovasc Med. 2012;04:297–9. Zeng X, Liu H, Chen X, Leng W. Meta-analysis series 4: the methodological quality assessment tools for observational study. Chinese J Evid Based Cardiovasc Med. 2012;04:297–9.
36.
go back to reference Cao S. The application of meta-analysis of observational studies in social factors and health—illustrated by the case of smoking. Hubei: Huazhong University Of Science And Technology; 2014. Cao S. The application of meta-analysis of observational studies in social factors and health—illustrated by the case of smoking. Hubei: Huazhong University Of Science And Technology; 2014.
37.
go back to reference Rostom A, Dubé C, Cranney A, Saloojee N, Sy R, Mack D, et al. Celiac disease. Rockville (MD): US Agency for Healthcare Research and Quality; 2004 Sep. (Evidence Reports/Technology Assessments, No. 104.) Appendix D. Quality Assessment Forms [online]. http://www.ncbi.nlm.nih.gov/books/NBK35156/. Accessed 14 Dec 2015. Rostom A, Dubé C, Cranney A, Saloojee N, Sy R, Mack D, et al. Celiac disease. Rockville (MD): US Agency for Healthcare Research and Quality; 2004 Sep. (Evidence Reports/Technology Assessments, No. 104.) Appendix D. Quality Assessment Forms [online]. http://​www.​ncbi.​nlm.​nih.​gov/​books/​NBK35156/​. Accessed 14 Dec 2015.
39.
go back to reference Stang A. Critical evaluation of the Newcastle-Ottawa scale for the assessment of the quality of nonrandomized studies in meta-analyses. Eur J Epidemiol. 2010;25:603–5.CrossRefPubMed Stang A. Critical evaluation of the Newcastle-Ottawa scale for the assessment of the quality of nonrandomized studies in meta-analyses. Eur J Epidemiol. 2010;25:603–5.CrossRefPubMed
40.
go back to reference Shafazand S, Goldstein MK, Doyle RL, Hlatky MA, Gould MK. Health-related quality of life in patients with pulmonary arterial hypertension. Chest. 2004;126:1452–9.CrossRefPubMed Shafazand S, Goldstein MK, Doyle RL, Hlatky MA, Gould MK. Health-related quality of life in patients with pulmonary arterial hypertension. Chest. 2004;126:1452–9.CrossRefPubMed
41.
go back to reference Taichman DB, Shin J, Hud L, Archer-Chicko C, Kaplan S, Sager JS, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Respir Res. 2005;6. Taichman DB, Shin J, Hud L, Archer-Chicko C, Kaplan S, Sager JS, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Respir Res. 2005;6.
42.
go back to reference White J, Hopkins RO, Glissmeyer EW, Kitterman N, Elliott CG. Cognitive, emotional, and quality of life outcomes in patients with pulmonary arterial hypertension. Respir Res. 2006;7:55.CrossRefPubMedPubMedCentral White J, Hopkins RO, Glissmeyer EW, Kitterman N, Elliott CG. Cognitive, emotional, and quality of life outcomes in patients with pulmonary arterial hypertension. Respir Res. 2006;7:55.CrossRefPubMedPubMedCentral
43.
go back to reference Zlupko M, Harhay MO, Gallop R, Shin J, Archer-Chicko C, Patel R, et al. Evaluation of disease-specific health-related quality of life in patients with pulmonary arterial hypertension. Respir Med. 2008;102:1431–8.CrossRefPubMed Zlupko M, Harhay MO, Gallop R, Shin J, Archer-Chicko C, Patel R, et al. Evaluation of disease-specific health-related quality of life in patients with pulmonary arterial hypertension. Respir Med. 2008;102:1431–8.CrossRefPubMed
44.
go back to reference Chen H, De Marco T, Kobashigawa EA, Katz PP, Chang VW, Blanc PD. Comparison of cardiac and pulmonary-specific quality-of-life measures in pulmonary arterial hypertension. Eur Respir J. 2011;38:608–16.CrossRefPubMedPubMedCentral Chen H, De Marco T, Kobashigawa EA, Katz PP, Chang VW, Blanc PD. Comparison of cardiac and pulmonary-specific quality-of-life measures in pulmonary arterial hypertension. Eur Respir J. 2011;38:608–16.CrossRefPubMedPubMedCentral
45.
go back to reference Matura LA, McDonough A, Carroll DL. Cluster analysis of symptoms in pulmonary arterial hypertension: a pilot study. Eur J Cardiovasc Nurs. 2012;11:51–61.CrossRefPubMed Matura LA, McDonough A, Carroll DL. Cluster analysis of symptoms in pulmonary arterial hypertension: a pilot study. Eur J Cardiovasc Nurs. 2012;11:51–61.CrossRefPubMed
46.
go back to reference Matura LA, McDonough A, Carroll DL. Predictors of health-related quality of life in patients with idiopathic pulmonary arterial hypertension. J Hosp Palliat Nurs. 2012;14:283–92.CrossRef Matura LA, McDonough A, Carroll DL. Predictors of health-related quality of life in patients with idiopathic pulmonary arterial hypertension. J Hosp Palliat Nurs. 2012;14:283–92.CrossRef
47.
go back to reference Swetz KM, Shanafelt TD, Drozdowicz LB, Sloan JA, Novotny PJ, Durst LA, 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 Transpl. 2012;31:1102–8.CrossRef Swetz KM, Shanafelt TD, Drozdowicz LB, Sloan JA, Novotny PJ, Durst LA, 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 Transpl. 2012;31:1102–8.CrossRef
48.
go back to reference Matura LA, McDonough A, Carroll DL. Health-related quality of life and psychological states in patients with pulmonary arterial hypertension. J Cardiovasc Nurs. 2014;29:178–84.CrossRefPubMed Matura LA, McDonough A, Carroll DL. Health-related quality of life and psychological states in patients with pulmonary arterial hypertension. J Cardiovasc Nurs. 2014;29:178–84.CrossRefPubMed
49.
go back to reference Matura LA, McDonough A, Hanlon AL, Carroll DL, Riegel B. Sleep disturbance, symptoms, psychological distress, and health-related quality of life in pulmonary arterial hypertension. Eur J Cardiovasc Nurs. 2015;14:423–30.CrossRefPubMed Matura LA, McDonough A, Hanlon AL, Carroll DL, Riegel B. Sleep disturbance, symptoms, psychological distress, and health-related quality of life in pulmonary arterial hypertension. Eur J Cardiovasc Nurs. 2015;14:423–30.CrossRefPubMed
50.
go back to reference Talwar A, Sahni S, Kim EJ, Verma S, Kohn N. Dyspnea, depression and health related quality of life in pulmonary arterial hypertension patients. J Exerc Rehabil. 2015;11:259–65.CrossRefPubMedPubMedCentral Talwar A, Sahni S, Kim EJ, Verma S, Kohn N. Dyspnea, depression and health related quality of life in pulmonary arterial hypertension patients. J Exerc Rehabil. 2015;11:259–65.CrossRefPubMedPubMedCentral
51.
go back to reference Matura LA, McDonough A, Carroll DL. Symptom interference severity and health-related quality of life in pulmonary arterial hypertension. J Pain Symptom Manage. 2016;51:25–32.CrossRefPubMed Matura LA, McDonough A, Carroll DL. Symptom interference severity and health-related quality of life in pulmonary arterial hypertension. J Pain Symptom Manage. 2016;51:25–32.CrossRefPubMed
52.
go back to reference Wilkens H, Grimminger F, Hoeper M, Stahler G, Ehlken B, Plesnila-Frank C, et al. Burden of pulmonary arterial hypertension in Germany. Respir Med. 2010;104:902–10.CrossRefPubMed Wilkens H, Grimminger F, Hoeper M, Stahler G, Ehlken B, Plesnila-Frank C, et al. Burden of pulmonary arterial hypertension in Germany. Respir Med. 2010;104:902–10.CrossRefPubMed
53.
go back to reference Roman A, Barbera JA, Castillo MJ, Munoz R, Escribano P. Health-related quality of life in a national cohort of patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Arch Bronconeumol. 2013;49:181–8.CrossRefPubMed Roman A, Barbera JA, Castillo MJ, Munoz R, Escribano P. Health-related quality of life in a national cohort of patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Arch Bronconeumol. 2013;49:181–8.CrossRefPubMed
54.
go back to reference McCabe C, Bennett M, Doughty N, Ross RM, Sharples L, Pepke-Zaba J. Patient-reported outcomes assessed by the CAMPHOR questionnaire predict clinical deterioration in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Chest. 2013;144:522–30.CrossRefPubMedPubMedCentral McCabe C, Bennett M, Doughty N, Ross RM, Sharples L, Pepke-Zaba J. Patient-reported outcomes assessed by the CAMPHOR questionnaire predict clinical deterioration in idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Chest. 2013;144:522–30.CrossRefPubMedPubMedCentral
55.
go back to reference Vanhoof JMM, Delcroix M, Vandevelde E, Denhaerynck K, Wuyts W, Belge C, et al. Emotional symptoms and quality of life in patients with pulmonary arterial hypertension. J Heart Lung Transpl. 2014;33:800–8.CrossRef Vanhoof JMM, Delcroix M, Vandevelde E, Denhaerynck K, Wuyts W, Belge C, et al. Emotional symptoms and quality of life in patients with pulmonary arterial hypertension. J Heart Lung Transpl. 2014;33:800–8.CrossRef
56.
go back to reference Cui M, Lin M, Zhang Q. The correlation between health behavior and quality of life in patients with pulmonary arterial hypertension. Chin J Prevent Control Chronic Dis. 2014;22:202–4. Cui M, Lin M, Zhang Q. The correlation between health behavior and quality of life in patients with pulmonary arterial hypertension. Chin J Prevent Control Chronic Dis. 2014;22:202–4.
57.
go back to reference Small M, Piercy J, Pike J, Cerulli A. Incremental burden of disease in patients diagnosed with pulmonary arterial hypertension receiving monotherapy and combination vasodilator therapy. Adv Ther. 2014;31:168–79.CrossRefPubMed Small M, Piercy J, Pike J, Cerulli A. Incremental burden of disease in patients diagnosed with pulmonary arterial hypertension receiving monotherapy and combination vasodilator therapy. Adv Ther. 2014;31:168–79.CrossRefPubMed
58.
go back to reference Yorke J, Armstrong I. The assessment of breathlessness in pulmonary arterial hypertension: reliability and validity of the Dyspnoea-12. Eur J Cardiovasc Nurs. 2014;13:506–14.CrossRefPubMed Yorke J, Armstrong I. The assessment of breathlessness in pulmonary arterial hypertension: reliability and validity of the Dyspnoea-12. Eur J Cardiovasc Nurs. 2014;13:506–14.CrossRefPubMed
59.
go back to reference Blok IM, van Riel A, Schuuring MJ, Duffels MG, Vis JC, van Dijk A, et al. Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease. Neth Heart J. 2015;23:278–84.CrossRefPubMedPubMedCentral Blok IM, van Riel A, Schuuring MJ, Duffels MG, Vis JC, van Dijk A, et al. Decrease in quality of life predicts mortality in adult patients with pulmonary arterial hypertension due to congenital heart disease. Neth Heart J. 2015;23:278–84.CrossRefPubMedPubMedCentral
60.
go back to reference Allen LA, Stevenson LW, Grady KL, Goldstein NE, Matlock DD, Arnold RM, et al. Decision making in advanced heart failure: a scientific statement from the American Heart Association. Circulation. 2012;125:1928–52.CrossRefPubMedPubMedCentral Allen LA, Stevenson LW, Grady KL, Goldstein NE, Matlock DD, Arnold RM, et al. Decision making in advanced heart failure: a scientific statement from the American Heart Association. Circulation. 2012;125:1928–52.CrossRefPubMedPubMedCentral
61.
go back to reference Tartavoulle T. A predictive model of the effects of depression, anxiety, stress, six minute walk distance, and social support on health related quality of life in an adult pulmonary hypertension population. Clin Nurse Spec. 2014;28:E40.CrossRef Tartavoulle T. A predictive model of the effects of depression, anxiety, stress, six minute walk distance, and social support on health related quality of life in an adult pulmonary hypertension population. Clin Nurse Spec. 2014;28:E40.CrossRef
62.
go back to reference Batal O, Khatib OF, Bair N, Aboussouan LS, Minai OA. Sleep quality, depression, and quality of life in patients with pulmonary hypertension. Lung. 2011;189:141–9.CrossRefPubMed Batal O, Khatib OF, Bair N, Aboussouan LS, Minai OA. Sleep quality, depression, and quality of life in patients with pulmonary hypertension. Lung. 2011;189:141–9.CrossRefPubMed
63.
go back to reference Ulrich S, Fischler M, Speich R, Bloch KE. Sleep-related breathing disorders in patients with pulmonary hypertension. Chest. 2008;133:1375–80.CrossRefPubMed Ulrich S, Fischler M, Speich R, Bloch KE. Sleep-related breathing disorders in patients with pulmonary hypertension. Chest. 2008;133:1375–80.CrossRefPubMed
64.
go back to reference McCollister DH, Beutz M, McLaughlin V, Rumsfeld J, Masoudi FA, Tripputi M, et al. Depressive symptoms in pulmonary arterial hypertension: prevalence and association with functional status. Psychosomatics. 2010;51:339.CrossRefPubMed McCollister DH, Beutz M, McLaughlin V, Rumsfeld J, Masoudi FA, Tripputi M, et al. Depressive symptoms in pulmonary arterial hypertension: prevalence and association with functional status. Psychosomatics. 2010;51:339.CrossRefPubMed
65.
go back to reference Lowe B, Grafe K, Ufer C, Kroenke K, Grunig E, Herzog W, et al. Anxiety and depression in patients with pulmonary hypertension. Psychosom Med. 2004;66:831–6.CrossRefPubMed Lowe B, Grafe K, Ufer C, Kroenke K, Grunig E, Herzog W, et al. Anxiety and depression in patients with pulmonary hypertension. Psychosom Med. 2004;66:831–6.CrossRefPubMed
67.
go back to reference Harzheim D, Klose H, Pinado FP, Ehlken N, Nagel C, Fischer C, et al. Anxiety and depression disorders in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Respir Res. 2013;14:104.CrossRefPubMedPubMedCentral Harzheim D, Klose H, Pinado FP, Ehlken N, Nagel C, Fischer C, et al. Anxiety and depression disorders in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Respir Res. 2013;14:104.CrossRefPubMedPubMedCentral
68.
go back to reference Kim EJ, Verma S, Sahni S, Athaanasiou K, Patel P, Kohn N, et al. Dyspnea, depression, and health related quality of life [HRQOL] in patients with pulmonary artery hypertension [PAH]. Chest. 2013;144S. Kim EJ, Verma S, Sahni S, Athaanasiou K, Patel P, Kohn N, et al. Dyspnea, depression, and health related quality of life [HRQOL] in patients with pulmonary artery hypertension [PAH]. Chest. 2013;144S.
69.
go back to reference Halank M, Einsle F, Lehman S, Bremer H, Ewert R, Wilkens H, et al. Exercise capacity affects quality of life in patients with pulmonary hypertension. Lung. 2013;191:337–43.CrossRefPubMed Halank M, Einsle F, Lehman S, Bremer H, Ewert R, Wilkens H, et al. Exercise capacity affects quality of life in patients with pulmonary hypertension. Lung. 2013;191:337–43.CrossRefPubMed
70.
go back to reference Pepke-Zaba J, Beardsworth A, Chan M, Angalakuditi M. Tadalafil therapy and health-related quality of life in pulmonary arterial hypertension. Curr Med Res Opin. 2009;25:2479–85.CrossRefPubMed Pepke-Zaba J, Beardsworth A, Chan M, Angalakuditi M. Tadalafil therapy and health-related quality of life in pulmonary arterial hypertension. Curr Med Res Opin. 2009;25:2479–85.CrossRefPubMed
71.
go back to reference Cicero C, Franchi SM, Barreto AC, Lopes AA. Lack of tight association between quality of life and exercise capacity in pulmonary arterial hypertension. Arq Bras Cardiol. 2012;99:876–85.CrossRefPubMed Cicero C, Franchi SM, Barreto AC, Lopes AA. Lack of tight association between quality of life and exercise capacity in pulmonary arterial hypertension. Arq Bras Cardiol. 2012;99:876–85.CrossRefPubMed
72.
go back to reference Keogh AM, McNeil KD, Wlodarczyk J, Gabbay E, Williams TJ. Quality of life in pulmonary arterial hypertension: improvement and maintenance with bosentan. J Heart Lung Transpl. 2007;26:181–7.CrossRef Keogh AM, McNeil KD, Wlodarczyk J, Gabbay E, Williams TJ. Quality of life in pulmonary arterial hypertension: improvement and maintenance with bosentan. J Heart Lung Transpl. 2007;26:181–7.CrossRef
73.
go back to reference Wang RC, Jiang FM, Zheng QL, Li CT, Peng XY, He CY, et al. Efficacy and safety of sildenafil treatment in pulmonary arterial hypertension: a systematic review. Respir Med. 2014;108:531–7.CrossRefPubMed Wang RC, Jiang FM, Zheng QL, Li CT, Peng XY, He CY, et al. Efficacy and safety of sildenafil treatment in pulmonary arterial hypertension: a systematic review. Respir Med. 2014;108:531–7.CrossRefPubMed
74.
go back to reference Udeoji DU, Schwarz ER. Tadalafil as monotherapy and in combination regimens for the treatment of pulmonary arterial hypertension. Ther Adv Respir Dis. 2013;7:39–49.CrossRefPubMed Udeoji DU, Schwarz ER. Tadalafil as monotherapy and in combination regimens for the treatment of pulmonary arterial hypertension. Ther Adv Respir Dis. 2013;7:39–49.CrossRefPubMed
75.
go back to reference Croxtall JD, Lyseng-Williamson KA. Tadalafil: in pulmonary arterial hypertension. Drugs. 2010;70:479–88.CrossRefPubMed Croxtall JD, Lyseng-Williamson KA. Tadalafil: in pulmonary arterial hypertension. Drugs. 2010;70:479–88.CrossRefPubMed
76.
go back to reference Somaini SU, Vatandaslar S, Huber LC, Treder U, Breitenstein A, Speich R. Imatinib improves hemodynamics and quality of life in pulmonary arterial hypertension. Am J Resp Crit Care. 2011;183. Somaini SU, Vatandaslar S, Huber LC, Treder U, Breitenstein A, Speich R. Imatinib improves hemodynamics and quality of life in pulmonary arterial hypertension. Am J Resp Crit Care. 2011;183.
77.
go back to reference Jacobs W, Vonk-Noordegraaf A. Epoprostenol in pulmonary arterial hypertension. Expert Opin Drug Metab Toxicol. 2009;5:83–90.CrossRefPubMed Jacobs W, Vonk-Noordegraaf A. Epoprostenol in pulmonary arterial hypertension. Expert Opin Drug Metab Toxicol. 2009;5:83–90.CrossRefPubMed
78.
go back to reference Ewert R, Glaser S, Bollmann T, Schaper C. Inhaled iloprost for therapy in pulmonary arterial hypertension. Expert Rev Respir Med. 2011;5:145–52.CrossRefPubMed Ewert R, Glaser S, Bollmann T, Schaper C. Inhaled iloprost for therapy in pulmonary arterial hypertension. Expert Rev Respir Med. 2011;5:145–52.CrossRefPubMed
79.
go back to reference Schuuring MJ, Vis JC, van Dijk AP, van Melle JP, Vliegen HW, Pieper PG, et al. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail. 2013;15:690–8.CrossRefPubMed Schuuring MJ, Vis JC, van Dijk AP, van Melle JP, Vliegen HW, Pieper PG, et al. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail. 2013;15:690–8.CrossRefPubMed
80.
go back to reference McLaughlin VV. Medical management of primary pulmonary hypertension. Expert Opin Pharmacother. 2002;3:159–65.CrossRefPubMed McLaughlin VV. Medical management of primary pulmonary hypertension. Expert Opin Pharmacother. 2002;3:159–65.CrossRefPubMed
81.
go back to reference Chen H, Rosenzweig EB, Gotzkowsky SK, Arneson C, Nelsen AC, Bourge RC. Treatment satisfaction is associated with improved quality of life in patients treated with inhaled treprostinil for pulmonary arterial hypertension. Health Qual Life Outcomes. 2013;11:31.CrossRefPubMedPubMedCentral Chen H, Rosenzweig EB, Gotzkowsky SK, Arneson C, Nelsen AC, Bourge RC. Treatment satisfaction is associated with improved quality of life in patients treated with inhaled treprostinil for pulmonary arterial hypertension. Health Qual Life Outcomes. 2013;11:31.CrossRefPubMedPubMedCentral
82.
go back to reference Rubenfire M, Lippo G, Bodini BD, Blasi F, Allegra L, Bossone E. Evaluating health-related quality of life, work ability, and disability in pulmonary arterial hypertension: an unmet need. Chest. 2009;136:597–603.CrossRefPubMed Rubenfire M, Lippo G, Bodini BD, Blasi F, Allegra L, Bossone E. Evaluating health-related quality of life, work ability, and disability in pulmonary arterial hypertension: an unmet need. Chest. 2009;136:597–603.CrossRefPubMed
83.
go back to reference Cenedese E, Speich R, Dorschner L, Ulrich S, Maggiorini M, Jenni R, et al. Measurement of quality of life in pulmonary hypertension and its significance. Eur Respir J. 2006;28:808–15.CrossRefPubMed Cenedese E, Speich R, Dorschner L, Ulrich S, Maggiorini M, Jenni R, et al. Measurement of quality of life in pulmonary hypertension and its significance. Eur Respir J. 2006;28:808–15.CrossRefPubMed
84.
go back to reference Gomberg-Maitland M, Thenappan T, Rizvi K, Chandra S, Meads DM, McKenna SP. United States validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). J Heart Lung Transpl. 2008;27:124–30.CrossRef Gomberg-Maitland M, Thenappan T, Rizvi K, Chandra S, Meads DM, McKenna SP. United States validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). J Heart Lung Transpl. 2008;27:124–30.CrossRef
85.
go back to reference Coffin D, Duval K, Martel S, Granton J, Lefebvre MC, Meads DM, et al. Adaptation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) into French-Canadian and English-Canadian. Can Respir J. 2008;15:77–83.CrossRefPubMedPubMedCentral Coffin D, Duval K, Martel S, Granton J, Lefebvre MC, Meads DM, et al. Adaptation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) into French-Canadian and English-Canadian. Can Respir J. 2008;15:77–83.CrossRefPubMedPubMedCentral
86.
go back to reference Ganderton L, Jenkins S, McKenna SP, Gain K, Fowler R, Twiss J, et al. Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Australian and New Zealand population. Respirology. 2011;16:1235–40.CrossRefPubMed Ganderton L, Jenkins S, McKenna SP, Gain K, Fowler R, Twiss J, et al. Validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) for the Australian and New Zealand population. Respirology. 2011;16:1235–40.CrossRefPubMed
87.
go back to reference Yorke J, Corris P, Gaine S, Gibbs JS, Kiely DG, Harries C, et al. emPHasis-10: development of a health-related quality of life measure in pulmonary hypertension. Eur Respir J. 2014;43:1106–13.CrossRefPubMed Yorke J, Corris P, Gaine S, Gibbs JS, Kiely DG, Harries C, et al. emPHasis-10: development of a health-related quality of life measure in pulmonary hypertension. Eur Respir J. 2014;43:1106–13.CrossRefPubMed
88.
go back to reference Brundage M, Blazeby J, Revicki D, Bass B, de Vet H, Duffy H, et al. Patient-reported outcomes in randomized clinical trials: development of ISOQOL reporting standards. Qual Life Res. 2013;22:1161–75.CrossRefPubMed Brundage M, Blazeby J, Revicki D, Bass B, de Vet H, Duffy H, et al. Patient-reported outcomes in randomized clinical trials: development of ISOQOL reporting standards. Qual Life Res. 2013;22:1161–75.CrossRefPubMed
89.
go back to reference Kirson NY, Birnbaum HG, Ivanova JI, Waldman T, Joish V, Williamson T. Excess costs associated with patients with pulmonary arterial hypertension in a US privately insured population. Appl Health Econ Health Policy. 2011;9:293–303.CrossRefPubMed Kirson NY, Birnbaum HG, Ivanova JI, Waldman T, Joish V, Williamson T. Excess costs associated with patients with pulmonary arterial hypertension in a US privately insured population. Appl Health Econ Health Policy. 2011;9:293–303.CrossRefPubMed
90.
go back to reference Krumholz HM. Patient-centered medicine: the next phase in health care. Circ Cardiovasc Qual Outcomes. 2011;4:374–5.CrossRefPubMed Krumholz HM. Patient-centered medicine: the next phase in health care. Circ Cardiovasc Qual Outcomes. 2011;4:374–5.CrossRefPubMed
91.
go back to reference Charles C, Gafni A, Whelan T. Shared decision-making in the medical encounter: what does it mean? (or it takes at least two to tango). Soc Sci Med. 1997;44:681–92.CrossRefPubMed Charles C, Gafni A, Whelan T. Shared decision-making in the medical encounter: what does it mean? (or it takes at least two to tango). Soc Sci Med. 1997;44:681–92.CrossRefPubMed
92.
go back to reference Goodlin SJ, Hauptman PJ, Arnold R, Grady K, Hershberger RE, Kutner J, et al. Consensus statement: palliative and supportive care in advanced heart failure. J Card Fail. 2004;10:200–9.CrossRefPubMed Goodlin SJ, Hauptman PJ, Arnold R, Grady K, Hershberger RE, Kutner J, et al. Consensus statement: palliative and supportive care in advanced heart failure. J Card Fail. 2004;10:200–9.CrossRefPubMed
93.
go back to reference Jaarsma T, Beattie JM, Ryder M, Rutten FH, McDonagh T, Mohacsi P, et al. Palliative care in heart failure: a position statement from the palliative care workshop of the Heart Failure Association of the European Society of Cardiology. Eur J Heart Fail. 2009;11:433–43.CrossRefPubMed Jaarsma T, Beattie JM, Ryder M, Rutten FH, McDonagh T, Mohacsi P, et al. Palliative care in heart failure: a position statement from the palliative care workshop of the Heart Failure Association of the European Society of Cardiology. Eur J Heart Fail. 2009;11:433–43.CrossRefPubMed
94.
go back to reference Pantilat SZ, Steimle AE. Palliative care for patients with heart failure. JAMA. 2004;291:2476–82.CrossRefPubMed Pantilat SZ, Steimle AE. Palliative care for patients with heart failure. JAMA. 2004;291:2476–82.CrossRefPubMed
95.
go back to reference Rizzieri AG, Verheijde JL, Rady MY, McGregor JL. Ethical challenges with the left ventricular assist device as a destination therapy. Philos Ethics Humanit Med. 2008;3:20.CrossRefPubMedPubMedCentral Rizzieri AG, Verheijde JL, Rady MY, McGregor JL. Ethical challenges with the left ventricular assist device as a destination therapy. Philos Ethics Humanit Med. 2008;3:20.CrossRefPubMedPubMedCentral
96.
go back to reference Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, 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 Respir J. 2015;46:903–75.CrossRefPubMed Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, 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 Respir J. 2015;46:903–75.CrossRefPubMed
97.
go back to reference Arena R, Cahalin LP, Borghi-Silva A, Myers J. The effect of exercise training on the pulmonary arterial system in patients with pulmonary hypertension. Prog Cardiovasc Dis. 2015;57:480–8.CrossRefPubMed Arena R, Cahalin LP, Borghi-Silva A, Myers J. The effect of exercise training on the pulmonary arterial system in patients with pulmonary hypertension. Prog Cardiovasc Dis. 2015;57:480–8.CrossRefPubMed
Metadata
Title
Systematic Review of Health-Related Quality of Life in Patients with Pulmonary Arterial Hypertension
Authors
Shuyan Gu
Huimei Hu
Hengjin Dong
Publication date
01-08-2016
Publisher
Springer International Publishing
Published in
PharmacoEconomics / Issue 8/2016
Print ISSN: 1170-7690
Electronic ISSN: 1179-2027
DOI
https://doi.org/10.1007/s40273-016-0395-y

Other articles of this Issue 8/2016

PharmacoEconomics 8/2016 Go to the issue