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Health-related quality of life of patients with pulmonary arterial hypertension associated with CHD: the multicentre cross-sectional ACHILLE study

Published online by Cambridge University Press:  16 March 2016

Pascal Amedro Open the ORCID record for Pascal Amedro [Opens in a new window] ,
Adeline Basquin ,
Virginie Gressin ,
Pierre Clerson ,
Xavier Jais ,
Jean-Benoit Thambo ,
Patrice Guerin ,
Sarah Cohen  and
Damien Bonnet
Pascal Amedro*
Affiliation:
Pediatric and Congenital Cardiology, Arnaud de Villeneuve University Hospital, PhyMedexp, INSERM U1046, CNRS UMR 9214, University of Montpellier, Montpellier, France Department of Public Health, EA3279 Self-perceived Health Assessment Research Unit, Aix-Marseille University, Marseille, France
Adeline Basquin
Affiliation:
Pediatric and Congenital Cardiology, University Hospital, Rennes, France
Virginie Gressin
Affiliation:
Actelion Pharmaceuticals France, Paris, France
Pierre Clerson
Affiliation:
Orgametrie Biostatistics, Roubaix, France
Xavier Jais
Affiliation:
Bicêtre University Hospital, Le Kremlin-Bicêtre, France
Jean-Benoit Thambo
Affiliation:
Pediatric and Congenital Cardiology, Haut-Leveque University Hospital, Bordeaux, France
Patrice Guerin
Affiliation:
Pediatric and Congenital Cardiology, Guillaume and René Laennec University Hospital, Nantes, France
Sarah Cohen
Affiliation:
Adult Congenital Heart Disease Unit, M3C-George Pompidou European Hospital, AP-HP, Paris Descartes University, France INSERM-UMRS 1138, Cordelier Research Center, Team 22, France
Damien Bonnet
Affiliation:
Pediatric and Congenital Cardiology, M3C-Necker University Hospital, AP-HP, Paris Descartes University, Paris, France, France
*
Correspondence to: P. Amedro, Pediatric and Congenital Cardiology, Arnaud de Villeneuve University Hospital, 37, avenue du Doyen Gaston Giraud, 34295 Montpellier Cedex 5, France. Tel: +33 4 67 33 66 39; Fax: +33 4 37 33 21 29; E-mail: p-amedro@chu-montpellier.fr
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Abstract

Background

The aim of this study was to assess health-related quality of life in patients with pulmonary arterial hypertension associated with CHD and correlations with clinical status.

Methods

This prospective cross-sectional observational study included CHD patients with pulmonary arterial hypertension in 14 tertiary-care centres in France. We used two health-related quality of life questionnaires – SF-36 and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) – and one anxiety/depression Hospital Anxiety and Depression Scale (HADS) questionnaire.

Results

Clinical data were collected for the 208 included patients (mean age: 42.6 years, range from 15.1 to 85.8 years, 69.7% female). Most patients were in NYHA functional class II (48.1%) and III (37.5%). Patients’ phenotype was classified as Eisenmenger syndrome (70.7%), pulmonary arterial hypertension associated with systemic-to-pulmonary shunts (12.0%), with small defects (3.4%), or after corrective cardiac surgery (13.9%). In total, 76.4% of the patients were receiving pulmonary arterial hypertension-specific treatments. SF-36 scores showed impairment compared with normalised data. Health-related quality of life scores were significantly lower in females than in males for most dimensions of both questionnaires and were independent of the patients’ phenotype, even after gender adjustment – except for CAMPHOR functioning – but significantly depended on NYHA functional class. The Hospital Anxiety and Depression Scale (HADS) scores suggested anxiety and depression associated with increasing NYHA functional class but independent of patients’ phenotype. NYHA functional class, 6-minute walk distance, HADS, gender, and recent stressful event significantly affected quality of life in the multivariate analysis.

Conclusions

This study showed impairment of quality of life in a large cohort of patients with pulmonary arterial hypertension associated with CHD with both generic and specific questionnaires. NYHA functional class and HADS scores were predictive of most quality of life scores.

Keywords

Health-related quality of lifepulmonary arterial hypertensionCHDEisenmenger syndromeCAMPHOR
Type
Original Articles
Information
Cardiology in the Young , Volume 26 , Issue 7 , October 2016 , pp. 1250 - 1259
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Copyright
© Cambridge University Press 2016 

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References

1. Hoffman, JI, Kaplan, S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39: 1890–1900.CrossRefGoogle ScholarPubMed
2. Marelli, AJ, Mackie, AS, Ionescu-Ittu, R, Rahme, E, Pilote, L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation 2007; 115: 163–172.CrossRefGoogle ScholarPubMed
3. Khairy, P, Ionescu-Ittu, R, Mackie, AS, Abrahamowicz, M, Pilote, L, Marelli, AJ. Changing mortality in congenital heart disease. J Am Coll Cardiol 2010; 56: 1149–1157.CrossRefGoogle ScholarPubMed
4. Duffels, MG, Engelfriet, PM, Berger, RM, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007; 120: 198–204.CrossRefGoogle ScholarPubMed
5. Beghetti, M, Tissot, C. Pulmonary arterial hypertension in congenital heart diseases. Semin Respir Crit Care Med 2009; 30: 421–428.CrossRefGoogle ScholarPubMed
6. Lowe, BS, Therrien, J, Ionescu-Ittu, R, Pilote, L, Martucci, G, Marelli, AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol 2011; 58: 538–546.CrossRefGoogle ScholarPubMed
7. Dimopoulos, K, Inuzuka, R, Goletto, S, et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation 2010; 121: 20–25.CrossRefGoogle ScholarPubMed
8 Guillevin, L, Armstrong, I, Aldrighetti, R, et al. Understanding the impact of pulmonary arterial hypertension on patients’ and carers’ lives. Eur Respir Rev 2013; 22: 535–542.CrossRefGoogle ScholarPubMed
9. 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: 178–184.CrossRefGoogle ScholarPubMed
10. 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: 623–630.CrossRefGoogle ScholarPubMed
11. McLaughlin, VV, Archer, SL, Badesch, DB, 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: 1573–1619.CrossRefGoogle Scholar
12. Becker-Grunig, T, Klose, H, Ehlken, N, et al. Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol 2013; 168: 375–381.CrossRefGoogle ScholarPubMed
13. Duffels, MG, Vis, JC, van Loon, RL, et al. Down patients with Eisenmenger syndrome: is bosentan treatment an option? I J Cardiol 2009; 134: 378–383.Google ScholarPubMed
14. Duffels, MG, Vis, JC, van Loon, RL, 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: 1309–1315.CrossRefGoogle ScholarPubMed
15. Muller, J, Hess, J, Hager, A. Exercise performance and quality of life is more impaired in Eisenmenger syndrome than in complex cyanotic congenital heart disease with pulmonary stenosis. Int J Cardiol 2011; 150: 177–181.CrossRefGoogle ScholarPubMed
16. Tay, EL, Papaphylactou, M, Diller, GP, et al. Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int J Cardiol 2011; 149: 372–376.CrossRefGoogle ScholarPubMed
17. Tay, EL, Peset, A, Papaphylactou, M, et al. Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol 2011; 151: 307–312.CrossRefGoogle ScholarPubMed
18. Simonneau, G, Robbins, IM, Beghetti, M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: S43–S54.CrossRefGoogle ScholarPubMed
19. Ware, JE Jr, Sherbourne, CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care 1992; 30: 473–483.CrossRefGoogle ScholarPubMed
20. Ware, JE Jr, Gandek, B, Kosinski, M, et al. The equivalence of SF-36 summary health scores estimated using standard and country-specific algorithms in 10 countries: results from the IQOLA Project. International Quality of Life Assessment. J Clin Epidemiol 1998; 51: 1167–1170.CrossRefGoogle ScholarPubMed
21. Perneger, TV, Leplege, A, Etter, JF, Rougemont, A. Validation of a French-language version of the MOS 36-Item Short Form Health Survey (SF-36) in young healthy adults. J Clin Epidemiol 1995; 48: 1051–1060.CrossRefGoogle ScholarPubMed
22. Perneger, TV, Leplege, A, Etter, JF. Cross-cultural adaptation of a psychometric instrument: two methods compared. J Clin Epidemiol 1999; 52: 1037–1046.CrossRefGoogle ScholarPubMed
23. 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: 103–115.CrossRefGoogle ScholarPubMed
24. Coffin, D, Duval, K, Martel, S, et al. Adaptation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) into French-Canadian and English-Canadian. Can Respir J 2008; 15: 77–83.CrossRefGoogle ScholarPubMed
25. Zigmond, AS, Snaith, RP. The hospital anxiety and depression scale. Acta Psychiatr Scand 1983; 67: 361–370.CrossRefGoogle ScholarPubMed
26. Crawford, JR, Henry, JD, Crombie, C, Taylor, EP. Normative data for the HADS from a large non-clinical sample. Br J Clin Psychol 2001; 40: 429–434.CrossRefGoogle ScholarPubMed
27. Taichman, DB, Shin, J, Hud, L, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Respir Res 2005; 6: 92.CrossRefGoogle ScholarPubMed
28. Zlupko, M, Harhay, MO, Gallop, R, et al. Evaluation of disease-specific health-related quality of life in patients with pulmonary arterial hypertension. Respir Med 2008; 102: 1431–1438.CrossRefGoogle ScholarPubMed
29. 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: 608–616.CrossRefGoogle ScholarPubMed
30. Dimopoulos, K, Giannakoulas, G, Wort, SJ, Gatzoulis, MA. Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications. Curr Opin Cardiol 2008; 23: 545–554.CrossRefGoogle ScholarPubMed
31. Manes, A, Palazzini, M, Leci, E, Bacchi Reggiani, ML, Branzi, A, Galie, N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J 2014; 35: 716–724.CrossRefGoogle ScholarPubMed
32. Shafazand, S, Goldstein, MK, Doyle, RL, Hlatky, MA, Gould, MK. Health-related quality of life in patients with pulmonary arterial hypertension. Chest 2004; 126: 1452–1459.CrossRefGoogle ScholarPubMed
33. 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.CrossRefGoogle ScholarPubMed
34. McCollister, DH, Beutz, M, McLaughlin, V, et al. Depressive symptoms in pulmonary arterial hypertension: prevalence and association with functional status. Psychosomatics 2010; 51: 339–339 e8.CrossRefGoogle ScholarPubMed
35. Yorke, J, Armstrong, I. The assessment of breathlessness in pulmonary arterial hypertension: reliability and validity of the Dyspnoea-12. Eur J Cardiovasc Nurs 2013.Google ScholarPubMed
36. Halank, M, Einsle, F, Lehman, S, et al. Exercise capacity affects quality of life in patients with pulmonary hypertension. Lung 2013; 191: 337–343.CrossRefGoogle ScholarPubMed
37. McDonough, A, Matura, LA, Carroll, DL. Symptom experience of pulmonary arterial hypertension patients. Clin Nurs Res 2011; 20: 120–134.CrossRefGoogle ScholarPubMed
38. 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.CrossRefGoogle ScholarPubMed
39. Fernandes, CJ, Martins, BC, Jardim, CV, et al. Quality of life as a prognostic marker in pulmonary arterial hypertension. Health Qual Life Outcomes 2014; 12: 130.CrossRefGoogle ScholarPubMed
40. Blok, IM, van Riel, AC, Schuuring, MJ, 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: 278–284.CrossRefGoogle ScholarPubMed
41. 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 Transplant 2008; 27: 124–130.CrossRefGoogle ScholarPubMed
42. Moons, P. Patient-reported outcomes in congenital cardiac disease: are they as good as you think they are? Cardiol Young 2010; 20 (Suppl 3): 143–148.CrossRefGoogle ScholarPubMed
43. Moons, P, Budts, W, De Geest, S. Critique on the conceptualisation of quality of life: a review and evaluation of different conceptual approaches. Int J Nurs Stud 2006; 43: 891–901.CrossRefGoogle ScholarPubMed
44. Marino, BS, Tomlinson, RS, Drotar, D, et al. Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease. Pediatrics 2009; 123: e708–e715.CrossRefGoogle ScholarPubMed