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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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