Health‑related quality of life in pulmonary arterial hypertension (RCD code: II)

Anna Tyrka, Agnieszka Sarnecka, Kamil Jonas, Marcin Waligóra, Grzegorz Kopeć, Piotr Podolec

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Health-related QoL (HRQoL) is defined as ‘‘the functional effect of an illness and its consequent therapy upon a patient, as perceived by the patient’’ and is a reflection of one’s health on physical, psychological, and social aspects of life. PAH is a condition associated with symptoms that may severely affect patient’s QoL. Awareness of having a rare disease of poor prognosis, with limited therapeutic options and symptoms restricting daily activities may cause anxiety, panic, depression, anger, and hopelessness. Current European Society of Cardiology guidelines suggest that psychosocial support should be considered in patients with PAH (with recommendation class IIa and level of evidence C). PAH is a progressive disease, leading to a gradual reduction in exercise tolerance and significantly decreasing the HRQoL. A recent development of PAH-specific therapies has significantly improved patients’ survival and rate of clinical deterioration. It may be useful to consider patient’s personal outcomes in predicting treatment benefits and decision making. JRCD 2014; 2 (1): 5–8


physical activity, QoL, questionnaire, depression, CAMPHOR


Revicki DA, Osoba D, Fairclough D, et al. Recommendations on health-related quality of life research to support labeling and promotional claims in the united states. Qual Life Res 2000; 9: 887–900.

Galie N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2009; 30: 2493–2537.

Schipper H, Clinch J, Powell V. Definitions and conceptual issues. In: Spilker B (ed.). Quality of life assessments in clinical trials. New York: Raven Press 1990: 11–24.

Hoeper MM, Oudiz RJ, Peacock A, et al. End points and clinical trial designs in pulmonary arterial hypertension: clinical and regulatory perspectives. J Am Coll Cardiol 2004; 43: 48S–55S.

Taichman DB, Shin J, Hud L, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Respir Res 2005; 6: 92.

Wilson IB, Cleary PD. Linking clinical variables with health-related quality of life: a conceptual model of patient outcomes. JAMA 1995; 273: 59–65.

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.

Rubenfire M, Lippo G, Bodini BD, et al.; Evaluating health-related quality of life, work ability, and disability in pulmonary arterial hypertension: an unmet need. Chest 2009; 136: 597–603

Shafazand S, Goldstein MK, Doyle RL, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Chest 2004; 126: 1452–1459

Löwe B, Gräfe K, Ufer C, et al. Anxiety and depression in patients with pulmonary hypertension. Psychosom Med. 2004; 66: 831–836.

de Zwart BC, Frings-Dresen MH, van Duivenbooden JC. Test-retest reliability of the Work Ability Index questionnaire. Occup Med 2002; 52: 177–181.

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.

Hunt SM, McEwen J. The development of a subjective health indicator. Sociol Health Illn 1980; 2: 231–246.

The EuroQol Group. EuroQol–a new facility for the measurement of health-relatedquality of life. Health Policy 1990; 16: 199–208.

Hawthorne G, Richardson J, Osborne R. The assessment of quality of life (aqol) instrument: a psychometric measure of health-related quality of life. Qual Life Res 1999; 8: 209–224.

Cenedese E, Speich R, Dorschner L, et al. Measurement of quality of life in pulmonary hypertension and its significance. Eur Respir J 2006; 28: 808–815.

McKenna SP, Doughty N, Meads DM, et al. The Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR): ameasure of health-related quality of life and quality of life for patients with pulmonary hypertension. Qual Life Res 2006; 15: 103–115.

Gomberg-Maitland M, Thenappan T, Rizvi K, et al. United States validation of the Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR). J Heart Lung Transplant 2008; 27: 124–130.

Chua R, Keogh AM, Byth K, O’Loughlin A. Comparison and validation of three measures of quality of life in patients with pulmonary hypertension. Intern Med J 2006; 36: 705–710.

Sastry BK, Narasimhan C, Reddy NK, Raju BS. Clinical efficacy of sildenafil in primary pulmonary hypertension: a randomized, placebo-controlled, double-blind, crossover study. J Am Coll Cardiol. 2004; 43: 1149–1153.

Pepke-Zaba J, Gilbert C, Collings L, et al. Sildenafil improves health-related quality of life in patients with pulmonary arterial hypertension. Chest 2008; 133: 183–189.

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: 2479–2485.

Keogh A, McNeil K, Wlodarczyk J, et al. Quality of life in pulmonary arterial hypertension: improvement and maintenance with bosentan. J Heart Lung Transplantation 2007; 26: 181–187.

Galie N, Olschewski H, Oudiz RJ, et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. 2008; 117: 3010–3019.

Olschewski H, Simonneau G, Galie N, et al. Inhaled iloprost for severe pulmonary hypertension. N Engl J Med. 2002; 347: 322–329.

Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002; 165: 800–804.

Barst RJ, Rubin LJ, Long WA, et al. A comparison of continuous intravenous epoprostenol(prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med. 1996; 334: 296–302.



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