Prognostic impact of pulmonary arterial hypertension: A population-based analysis

Carrington, M., Murphy, N.F., Strange, G., Peacock, A., McMurray, J.J. and Stewart, S. (2008) Prognostic impact of pulmonary arterial hypertension: A population-based analysis. International Journal of Cardiology, 124(2), pp. 183-187. (doi: 10.1016/j.ijcard.2006.12.045)

Abstract

Background: Although pulmonary arterial hypertension (PAH) is widely accepted as deadly, if not a rare disease, its prognostic impact beyond reports from specialist centres is unknown. Methods: Using the unique Scottish Morbidity Record Scheme and linked survival data, we tracked the survival of all Scottish adults aged :5; 65 years admitted for the first time during the period of 1986 to 2001 with a probable diagnosis of Idiopathic PAH and a PAH related to connective tissue disorders (Connective PAM and congenital abnormalities (Congenital PAM - the three most common forms of PAH. Results: Overall, 374 Scottish men and women were discharged from the hospital with incident PAH during the period 1986 to 2001. On an unadjusted basis, Congenital PAH (40-45%) was associated with the lowest case fatality at 5 years in both men and women. In both sexes, Idiopathic PAH and Connective PAH were associated with high initial one-year case fatality (20-30%) with a steady accumulation of fatal events in the four years thereafter (60-75% case fatality at 5 years). Overall, the adjusted risk of dying within one year in the period 1986 to 1989 was 2.22-fold greater (OR 95% CI, 1.27 to 3.85) than in 1998 to 2001 (P<0.001). The greatest falls in one year case fatality were seen in those with Connective PAH (I 8-fold increased risk of dying in 1986 to 1989 versus 1998 to 2001: P=0.013). Similarly, women (adjusted OR 1.38, 95% CI 1.16 to 1.63: P<0.001) and the most deprived individuals (OR 2.38, 95% CI 1.17 to 4.82: P<0.05) were at greater risk of dying within 5 years. Alternatively,- those patients discharged in 1997 were less likely to die during this period compared to their 1986 counterparts, although this difference did not quite reach statistical significance (OR 0.45, 95% CI 0.22 to 1.06: P=0.056). Conclusion: This population-based study has confirmed the deadly impact of the three most common forms of PAH. Overall, there are encouraging trends in relation to one and five year adjusted survival rates; particularly in relation to PAH related to connective tissue disorders.

• Item Type: Articles
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Peacock, Professor Andrew and McMurray, Professor John
• Authors: Carrington, M., Murphy, N.F., Strange, G., Peacock, A., McMurray, J.J., and Stewart, S.
• College/School: College of Medical Veterinary and Life Sciences; College of Medical Veterinary and Life Sciences > School of Cardiovascular & Metabolic Health
• Journal Name: International Journal of Cardiology
• Publisher: Elsevier
• ISSN: 0167-5273