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Pulmonary Hypertension - Scottish Pulmonary Vascular Unit - Health Care Professional Section











Untreated IPAH has a poor prognosis. In the American prospective study the median survival was 2.8 years (1). Patients with PAH associated with connective tissue disease have a worse prognosis whereas those with congenital heart disease fare better.


Disease-targeted therapies for PAH have been shown to improve survival and many prognostic indicators, and there are now numerous studies demonstrating this for prostanoids, endothelin receptor antagonists, phosphodiesterase inhibitors and soluble guanylate cyclase stimulators, one of the new classes of drugs becoming available  (2-21). 


Part of the investigation process for patients with PAH involves an assessment of their individual prognostic factors. These include WHO functional class, six minute walk distance, cardiac echo appearances, haemodynamics at right heart catheterization (especially right atrial pressure and cardiac index), NTproBNP levels and cardiopulmonary exercise test variables.


1. Rich S et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987; 107:216-223.

2. Barst RJ, Rubin LJ, Long WA, McGoon MD, Rich S, Badesch DB, et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. The New England journal of medicine. 1996;334(5):296-302.

3. Barst RJ, Rubin LJ, McGoon MD, Caldwell EJ, Long WA, Levy PS. Survival in primary pulmonary hypertension with long-term continuous intravenous prostacyclin. Annals of internal medicine. 1994 Sep 15;121(6):409-15.

4. Higenbottam T, Butt AY, McMahon A, Westerbeck R, Sharples L. Long-term intravenous prostaglandin (epoprostenol or iloprost) for treatment of severe pulmonary hypertension. Heart (British Cardiac Society). 1998 Aug;80(2):151-5.

5. Sitbon O, Humbert M, Nunes H, Parent F, Garcia G, Herve P, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. Journal of the American College of Cardiology. 2002 Aug 21;40(4):780-8.

6. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation. 2002 Sep 17;106(12):1477-82.

7. Lang I, Gomez-Sanchez M, Kneussl M, Naeije R, Escribano P, Skoro-Sajer N, et al. Efficacy of long-term subcutaneous treprostinil sodium therapy in pulmonary hypertension. Chest. 2006 Jun;129(6):1636-43.

8. Barst RJ, Galie N, Naeije R, Simonneau G, Jeffs R, Arneson C, et al. Long-term outcome in pulmonary arterial hypertension patients treated with treprostinil. Eur Respir J. 2006 Aug 9.

9. Opitz CF, Wensel R, Winkler J, Halank M, Bruch L, Kleber FX, et al. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. European heart journal. 2005 Sep;26(18):1895-902.

10. R. J. Barst, N. Galie, R. Naeije, G. Simonneau, R. Jeffs, C. Arneson, and L. J. Rubin. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J 2006 28: 1195-1203.

11. Denton, C.P., et al., Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis, 2006. 65(10): p. 1336-40.

12. McLaughlin, V.V., et al., Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J, 2005. 25(2): p. 244-9.

13. Provencher, S., et al., Long-term outcome with first-line bosentan therapy in idiopathic pulmonary arterial hypertension. Eur Heart J, 2006. 27(5): p. 589-95.

14. Hoeper, M.M., et al., Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J, 2005. 26(5): p. 858-63.

15. M H Williams, C Das, C E Handler, M R Akram, J Davar, C P Denton, C J Smith, C M Black, J G Coghlan. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 2006;92:926?932.

16. Galie, N et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005;353:2148-2157
17. Simonneau, G et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomised trial. Ann Intern Med 2008;149:521-530
18. Galie, N et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009;119:2894-2903
19. Ghofrani, H et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013; 369:330-340
20. Ghofrani, H et al. Riociguat for the treatment of chronic thromboembolic pulmonary hypertension. N Engl J Med 2013;369:319-329
21. Pulido, T et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013;369:809-818



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