Contemporary treatment of pulmonary arterial hypertension: the North-West Registry data analysis
https://doi.org/10.15829/1728-8800-2012-4-79-84
Abstract
Aim. Using the prospective Registry data, to assess the effects of conventional and specific therapy on the clinical course and survival of the patients with pulmonary arterial hypertension (PAH).
Material and methods. The study included 124 patients (mean age 38,2±13,7 years; 34 men and 78 women): 31 with idiopathic PAH (IPAH), 52 with Eisenmenger syndrome, 17 with inoperable chronic thromboembolic pulmonary hypertension, 9 with PAH and corrected congenital heart disease, 6 with PAH and systemic scleroderma, and 6 with PAH and HIV infection.
Results. The cumulative one-year and three-year survival rates were 94% and 75%, respectively. Irrespective of the absence of right heart catheterisation and vasoreactive testing, 42,7% of the patients were treated with calcium antagonists. PAH-specific therapy was administered to 40,3% of the participants (64,5% and 21% of those with IPAH and Eisenmenger syndrome, respectively). PAH-specific therapy was associated with an increase in survival time.
Conclusion. In PAH patients, the prognosis is linked to early administration of specific monotherapy and possible combination therapy. Developing a national registry of pulmonary hypertension will facilitate the assessment of the real-world demand for specific therapy and the related costs.
About the Authors
N. S. GoncharovaRussian Federation
A. V. Kazymly
Russian Federation
A. V. Naimushin
Russian Federation
O. M. Moiseeva
Russian Federation
References
1. Galie`N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J 2009; 30(20): 2493-537.
2. Tuder RM, Abman SH, Braun T, et al. Development and Pathology of Pulmonary Hypertension. JACC 2009; 54 (1): S3-9.
3. Barst RJ, Gibbs JSR, Ghofrani HA, et al. Updated Evidence-Based Treatment Algorithm in Pulmonary Arterial Hypertension. JACC 2009; 54(1): S78-84.
4. Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting Survival in Pulmonary Arterial Hypertension: Insights From the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 2010; 122(2): 164-72.
5. Humbert M, Sitbon O, Yaéci A, et al. Survival in Patients With Idiopathic, Familial, and Anorexigen-Associated Pulmonary Arterial Hypertension in the Modern Management Era. Eur Respir J 2010; 36(3): 549-55.
6. D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Ann Intern Med 1991; 115(5): 343-9.
7. Peacock AJ, Murphy NF, McMurray JJV, et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30(1): 1390-5.
8. Sitbon O, Humbert M, Jaés X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111(23): 3105-11.
9. Galie N, Manes A, Palazzini M, et al. Management of Pulmonary Arterial Hypertesnion associated with Congenital Systemic to Pulmonary Shunts and Eisenmenger Syndrome. Drugs 2008; 68 (8): 1049-66.
10. Johnson SR, Mehta S, Granton JT. Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Eur Respir J 2006; 28(5): 999-1004.
11. Galie N, Manes A, Negro L, et al. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009; 30 (4): 394- 403.
12. Galiè N, Rubin L, Hoeper M, et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomized controlled trial. Lancet 2008; 371(9630): 2093-100.
13. Hoeper MM, Markevych I, Spiekerkoetter E, et al. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26(5): 858-63.
14. Sitbon O, Galie N. Treat-to-target strategies in pulmonary arterial hypertension: the importance of using multiple goals. Eur Respir Rev 2010; 19(118): 272-8.
Review
For citations:
Goncharova N.S., Kazymly A.V., Naimushin A.V., Moiseeva O.M. Contemporary treatment of pulmonary arterial hypertension: the North-West Registry data analysis. Cardiovascular Therapy and Prevention. 2012;11(4):79-84. (In Russ.) https://doi.org/10.15829/1728-8800-2012-4-79-84