Medicamentous treatment of pulmonary hypertension in 2011

Czech version

Authors: M. Aschermann; P. Jansa
Published in: Kardiol Rev Int Med 2011, 13(1): 37-40
Category: From Hypertension to Heart Failure

Overview

Pulmonary hypertension is a primary illness of the pulmonary arterioles that leads to the gradual worsening of precapillary pulmonary hypertension and, if untreated, to the relatively rapid failure of the right heart ventricle and the death of the patient. Pulmonary hypertension occurs either due to unknown causes, or is associated with a known cause (systemic disease of the connective tissue, liver disease, congenital heart defects, HIV infections, anorexia). Current medicamentous treatment of pulmonary hypertension is divided into two categories: conventional (anti-coagulant treatment, calcium channel blockers, treatment for heart failure) and specific (prostanoids, antagonists of endothelin receptors, phosphodiesterases 5 inhibitors). Patients with a positive result in vasodilatory tests were marked for treatment with high doses of calcium channel blockers. Patients with a negative result in vasodilatory tests were marked for chronic anti-coagulant treatment and specific pharmacotherapy, employed as either monotherapy or combined treatment. New treatment options for pulmonary hypertension, which are currently under experimental study, include a number of substances: agonists for prostacyclin receptors, soluble guanylate cyclase activators and simulators, statins, antagonists for seratonin receptors and seratonin transporter blockers, Rho-associated protein kinase inhibitors, vasoactive intestinal peptides and tyrosine kinase inhibitors.

Keywords:
pulmonary hypertension – conventional and specific treatment – prostanoids – antagonists of endothelin receptors – antagonists for endotheline receptors – phosphodiesterases 5 inhibitors – experimental treatment options

Sources

1. Simonneau G, Robbins IM, Beghetti M et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009; 54: S43–S54.

2. Sitbon O, Humbert M, Jaïs X et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105–3111.

3. 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: 296–301.

4. Kurzyna M, Dabrowski M, Bielicki D et al. Atrila septostomy in treatment of end stage right heart failure in patients with pulmonary hypertension. Chest 2007; 131: 977–983.

5. de Perrot M, Chaparro C, McRae K et al. Twenty-year experience of lung transplantation at a single center: influence of recipient diagnosis on long-term survival. J Thorac Cardiovasc Surg 2004; 127: 1493–1501.

6. McGoon MD, Kane GC. Pulmonary hypertension: diagnosis and management. Mayo Clin Proc 2009; 84: 191–207.

7. Barst RJ, Gibbs JS, Ghofrani HA et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2009; 54: S78–S84.

8. Barst RJ, Galie N, Naeije R et al. Long-term outcome in pulmonary arterial hypertension patients treated with subcutaneous treprostinil. Eur Respir J 2006; 28: 1195–1203.

9. Opitz CF, Wensel R, Winkler J et al. Clinical efficacy and survival with first-line inhaled iloprost therapy in patients with idiopathic pulmonary arterial hypertension. Eur Heart J 2005; 26: 1895–1902.

10. Barst RJ, McGoon MD, McLaughlin VV et al. Beraprost Study Group. Beraprost therapy for pulmonary arterial hypertension. J Am Coll Cardiol 2003; 41: 2119–2125.

11. Nishida M, Eshiro K, Okada Y et al. Roles of endothelin ETA and ETB receptors in the pathogenesis of monocrotaline-induced pulmonary hypertension. J Cardiovasc Pharmacol 2004; 44: 187–191.

12. Rubin LJ, Badesch DB, Barst RJ et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002; 346: 896–903.

13. Barst RJ, Langleben D, Badesch D et al. STRIDE-2 Study Group. Treatment of pulmonary arterial hypertension with the selective endothelin-A receptor antagonist sitaxsentan. J Am Coll Cardiol 2006; 47: 2049–2056.

14. Galiè N, Olschewski H, Oudiz RJ et al. Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies (ARIES) Group. 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.

15. Iglarz M, Binkert C, Morrison K et al. Pharmacology of macitentan, an orally active tissue-targeting dual endothelin receptor antagonist. J Pharmacol Exp Ther 2008; 327: 736–745.

16. Galiè N, Ghofrani HA, Torbicki A et al. Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005; 353: 2148–2157.

17. Galiè N, Brundage BH, Ghofrani HA et al. Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009; 119: 2894–2903.

18. Simonneau G, Rubin LJ, Galiè N et al. PACES Study Group. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149: 521–530.

19. Leuchte HH, Baezner C, Baumgartner RA et al. Inhalation of vasoactive intestinal peptide in pulmonary hypertension. Eur Respir J 2008; 32: 1289–1294.

20. Grimminger F, Weimann G, Frey R et al. First acute haemodynamic study of soluble guanylate cyclase stimulator riociguat in pulmonary hypertension. Eur Respir J 2009; 33: 785–792.

21. Souza R, Sitbon O, Parent F et al. Long term imatinib treatment in pulmonary arterial hypertension. Thorax 2006; 61: 736.