[Treatments for pulmonary arterial hypertension].
OBJECTIVE
Pulmonary arterial hypertension (PAH) is characterized by vasoconstriction, in situ thrombosis and vascular remodeling of small pulmonary arteries inducing a fixed pulmonary arterial obstruction and persistent elevation of pulmonary arterial resistance. Conventional treatment is based on simple measures (exercise limitation) and non-specific drugs (warfarine, diuretics, oxygen).


BACKGROUND
Pure vasodilators like calcium channel antagonists have little or no effect on the vast majority of patients, presumably because fixed pulmonary arteriopathy predominate over vasoconstriction. Intravenous prostacyclin (epoprostenol) and endothelin receptor antagonists have vasodilator and antiproliferative properties. Epoprostenol therapy has resulted in significant improvements in prognosis of this disease and this drug remains the first-line treatment of the most severe patients. Bosentan is an interesting first-line treatment for NYHA functional class III patients. Availability of novel specific drugs (endothelin receptor type A antagonists, prostacyclin analogues, type 5 phosphodiesterase inhibitors) open new perspectives in treatment of PAH. The long-term benefit of these drugs remains to be evaluated and their respective place in treatment of these patients is still uncertain. We here present the different therapeutic alternatives available in the PAH and propose an algorithm for treatment of these patients.


CONCLUSIONS
The evolution of therapy from vasodilators to antiproliferative agents reflects the advancement in our understanding of the mechanisms mediating pulmonary arterial hypertension.
Centre des maladies vasculaires pulmonaires, UPRES EA2705, service de pneumologie et réanimation respiratoire, hôpital Antoine-Béclère, université Paris-Sud, Assistance publique-Hôpitaux de Paris, 157, rue de la Porte-de-Trivaux, 92140 Clamart, France.
D
D
Montani
X
X
Jaïs
V
V
Ioos
O
O
Sitbon
G
G
Simonneau
M
M
Humbert
D000465:Algorithms; D000925:Anticoagulants / Q000627:therapeutic use; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000077300:Bosentan; D002121:Calcium Channel Blockers / Q000008:administration & dosage / Q000627:therapeutic use; D004232:Diuretics / Q000627:therapeutic use; D065128:Endothelin Receptor Antagonists; D005544:Forecasting; D006346:Heart Septum / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology / Q000601:surgery / Q000628:therapy*; D007275:Injections, Intravenous; D016040:Lung Transplantation; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D011446:Prospective Studies; D044062:Prostaglandins I / Q000008:administration & dosage / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D012189:Retrospective Studies; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use
D000925:Anticoagulants; D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D004232:Diuretics; D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D044062:Prostaglandins I; D013449:Sulfonamides; D000077300:Bosentan