title: Medical therapies for pulmonary arterial hypertension.
abstract: Pulmonary Arterial hypertension (PAH) is a chronic and progressive disease characterized by an increase in pulmonary vascular resistance due to severe remodeling of the small pulmonary arteries. In PAH, the endothelial cells fail to maintain their homeostatic balance, with the consequent impaired production of vasodilators and over-expression of vasoconstrictors and proliferators. Current treatment of PAH is based on the discovery of three main pathways of endothelial dysfunction (prostacyclin, nitric oxide and endothelin-1), and includes drugs such as prostacyclin analogs, phosphodiesterase-5 inhibitors and endothelin receptor antagonists (ERAs). Recently approved drugs that act through these classic pathways include riociguat (cyclic GMP stimulator) and macitentan (a tissue specific dual ERA). However, several new drugs and new pathways are under study. New targeted therapies include tyrosine kinase inhibitors, Rho kinase inhibitors and serotonin receptor blockers. There are now ten drugs approved for the treatment of PAH that, alone or in combination, have changed the natural history of this disease. The new drugs will allow us to further modified the patients' life expectancy and move towards a cure.
authors.affiliation: Cardiopulmonary Department, Ignacio Chavez National Heart Institute, 1 Juan Badiano, 4th Floor, 14080, Mexico City, Mexico. pulidot@me.com.
authors.forename: Tomas
authors.initials: T
authors.lastname: Pulido
authors.affiliation: Cardiopulmonary Department, Ignacio Chavez National Heart Institute, 1 Juan Badiano, 4th Floor, 14080, Mexico City, Mexico.
authors.forename: Nayeli
authors.initials: N
authors.lastname: Zayas
authors.affiliation: Cardiopulmonary Department, Ignacio Chavez National Heart Institute, 1 Juan Badiano, 4th Floor, 14080, Mexico City, Mexico.
authors.forename: Maitane Alonso
authors.initials: MA
authors.lastname: de Mendieta
authors.affiliation: Cardiopulmonary Department, Ignacio Chavez National Heart Institute, 1 Juan Badiano, 4th Floor, 14080, Mexico City, Mexico.
authors.forename: Karen
authors.initials: K
authors.lastname: Plascencia
authors.affiliation: Cardiopulmonary Department, Ignacio Chavez National Heart Institute, 1 Juan Badiano, 4th Floor, 14080, Mexico City, Mexico.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Escobar
mesh_terms: D004359:Drug Therapy, Combination; D015507:Drugs, Investigational; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D019332:Endothelin-1 / Q000378:metabolism*; D011464:Epoprostenol / Q000378:metabolism*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D009569:Nitric Oxide / Q000378:metabolism*; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use*; D016032:Randomized Controlled Trials as Topic; D013449:Sulfonamides / Q000627:therapeutic use*
chemical_list: D015507:Drugs, Investigational; D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1; D058986:Phosphodiesterase 5 Inhibitors; D011743:Pyrimidines; D013449:Sulfonamides; D009569:Nitric Oxide; D011464:Epoprostenol; C533860:macitentan
keywords: Endothelin-1; New treatments; Nitric oxide; Prostacyclin; Pulmonary arterial hypertension