Medical therapies for pulmonary arterial hypertension

Heart Failure Reviews
Tomas PulidoJennifer Escobar

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.

References

Feb 1, 1996·The New England Journal of Medicine·R J BarstUNKNOWN Primary Pulmonary Hypertension Study Group
Oct 16, 1999·Journal of the American College of Cardiology·S Rich, V V McLaughlin
Mar 19, 2002·American Journal of Respiratory and Critical Care Medicine·Gerald SimonneauUNKNOWN Treprostinil Study Group
Mar 22, 2002·The New England Journal of Medicine·Lewis J RubinGerald Simonneau
May 3, 2002·Journal of the American College of Cardiology·Nazzareno GalièUNKNOWN Arterial Pulmonary Hypertension and Beraprost European (ALPHABET) Study Group
Aug 2, 2002·The New England Journal of Medicine·Horst OlschewskiUNKNOWN Aerosolized Iloprost Randomized Study Group
Sep 3, 2002·Journal of the American College of Cardiology·Olivier SitbonGérald Simonneau
Sep 18, 2002·Circulation·Vallerie V McLaughlinStuart Rich
Jun 25, 2003·Journal of the American College of Cardiology·Robyn J BarstUNKNOWN Beraprost Study Group
Aug 23, 2003·Circulation Research·Sergei D RybalkinJoseph A Beavo
Mar 3, 2004·Journal of Comparative Physiology. A, Neuroethology, Sensory, Neural, and Behavioral Physiology·D L McLean, K T Sillar
Oct 2, 2004·The New England Journal of Medicine·Marc HumbertGérald Simonneau
Feb 3, 2005·The European Respiratory Journal·V V McLaughlinL J Rubin
Aug 2, 2005·Journal of the American College of Cardiology·Nazzareno GaliéLewis J Rubin
Oct 4, 2005·The Journal of Clinical Investigation·Ralph Theo SchermulyFriedrich Grimminger
Nov 18, 2005·The New England Journal of Medicine·Nazzareno GalièUNKNOWN Sildenafil Use in Pulmonary Arterial Hypertension (SUPER) Study Group
May 29, 2008·Circulation·Nazzareno GalièUNKNOWN Ambrisentan in Pulmonary Arterial Hypertension, Randomized, Double-Blind, Placebo-Controlled, Multicenter, Efficacy Studies
Sep 11, 2008·The Journal of Pharmacology and Experimental Therapeutics·Marc IglarzMartine Clozel
Jan 9, 2009·The European Respiratory Journal·F GrimmingerH A Ghofrani
May 28, 2009·Circulation·Nazzareno GalièUNKNOWN Pulmonary Arterial Hypertension and Response to Tadalafil (PHIRST) Study Group
Dec 17, 2009·Clinical Pharmacology and Therapeutics·M Gomberg-MaitlandM J Ratain
Jan 26, 2010·Expert Opinion on Emerging Drugs·Reda E Girgis
Mar 31, 2010·The European Respiratory Journal·K T B MouchaersW J van der Laarse
May 1, 2010·Journal of the American College of Cardiology·Vallerie V McLaughlinWerner Seeger
Jun 29, 2010·American Journal of Respiratory and Critical Care Medicine·Hossein A GhofraniSteve Pascoe
Oct 16, 2010·The European Respiratory Journal·R DumitrascuS S Pullamsetti
Mar 1, 2012·The European Respiratory Journal·Gérald SimonneauIrene M Lang
Mar 28, 2012·Circulation·David MontaniMarc Humbert
May 9, 2012·Drug Design, Development and Therapy·Olivier LambertNagesh Palepu
May 18, 2012·Canadian Journal of Physiology and Pharmacology·Edward A PankeyPhilip J Kadowitz
Dec 4, 2012·European Respiratory Review : an Official Journal of the European Respiratory Society·Olivier Sitbon, Nicholasw Morrell

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Citations

Oct 25, 2016·Biomedicine & Pharmacotherapy = Biomédecine & Pharmacothérapie·Haiying ChenLexin Wang
Dec 19, 2016·American Journal of Cardiovascular Drugs : Drugs, Devices, and Other Interventions·Sean T DugganCeleste B Burness

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