A review of therapeutic agents for the management of pulmonary arterial hypertension.
Pulmonary arterial hypertension (PAH) is an uncommon, progressive and life threatening disease characterized by a proliferative vasculopathy of the small muscular pulmonary arterioles resulting in elevated pulmonary vascular resistance and eventually right ventricular failure. An increasing understanding of the pathobiology of PAH and its natural history has led to the development of numerous targeted therapies. Despite these advances there is significant progression of disease and the survival rate remains low. This article reviews the agents currently available for the medical management of PAH.
Northwell Health Division of Pulmonary Critical Care and Sleep Medicine, 410 Lakeville Road, Suite 107, New Hyde Park, NY 11042, USA.
Stella S
SS
Hahn
Northwell Health Division of Pulmonary Critical Care and Sleep Medicine, 410 Lakeville Road, Suite 107, New Hyde Park, NY 11042, USA.
Mina
M
Makaryus
Northwell Health Division of Pulmonary Critical Care and Sleep Medicine, 410 Lakeville Road, Suite 107, New Hyde Park, NY 11042, USA.
Arunabh
A
Talwar
Northwell Health Division of Pulmonary Critical Care and Sleep Medicine, 410 Lakeville Road, Suite 107, New Hyde Park, NY 11042, USA.
Mangala
M
Narasimhan
Northwell Health Division of Pulmonary Critical Care and Sleep Medicine, 410 Lakeville Road, Suite 107, New Hyde Park, NY 11042, USA.
Gulrukh
G
Zaidi
D018450:Disease Progression; D015195:Drug Design; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D058990:Molecular Targeted Therapy; D015996:Survival Rate; D014655:Vascular Resistance; D018497:Ventricular Dysfunction, Right / Q000209:etiology
endothelin receptor antagonist; phosphodiesterase inhibitor; prostanoid; pulmonary arterial hypertension; soluble guanylate cyclase stimulator