title: Advances in diagnosis and treatment in patients with pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) is a progressive disease marked by vasoconstriction and vascular remodeling within pulmonary arteries leading to right heart failure and death. Significant advances in understanding the pathobiology of the disease have identified three key pathways involved in progression of this disease, which are the endothelin pathway, the prostacyclin pathway, and the nitric oxide/cyclic guanosine monophosphate pathway. Echocardiogram is the best screening tool to obtain an estimation of the pulmonary artery systolic pressure but right heart catheterization remains the standard by which the diagnosis is made. There are currently six FDA approved therapies for PAH. The mechanistic rationale, evidence behind their use and side effect considerations in utilizing these therapies in PAH patients will be the focus of this review.
authors.affiliation: Division of Cardiology, Director of Pulmonary Vascular Disease Program, University of Maryland School of Medicine, Baltimore, Maryland 21201, USA. mpark@medicine.umaryland.edu
authors.forename: Myung H
authors.initials: MH
authors.lastname: Park
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D006328:Cardiac Catheterization; D018450:Disease Progression; D004359:Drug Therapy, Combination; D065128:Endothelin Receptor Antagonists; D004730:Endothelium, Vascular / Q000503:physiopathology; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D016285:Iloprost / Q000627:therapeutic use; D007555:Isoxazoles / Q000494:pharmacology; D010726:Phosphodiesterase Inhibitors / Q000494:pharmacology; D010879:Piperazines / Q000494:pharmacology; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011379:Prognosis; D011651:Pulmonary Artery / Q000503:physiopathology; D011687:Purines / Q000494:pharmacology; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000494:pharmacology; D013876:Thiophenes / Q000494:pharmacology; D014661:Vasoconstriction / Q000502:physiology
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D007555:Isoxazoles; D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D010975:Platelet Aggregation Inhibitors; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D013876:Thiophenes; D000068677:Sildenafil Citrate; D011464:Epoprostenol; C106276:sitaxsentan; D016285:Iloprost; D000077300:Bosentan; C427248:treprostinil