title: Pulmonary arterial hypertension: evaluation and management.
abstract: Pulmonary arterial hypertension (PAH), a rare disease involving the pulmonary vascular circuit, is defined as an elevation in pulmonary arterial pressures and is characterized by symptoms of dyspnea, chest pain, and syncope. If left untreated, the disease carries a high mortality rate, with the most common cause of death being decompensated right heart failure. Over the past 5 years, there have been significant advances in this field in regards to understanding the pathogenesis, diagnosis, and classification of PAH. The availability of newer drugs has resulted in a radical change in the management of this disease with significant improvement in both quality of life and mortality. Ongoing research promises to lead to a more comprehensive understanding of the genetics, etiology, and pathogenesis of pulmonary arterial hypertension, which may ultimately translate into more effective therapeutic options.
authors: Pulmonary Critical Care Unit and the Critical Care Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.
authors: Anne V
authors: AV
authors: LaRaia
authors: Aaron B
authors: AB
authors: Waxman
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D002121:Calcium Channel Blockers / Q000627:therapeutic use*; D006348:Cardiac Surgical Procedures / Q000379:methods*; D018450:Disease Progression; D004359:Drug Therapy, Combination; D065130:Endothelin A Receptor Antagonists; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use*; D011453:Prostaglandins / Q000627:therapeutic use*; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D065130:Endothelin A Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins