title: Pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) is a rare and debilitating disease characterized by abnormal proliferation and contraction of pulmonary vascular smooth muscle cells. The resulting increase in pressure and pulmonary vascular resistance results in progressive right heart failure, low cardiac output, and ultimately death if left untreated. PAH is defined by a persistent elevation in pulmonary artery pressure with normal left-sided pressures, differentiating it from left-sided heart disease. Symptoms progress from shortness of breath and decreasing exercise tolerance to right heart failure, with peripheral edema and marked functional limitation. Exercise-induced syncope, worsening symptoms at rest, and intractable right heart failure indicate critical disease. PAH may be idiopathic with no identifiable cause or associated with collagen vascular diseases, drugs, HIV, liver disease, and/or congenital heart disease. Familial or genetically mediated PAH accounts for a small percentage of cases. Advances in the understanding of pathobiological pathways that contribute to vascular proliferation and remodeling have resulted in new therapies that improve quality of life and survival. Emerging therapies focus on the nitric oxide, prostacyclin, and endothelin pathways. Nursing interventions are critical to ensure patients' success with these expensive and complex treatments and their optimal adjustment to living with PAH.
authors.affiliation: VA Greater LA Healthcare System, University of California, Los Angeles, CA 90073, USA. glenna.traiger@va.gov
authors.forename: Glenna L
authors.initials: GL
authors.lastname: Traiger
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D006328:Cardiac Catheterization; D003422:Critical Care / Q000379:methods*; D004232:Diuretics / Q000627:therapeutic use; D017548:Echocardiography, Transesophageal; D065130:Endothelin A Receptor Antagonists; D045462:Endothelium-Dependent Relaxing Factors / Q000627:therapeutic use; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology / Q000628:therapy*; D007260:Infusion Pumps; D020407:Internet; D008403:Mass Screening; D008487:Medical History Taking; D009569:Nitric Oxide / Q000627:therapeutic use; D010102:Oxygen Inhalation Therapy; D035583:Rare Diseases; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D004232:Diuretics; D065130:Endothelin A Receptor Antagonists; D045462:Endothelium-Dependent Relaxing Factors; D014665:Vasodilator Agents; D009569:Nitric Oxide