title: Treatment of pulmonary hypertension.
abstract: The most common forms of pulmonary hypertension are pulmonary arterial hypertension, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension due to left-sided heart and lung disease. The treatment of pulmonary arterial hypertension has advanced substantially over the past 20 years. Five different classes of drugs are now available-ie, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and prostacyclin receptor agonists. Long-term studies have provided evidence that various combinations of these compounds improve the progression-free survival of patients with pulmonary arterial hypertension. For patients with chronic thromboembolic pulmonary hypertension, surgical pulmonary endarterectomy is the treatment of choice. For patients who are inoperable and have chronic thromboembolic pulmonary hypertension, riociguat, a stimulator of soluble guanylate cyclase, has proven efficacious. Additionally, interventional approaches could become a treatment option for these patients. For patients with pulmonary hypertension due to left-sided heart disease or lung disease, the use of pulmonary vasodilator treatment has not been proven to be safe and effective.
authors.affiliation: Department of Respiratory Medicine, Hannover Medical School and German Centre for Lung Research, Hannover, Germany. Electronic address: hoeper.marius@mh-hannover.de.
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.affiliation: Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI, USA.
authors.forename: Vallerie V
authors.initials: VV
authors.lastname: McLaughlin
authors.affiliation: Section of Pulmonary Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors.forename: Abdullah M Al
authors.initials: AM
authors.lastname: Dalaan
authors.affiliation: Division of Cardiology, Department of Medicine, Kyorin University School of Medicine, Tokyo, Japan.
authors.forename: Toru
authors.initials: T
authors.lastname: Satoh
authors.affiliation: Department of Experimental, Diagnostic and Specialty Medicine (DIMES), University of Bologna, Bologna, Italy.
authors.forename: Nazzareno
authors.initials: N
authors.lastname: Galiè
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D018572:Disease-Free Survival; D065128:Endothelin Receptor Antagonists / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008171:Lung Diseases / Q000150:complications; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use; D044062:Prostaglandins I / Q000627:therapeutic use; D044006:Receptors, Epoprostenol / Q000819:agonists; D000071756:Soluble Guanylyl Cyclase / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors; D044062:Prostaglandins I; D044006:Receptors, Epoprostenol; D014665:Vasodilator Agents; D000071756:Soluble Guanylyl Cyclase