• Marius M Hoeper, Vallerie V McLaughlin, Abdullah M Al Dalaan, Toru Satoh, and Nazzareno Galiè.
• Department of Respiratory Medicine, Hannover Medical School and German Centre for Lung Research, Hannover, Germany. Electronic address: hoeper.marius@mh-hannover.de.
• Lancet Respir Med. 2016 Apr 1; 4 (4): 323-36.

AbstractThe 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.Copyright © 2016 Elsevier Ltd. All rights reserved.

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