Evidence-based treatment algorithm, including clinical trials published through 2009. A, B, and C are levels of evidence defined as follows—Level of Evidence:

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Evidence-based treatment algorithm, including clinical trials published through 2009. A, B, and C are levels of evidence defined as follows—Level of Evidence: A, data derived from multiple randomized clinical trials or meta-analyses; Level of Evidence: B, data derived from a single randomized clinical trial or from multiple randomized clinical trials with heterogeneous results; Level of Evidence: C, data derived from small nonrandomized studies and/or consensus opinion of experts. inh, inhaled; IV, continuous intravenous. (1) Owing to the complexity and dangers of the acute vasoreactivity tests and to the treatment options available, it is strongly recommended that consideration be given to referral of patients with pulmonary arterial hypertension (PAH) to a specialized center. (2) The acute vasoreactivity test should be performed in all patients with PAH even if the greater incidence of positive response is achieved in individuals with idiopathic PAH and PAH associated to anorexigen use. (3) A positive acute response to vasodilators is defined as a drop in mean pulmonary arterial hypertension of at least 10 mm Hg to ≤40 mm Hg, with an increase or unchanged cardiac output during acute challenge with inhaled nitric oxide (NO) or IV epoprostenol. (4) Sustained response to calcium channel blockers (CCBs) is defined as patients being in WHO functional class I or II with near-normal hemodynamics after several months of treatment. (5) In patients in WHO functional class III, first-line therapy may include oral endothelin receptor antagonists, phosphodiesterase type 5 (PDE5) inhibitors, or prostacyclin analogues. (6) Most experts consider that WHO functional class IV patients in unstable condition should be treated with chronic IV prostacyclin analogues. From Barst et al.34 Source: Chapter 71. Pulmonary Hypertension, Hurst's The Heart, 13e Citation: Fuster V, Walsh RA, Harrington RA. Hurst's The Heart, 13e; 2011 Available at: http://accessmedicine.mhmedical.com/DownloadImage.aspx?image=/data/books/hurs13/hurs13_c071f010.gif&sec=40292327&BookID=376&ChapterSecID=40279804&imagename= Accessed: October 31, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved