title: Updated evidence-based treatment algorithm in pulmonary arterial hypertension.
abstract: Uncontrolled and controlled clinical trials with different compounds and procedures are reviewed to define the risk-benefit profiles for therapeutic options in pulmonary arterial hypertension (PAH). A grading system for the level of evidence of treatments based on the controlled clinical trials performed with each compound is used to propose an evidence-based treatment algorithm. The algorithm includes drugs approved by regulatory agencies for the treatment of PAH and/or drugs available for other indications. The different treatments have been evaluated mainly in idiopathic PAH, heritable PAH, and in PAH associated with the scleroderma spectrum of diseases or with anorexigen use. Extrapolation of these recommendations to other PAH subgroups should be done with caution. Oral anticoagulation is proposed for most patients; diuretic treatment and supplemental oxygen are indicated in cases of fluid retention and hypoxemia, respectively. High doses of calcium-channel blockers are indicated only in the minority of patients who respond to acute vasoreactivity testing. Nonresponders to acute vasoreactivity testing or responders who remain in World Health Organization (WHO) functional class III, should be considered candidates for treatment with either an oral phosphodiesterase-5 inhibitor or an oral endothelin-receptor antagonist. Continuous intravenous administration of epoprostenol remains the treatment of choice in WHO functional class IV patients. Combination therapy is recommended for patients treated with PAH monotherapy who remain in WHO functional class III. Atrial septostomy and lung transplantation are indicated for refractory patients or where medical treatment is unavailable.
authors: Columbia University, New York, New York. Electronic address: robyn.barst@gmail.com.
authors: Robyn J
authors: RJ
authors: Barst
authors: Department of Clinical Cardiology, National Heart and Lung Institute, Imperial College London, and Department of Cardiology, Hammersmith Hospital, London, United Kingdom.
authors: J Simon R
authors: JSR
authors: Gibbs
authors: Division of Pulmonary Hypertension, Department of Medicine, University Hospital Giessen and Marburg GmbH, Giessen, Germany.
authors: Hossein A
authors: HA
authors: Ghofrani
authors: Department of Respiratory Medicine, University of Hannover Medical School, Hannover, Germany.
authors: Marius M
authors: MM
authors: Hoeper
authors: Department of Internal Medicine, Division of Cardiovascular Disease, University of Michigan Health System, Ann Arbor, Michigan.
authors: Vallerie V
authors: VV
authors: McLaughlin
authors: Pulmonary Hypertension Program, University of California San Diego Medical Center, La Jolla, California.
authors: Lewis J
authors: LJ
authors: Rubin
authors: Centre des Maladies Vasculaires et Pulmonaires, Hôpital Antoine Béclère, Clamart, France.
authors: Olivier
authors: O
authors: Sitbon
authors: Division of Pulmonary and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina.
authors: Victor F
authors: VF
authors: Tapson
authors: Pulmonary Hypertension Center, Institute of Cardiology, University of Bologna, Bologna, Italy.
authors: Nazzareno
authors: N
authors: Galiè
mesh_terms: D000465:Algorithms; D000959:Antihypertensive Agents / Q000008:administration & dosage; D002121:Calcium Channel Blockers / Q000008:administration & dosage; D011464:Epoprostenol / Q000008:administration & dosage; D019317:Evidence-Based Medicine; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D007262:Infusions, Intravenous; D016032:Randomized Controlled Trials as Topic; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D011464:Epoprostenol