Updated evidence-based treatment algorithm in pulmonary arterial hypertension.
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.
Barst
Robyn J
RJ
Columbia University, New York, New York. Electronic address: robyn.barst@gmail.com.
Gibbs
J Simon R
JSR
Department of Clinical Cardiology, National Heart and Lung Institute, Imperial College London, and Department of Cardiology, Hammersmith Hospital, London, United Kingdom.
Ghofrani
Hossein A
HA
Division of Pulmonary Hypertension, Department of Medicine, University Hospital Giessen and Marburg GmbH, Giessen, Germany.
Hoeper
Marius M
MM
Department of Respiratory Medicine, University of Hannover Medical School, Hannover, Germany.
McLaughlin
Vallerie V
VV
Department of Internal Medicine, Division of Cardiovascular Disease, University of Michigan Health System, Ann Arbor, Michigan.
Rubin
Lewis J
LJ
Pulmonary Hypertension Program, University of California San Diego Medical Center, La Jolla, California.
Sitbon
Olivier
O
Centre des Maladies Vasculaires et Pulmonaires, Hôpital Antoine Béclère, Clamart, France.
Tapson
Victor F
VF
Division of Pulmonary and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina.
Galiè
Nazzareno
N
Pulmonary Hypertension Center, Institute of Cardiology, University of Bologna, Bologna, Italy.
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
D000959:Antihypertensive Agents; D002121:Calcium Channel Blockers; D011464:Epoprostenol