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STATE-OF-THE-ART PAPER

Updated Evidence-Based Treatment Algorithm in Pulmonary Arterial Hypertension

Robyn J. Barst, MD^_*,_*, J. Simon R. Gibbs, MD, Hossein A. Ghofrani, MD, Marius M. Hoeper, MD, Vallerie V. McLaughlin, MD^||, Lewis J. Rubin, MD^¶, Olivier Sitbon, MD^#, Victor F. Tapson, MD^_** and Nazzareno Galiè, MD

^_* Columbia University, New York, New York
Department of Clinical Cardiology, National Heart and Lung Institute, Imperial College London, and Department of Cardiology, Hammersmith Hospital, London, United Kingdom
Division of Pulmonary Hypertension, Department of Medicine, University Hospital Giessen and Marburg GmbH, Giessen, Germany
Department of Respiratory Medicine, University of Hannover Medical School, Hannover, Germany
^|| Department of Internal Medicine, Division of Cardiovascular Disease, University of Michigan Health System, Ann Arbor, Michigan
^¶ Pulmonary Hypertension Program, University of California San Diego Medical Center, La Jolla, California
^# Centre des Maladies Vasculaires et Pulmonaires, Hôpital Antoine Béclère, Clamart, France
^_** Division of Pulmonary and Critical Care Medicine, Duke University Medical Center, Durham, North Carolina
Pulmonary Hypertension Center, Institute of Cardiology, University of Bologna, Bologna, Italy

Manuscript received February 6, 2009; accepted April 15, 2009.

^_* Reprint requests and correspondence: Dr. Robyn J. Barst, Professor Emerita of Pediatrics (in Medicine), Columbia University, 31 Murray Hill Road, Scarsdale, New York 10583 (Email: robyn.barst{at}gmail.com).

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.

Key Words: algorithm • evidence-based treatment • pulmonary arterial hypertension

Abbreviations and Acronyms   CCB = calcium-channel blocker   ERA = endothelin receptor antagonist   ETA = endothelin receptor A   ETB = endothelin receptor B   HPAH = heritable pulmonary arterial hypertension   INR = international normalized ratio   IPAH = idiopathic pulmonary arterial hypertension   IV = intravenous   PAH = pulmonary arterial hypertension   PDE = phosphodiesterase   PH = pulmonary hypertension   RCT = randomized controlled trial   WHO = World Health Organization

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