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J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D60-72. doi: 10.1016/j.jacc.2013.10.031.

Updated treatment algorithm of pulmonary arterial hypertension.

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Department of Experimental, Diagnostic and Specialty Medicine (DIMES), Bologna University Hospital, Bologna, Italy. Electronic address:
Institute of Cellular Medicine Newcastle University and The Newcastle Hospitals NHS Foundation Trust, Newcastle, United Kingdom.
Baylor College of Medicine, Houston, Texas.
Michigan State University, College of Human Medicine, Grand Rapids, Michigan.
Division of Respirology, University of Toronto, Toronto, Canada.
Fu Wai Hospital & National Center for Cardiovascular Disease Peking Union Medical College and Chinese Academy of Medical Science, Beijing, China.
Department of Thoracic Surgery, Medical University Vienna/Vienna General Hospital, Vienna, Austria.
Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
Cardiovascular Medicine, The University of Michigan, Ann Arbor, Michigan.
Pulmonary, Critical Care and Sleep Division, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts.
Medical School, University of California, San Diego, La Jolla, California.
Clinical Research, National Institute of Cardiology of Mexico, Mexico City, Mexico.
Max Planck Institute for Heart and Lung Research, Universities of Giessen and Marburg Lung Center, Giessen/Bad Nauheim, Germany.
Heart Transplant Unit, St Vincent's Hospital, Sydney, Australia.


The demands on a pulmonary arterial hypertension (PAH) treatment algorithm are multiple and in some ways conflicting. The treatment algorithm usually includes different types of recommendations with varying degrees of scientific evidence. In addition, the algorithm is required to be comprehensive but not too complex, informative yet simple and straightforward. The type of information in the treatment algorithm are heterogeneous including clinical, hemodynamic, medical, interventional, pharmacological and regulatory recommendations. Stakeholders (or users) including physicians from various specialties and with variable expertise in PAH, nurses, patients and patients' associations, healthcare providers, regulatory agencies and industry are often interested in the PAH treatment algorithm for different reasons. These are the considerable challenges faced when proposing appropriate updates to the current evidence-based treatment algorithm.The current treatment algorithm may be divided into 3 main areas: 1) general measures, supportive therapy, referral strategy, acute vasoreactivity testing and chronic treatment with calcium channel blockers; 2) initial therapy with approved PAH drugs; and 3) clinical response to the initial therapy, combination therapy, balloon atrial septostomy, and lung transplantation. All three sections will be revisited highlighting information newly available in the past 5 years and proposing updates where appropriate. The European Society of Cardiology grades of recommendation and levels of evidence will be adopted to rank the proposed treatments.


6-min walk distance; 6MWD; APAH; BAS; CCB; ERA; PAH; PVR; RCT; WHO-FC; World Health Organization Functional Class; associated pulmonary arterial hypertension; balloon atrial septostomy; calcium channel blocker; endothelin receptor antagonist; endothelin receptor antagonists; guanylate cyclase stimulators; hypertension, pulmonary; lung transplantation; phosphodiesterase type-5 inhibitors; prostanoids; pulmonary; pulmonary arterial hypertension; pulmonary vascular resistance; randomized controlled trials

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