Turk Kardiyol Dern Ars. 2014; 42(1): 78-94

Updated treatment algorithm of pulmonary arterial hypertension

Nazzareno Galiè¹, Paul A. Corris², Adaani Frost³, Reda E. Girgis⁴, John Granton⁵, Zhi Cheng Jing⁶, Walter Klepetko⁷, Michael D. Mcgoon⁸, Vallerie V. Mclaughlin⁹, Ioana R. Preston¹⁰, Lewis J. Rubin¹¹, Julio Sandoval¹², Werner Seeger¹³, Anne Keogh^14
1Bologna University Hospital, Department of Experimental Diagnostic and Specialty Medicine (DIMES), Bologna, Italy
²Institute of Cellular Medicine, Newcastle University vs Newcastle Hospitals NHS Foundation Trust, Newcastle, England
³Baylor College of Medicine, Houston, Texas, USA
⁴Michigan State University College of Human Medicine, Grand Rapids, Michigan, USA
⁵University of Toronto, Respiroloj Department, Toronto, Canada
⁶Fu Wei Peking Union Hospital and the National Center for Cardiovascular Disease Medical College and Chinese Academy of Medical Sciences, Beijing, China
⁷Medical University of Vienna, Vienna General Hospital, Breast Surgery, Vienna, Austria
⁸Mayo Clinic, Cardiovascular Diseases, Rochester, Minnesota, USA
⁹The University of Michigan Cardiovascular Medicine, Ann Arbor, Michigan, USA
¹⁰Tufts University School of Medicine, Tufts Mib Center, Pulmonary, Critical Care and Sleep Department, Boston, Massachusetts, USA
¹¹California University, School of Medicine, San Diego, La Jolla, California, USA
¹²Mexico National Institute of Cardiology, Clinical Research, Mexico City, Mexico
¹³Universities of Giessen und Marburg Lung Center, the Max Planck Institute for Heart and Lung, Giessen / Bad Nauheim, Germany
¹⁴St. Vincent's Hospital, Heart Transplantation Unit, 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. (J Am Coll Cardiol 2013;62: D60–72) ©2013 by the American College of Cardiology Foundation.

Keywords: Endothelin receptor antagonists, guanylate cyclase stimulators, hypertension, pulmonary; lung transplantation; phosphodiesterase type-5 inhibitors; prostanoids; pulmonary

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