Updated treatment algorithm of pulmonary arterial hypertension

Nazzareno Galiè, Paul A. Corris, Adaani Frost, Reda E. Girgis, John Granton, Zhi Cheng Jing, Walter Klepetko, Michael D. McGoon, Vallerie V. McLaughlin, Ioana R. Preston, Lewis J. Rubin, Julio Sandoval, Werner Seeger, Anne Keogh

Research output: Contribution to journalArticlepeer-review

488 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)D60-D72
JournalJournal of the American College of Cardiology
Issue number25 SUPPL.
StatePublished - Dec 24 2013
Externally publishedYes


  • endothelin receptor antagonists
  • guanylate cyclase stimulators
  • hypertension, pulmonary
  • lung transplantation
  • phosphodiesterase type-5 inhibitors
  • prostanoids
  • pulmonary

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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