Updated treatment algorithm of pulmonary arterial hypertension

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


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.

Keywords: 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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Algorithms*
  • Animals
  • Antihypertensive Agents / therapeutic use
  • Calcium Channel Blockers / therapeutic use
  • Familial Primary Pulmonary Hypertension
  • Humans
  • Hypertension, Pulmonary / diagnosis*
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / therapy*
  • Lung Transplantation / trends
  • Phosphodiesterase Inhibitors / therapeutic use
  • Treatment Outcome


  • Antihypertensive Agents
  • Calcium Channel Blockers
  • Phosphodiesterase Inhibitors