Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology

Anton Vonk-Noordegraaf*, François Haddad, Kelly M Chin, Paul R Forfia, Steven M Kawut, Joost Lumens, Robert Naeije, John Newman, Ronald J Oudiz, Steve Provencher, Adam Torbicki, Norbert F Voelkel, Paul M Hassoun

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Survival in patients with pulmonary arterial hypertension (PAH) is closely related to right ventricular (RV) function. Although pulmonary load is an important determinant of RV systolic function in PAH, there remains a significant variability in RV adaptation to pulmonary hypertension. In this report, the authors discuss the emerging concepts of right heart pathobiology in PAH. More specifically, the discussion focuses on the following questions. 1) How is right heart failure syndrome best defined? 2) What are the underlying molecular mechanisms of the failing right ventricle in PAH? 3) How are RV contractility and function and their prognostic implications best assessed? 4) What is the role of targeted RV therapy? Throughout the report, the authors highlight differences between right and left heart failure and outline key areas of future investigation.

Original languageEnglish
Pages (from-to)D22-33
JournalJournal of the American College of Cardiology
Issue number25 Suppl
Publication statusPublished - 24 Dec 2013


  • Adaptation, Physiological
  • Animals
  • Familial Primary Pulmonary Hypertension
  • Humans
  • Hypertension, Pulmonary
  • Ventricular Dysfunction, Right
  • Ventricular Function, Right
  • Ventricular Remodeling
  • Journal Article
  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

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