Right heart adaptation to pulmonary arterial hypertension: physiology and pathobiology

J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D22-33. doi: 10.1016/j.jacc.2013.10.027.


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.

Keywords: B type natriuretic peptide; BNP; CO; Ea; Ees; LV; MHC; MPAP; MRI; PAC; PAH; PH; PVR; RAP; RHF; RNA; RV; RVEF; arterial elastance; cardiac output; echocardiography; heart failure; left ventricular; mean pulmonary arterial pressure; myocardium; myosin heavy chain; pulmonary arterial compliance; pulmonary arterial hypertension; pulmonary artery hypertension; pulmonary hypertension; pulmonary vascular resistance; ribonucleic acid; right atrialpressure; right heart failure; right ventricle; right ventricular; right ventricular ejection fraction; ventricular elastance.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Review

MeSH terms

  • Adaptation, Physiological / physiology*
  • Animals
  • Familial Primary Pulmonary Hypertension
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / physiopathology*
  • Ventricular Dysfunction, Right / diagnosis
  • Ventricular Dysfunction, Right / physiopathology*
  • Ventricular Function, Right / physiology*
  • Ventricular Remodeling / physiology