Origins and consequences of congenital heart defects affecting the right ventricle

Cardiovasc Res. 2017 Oct 1;113(12):1509-1520. doi: 10.1093/cvr/cvx155.


Congenital heart disease is a major health issue, accounting for a third of all congenital defects. Improved early surgical management has led to a growing population of adults with congenital heart disease, including patients with defects affecting the right ventricle, which are often classified as severe. Defects affecting the right ventricle often cause right ventricular volume or pressure overload and affected patients are at high risk for complications such as heart failure and sudden death. Recent insights into the developmental mechanisms and distinct developmental origins of the left ventricle, right ventricle, and the outflow tract have shed light on the common features and distinct problems arising in specific defects. Here, we provide a comprehensive overview of the current knowledge on the development into the normal and congenitally malformed right heart and the clinical consequences of several congenital heart defects affecting the right ventricle.

Keywords: Cardiac development; Congenital heart disease; Right ventricle.

Publication types

  • Review

MeSH terms

  • Animals
  • Gene Expression Regulation, Developmental
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / diagnostic imaging
  • Heart Defects, Congenital / genetics
  • Heart Defects, Congenital / physiopathology
  • Heart Ventricles / abnormalities*
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / physiopathology
  • Humans
  • Hypertrophy, Right Ventricular / diagnostic imaging
  • Hypertrophy, Right Ventricular / etiology*
  • Hypertrophy, Right Ventricular / genetics
  • Hypertrophy, Right Ventricular / physiopathology
  • Morphogenesis
  • Risk Factors
  • Ventricular Dysfunction, Right / diagnostic imaging
  • Ventricular Dysfunction, Right / etiology*
  • Ventricular Dysfunction, Right / genetics
  • Ventricular Dysfunction, Right / physiopathology
  • Ventricular Function, Right*
  • Ventricular Remodeling