Adverse remodelling in tetralogy of Fallot: From risk factors to imaging analysis and future perspectives

Hellenic J Cardiol. 2024 Jan-Feb:75:48-59. doi: 10.1016/j.hjc.2023.07.008. Epub 2023 Jul 25.

Abstract

Although contemporary outcomes of initial surgical repair of tetralogy of Fallot (TOF) are excellent, the survival of adult patients remains significantly lower than that of the normal population due to the high incidence of heart failure, ventricular arrhythmias, and sudden cardiac death. The underlying mechanisms are only partially understood but involve an adverse biventricular response, so-called remodelling, to key stressors such as right ventricular (RV) pressure-and/or volume-overload, myocardial fibrosis, and electro-mechanical dyssynchrony. In this review, we explore risk factors and mechanisms of biventricular remodelling, from histological to electro-mechanical aspects, and the role of imaging in their assessment. We discuss unsolved challenges and future directions to better understand and treat the long-term sequelae of this complex congenital heart disease.

Keywords: cardiac magnetic resonance; congenital heart disease; echocardiography.

Publication types

  • Review

MeSH terms

  • Adult
  • Arrhythmias, Cardiac
  • Cardiac Surgical Procedures* / adverse effects
  • Heart Failure* / complications
  • Humans
  • Risk Factors
  • Tetralogy of Fallot* / complications
  • Tetralogy of Fallot* / diagnostic imaging
  • Tetralogy of Fallot* / surgery
  • Ventricular Dysfunction, Right*