Le Cœur en Sabot: shape associations with adverse events in repaired tetralogy of Fallot

J Cardiovasc Magn Reson. 2022 Aug 4;24(1):46. doi: 10.1186/s12968-022-00877-x.


Background: Maladaptive remodelling mechanisms occur in patients with repaired tetralogy of Fallot (rToF) resulting in a cycle of metabolic and structural changes. Biventricular shape analysis may indicate mechanisms associated with adverse events independent of pulmonary regurgitant volume index (PRVI). We aimed to determine novel remodelling patterns associated with adverse events in patients with rToF using shape and function analysis.

Methods: Biventricular shape and function were studied in 192 patients with rToF (median time from TOF repair to baseline evaluation 13.5 years). Linear discriminant analysis (LDA) and principal component analysis (PCA) were used to identify shape differences between patients with and without adverse events. Adverse events included death, arrhythmias, and cardiac arrest with median follow-up of 10 years.

Results: LDA and PCA showed that shape characteristics pertaining to adverse events included a more circular left ventricle (LV) (decreased eccentricity), dilated (increased sphericity) LV base, increased right ventricular (RV) apical sphericity, and decreased RV basal sphericity. Multivariate LDA showed that the optimal discriminative model included only RV apical ejection fraction and one PCA mode associated with a more circular and dilated LV base (AUC = 0.77). PRVI did not add value, and shape changes associated with increased PRVI were not predictive of adverse outcomes.

Conclusion: Pathological remodelling patterns in patients with rToF are significantly associated with adverse events, independent of PRVI. Mechanisms related to incident events include LV basal dilation with a reduced RV apical ejection fraction.

Keywords: Biomarker; Biventricular shape; Magnetic resonance imaging; Tetralogy of Fallot.

Publication types

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

MeSH terms

  • Cardiac Surgical Procedures* / adverse effects
  • Humans
  • Predictive Value of Tests
  • Pulmonary Valve Insufficiency* / diagnostic imaging
  • Pulmonary Valve Insufficiency* / etiology
  • Pulmonary Valve Insufficiency* / surgery
  • Tetralogy of Fallot* / complications
  • Tetralogy of Fallot* / diagnostic imaging
  • Tetralogy of Fallot* / surgery
  • Ventricular Function, Right