AIMS: Pulmonary regurgitation (PR) causes progressive right ventricle (RV) dilatation and dysfunction in repaired tetralogy of Fallot (rToF). Declining RV function is often insidious and the timing of pulmonary valve replacement remains under debate. Quantifying the pathophysiology of adverse RV remodelling due to worsening PR may help in defining the best timing for pulmonary valve replacement. Our aim was to identify whether complex three-dimensional (3D) deformations of RV shape, as assessed with computer modelling, could constitute an anatomical biomarker that correlated with clinical parameters in rToF patients. METHODS AND RESULTS: We selected 38 rToF patients (aged 10-30 years) who had complete data sets and had not undergone PVR from a population of 314 consecutive patients recruited in a collaborative study of four hospitals. All patients underwent cardiovascular magnetic resonance (CMR) imaging: PR and RV end-diastolic volumes were measured. An unbiased shape analysis framework was used with principal component analysis and linear regression to correlate shape with indexed PR volume. Regurgitation severity was significantly associated with RV dilatation (P = 0.01) and associated with bulging of the outflow tract (P = 0.07) and a dilatation of the apex (P = 0.08). CONCLUSION: In this study, we related RV shape at end-diastole to clinical metrics of PR in rToF patients. By considering the entire 3D shape, we identified a link between PR and RV dilatation, outflow tract bulging, and apical dilatation. Our study constitutes a first attempt to correlate 3D RV shape with clinical metrics in rToF, opening new ways to better quantify 3D RV change in rToF.