Identifying Risk Factors for Massive Right Ventricular Dilation in Patients With Repaired Tetralogy of Fallot

In repaired tetralogy of Fallot (rTOF), pulmonary insufficiency results in varying degrees of right ventricle (RV) dilation. A subset of patients is diagnosed at initial cardiac magnetic resonance imaging (CMR) with a massively dilated RV, far beyond pulmonary valve replacement (PVR) criteria, which is unlikely to return to normal size after PVR. This study aimed to identify risk factors for massive RV dilation at initial CMR. This nested case-control study included all patients at our institution with rTOF and massive RV dilation (indexed RV end-diastolic volume [RVEDVi] ≥200 ml/m²) on initial CMR. Patients were matched by age at first CMR, gender, and type of repair with rTOF controls with RVEDVi<200 ml/m². In 39 cases (median RVEDVi 227 ml/m², interquartile range [IQR] 213 to 250) and 73 controls (median RVEDVi 155 ml/m², IQR 130 to 169), repair at >6 months of age, longer QRS duration, and non-Caucasian race were significantly associated with massive RV dilation on univariate analysis. In multivariate analysis, repair at >6 months of age (adjusted odds ratio [AOR] 2.90, 95% confidence interval [CI] 1.12 to 7.55, p = 0.03), longer QRS duration (AOR = 1.03, 95% CI 1.01 to 1.05, p = 0.005), and non-Caucasian race (AOR = 7.84, 95% CI 1.76 to 34.8, p = 0.01) remained independently associated with massive RV dilation. Era of repair, history of systemic to pulmonary shunt palliation, genetic anomaly, and additional cardiac lesions did not differ between groups. In conclusion, these risk factors identify a subset of patients who may benefit from earlier CMR evaluation to avoid massive irreversible RV dilation.