Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome

Background - Late morbidity and mortality remain problematic after repair of tetralogy of Fallot (TOF). We hypothesized that fibrosis detected by late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) would be present in adults with repaired TOF and would be related to adverse markers of outcome. Method and Results - LGE was scored in the right and left ventricles (RV and LV) of 92 adult patients who had undergone TOF repair. RV LGE was seen in all patients at surgical sites located in the outflow tract (99%) or the site of ventricular septal defect patching (98%) and in the inferior RV insertion point (79%) and trabeculated myocardium (24%). LV LGE (53%) was located at the apex consistent with apical vent insertion (49%), in the inferior or lateral wall consistent with infarction (5%), or in other areas (8%). Patients with supramedian RV LGE score were older (38 versus 27 years, P < 0.001) and more symptomatic (38% versus 8% in New York Heart Association class II or greater, P = 0.001), had increased levels of atrial natriuretic peptide (7.3 versus 4.9 pmol/L, P = 0.041), and had a trend to higher brain natriuretic peptide (12.3 versus 7.2 pmol/L, P = 0.086), exercise intolerance (maximum (V)over dot O-2 24 versus 28 mL.min(-1).kg(-1), P = 0.021), RV dysfunction (RV end-systolic volume 61 versus 55 mL/m(2), P = 0.018; RV ejection fraction 50% versus 56%, P = 0.007), and clinical arrhythmia (26% versus 10%, P = 0.039). Non-apical vent LV LGE also correlated with markers of adverse outcome. In a multivariate model, RV LGE remained a predictor of arrhythmia. Conclusions - RV and LV LGE were common after TOF repair and were related to adverse clinical markers, including ventricular dysfunction, exercise intolerance, and neurohormonal activation. Furthermore, RV LGE was significantly associated with clinical arrhythmia.