Long term outcome after surgical tetralogy of Fallot repair at young age: Longitudinal follow-up up to 50 years after surgery.

Background: Little is known about the very long-term outcome in Tetralogy of Fallot (ToF) patients. Objectives: To prospectively evaluate clinical outcome and quality-of-life after surgical repair of ToF. Methods: Single-centre, longitudinal cohort-study evaluating every decade 144 ToF patients who underwent surgical repair <15 years of age between 1968 and 1980. Results: Evaluated were 66 patients (92 %) of the 72 eligible survivors (58 % male, median age at study 48.5 years) with a median follow-up of 45 (range 39-52) years. Cumulative survival at 50 years was 71 % and 84 % when excluding 30-days mortality, while event-free survival was 9 %. Reintervention (40 %) and symptomatic arrhythmias (21 %) were the most common complications, although ventricular tachycardia (VT) was rare (7 %). Cardiac magnetic resonance imaging showed a right ventricular ejection fraction <45 % in 45 % of the cohort and 42 % had a diminished left ventricular function. The aortic root diameter increased over time to >40 mm in 45 % of patients. VO ₂max was reduced in 53 % of patients but stable over time. Self-perceived quality-of-life was stable and comparable to the general Dutch population. Early post-operative arrhythmias, pre-operative low oxygen saturation of the left atrium, VT and declining exercise capacity over time were predictive for mortality, transannular patch for both arrhythmia and pulmonary valve replacement. Surgery post-1975 was protective for pulmonary valve replacement. Conclusion: Only 9 % of ToF patients is alive without a major event at 50 years after surgical repair. Reintervention and symptomatic arrhythmias are especially common, although symptomatic VT is rare. Exercise capacity and self-perceived quality-of-life remained stable.