Preservation of right ventricular structure and function following transatrial-transpulmonary repair of tetralogy of Fallot

OBJECTIVES Management strategy of patients with tetralogy of Fallot (TOF)-including timing, as well as surgical technique-remains a controversial topic. We sought to analyse both early and late results of our consistent policy of non-neonatal transatrial/transpulmonary (TA/TP) repair of TOF over almost 14 years, in order to assess preservation or possible deterioration of right ventricular (RV) function. METHODS All 245 consecutive patients with TOF, referred to our group for repair between September 1997 and December 2010, have been prospectively followed up. Their clinical and echocardiographic data were retrospectively analysed. All underwent complete TA/TP repair at a median age of 1.6 years (range 0.2 to 55.6 years). RESULTS Follow-up (median 8.5 years, range 0.5 to 14.6 years) was 100% completed. There was no operative death. There were three early re-operations: one for residual right ventricular outflow tract obstruction (RVOTO), one for intractable arrhythmias and one for remote second ventricular septal defect (VSD). There were three non-cardiac-related late deaths. Actuarial survival at 14 years was 98.8% (242/245). Twenty five patients required late re-operation, including 23 patients (9.4%, 23/245) who had pulmonary valve replacement (PVR). All other patients remained asymptomatic at follow-up. Mean residual right ventricular outflow tract (RVOT) pressure gradients have remained stable (6.8 ± 6.6 mmHg, 95% CI 5.9-7.6, early postoperatively, versus 7.6 ± 7.5 mmHg, 95% CI 6.6-8.6 at follow-up; P = 0.015). The mean qualitative grade of pulmonary valve insufficiency (PVI) increased (from 1.4 ± 0.9, 95% CI 1.3-1.5 at discharge to 2.1 ± 1.2, 95% CI 2-2.3 at follow-up; P < 0.001). Similarly, mean qualitative grade of tricuspid valve insufficiency (TVI) also increased (from 1 ± 0.7, 95% CI 0.9-1 at discharge to 1.5 ± 0.8, 95% CI 1.4-1.6 at follow-up; P < 0.001). However, RV function has remained normal in most patients. No significant arrhythmias have been noted. CONCLUSIONS Our management strategy of non-neonatal TA/TP repair for all patients with TOF is associated with minimal early and late mortality and morbidity, relatively low re-operation rate, preserved RV function and excellent clinical outcomes at follow-up over almost 14 years. Since many patients demonstrated progressive increase in PVI and TVI, a much longer follow-up is necessary to determine the ultimate rates of late re-operation for pulmonary valve replacement (PVR).