Early and long-term results of biventricular repair of tetralogy of Fallot with or without pulmonary atresia

Abstract

After complete repair, the long-term outcome difference between tetralogy of Fallot without pulmonary atresia (TOF) and tetralogy of Fallot with pulmonary atresia (TOF-PA) without MAPCAs is scarcely described. We performed a retrospective study on patients with a diagnostic of TOF and TOF-PA without MAPCAs who underwent complete repair between 1995 and 2016 in our center. We reviewed 960 patients, divided into two main anatomical groups: TOF ( n = 822, 85.7%), and patients with TOF-PA ( n = 138, 14.3%). Concerning surgical technique repair, three groups were distinguished: TOF without transannular patch ( n = 410, 42.7%), TOF/TOF-PA with transannular patch ( n = 494, 51.5%), and TOF-PA with RVOT tube ( n = 56, 5.8%). After complete repair, overall mortality was 11 (1.1%), five of them occurred before discharge (0.5%). Survival at 10 years after repair was 99.1% for TOF and 96.8% for TOF-PA ( P = 0.036). The median follow-up was 10.0 [95% CI: 6.3–14.1] years after repair. One hundred and sixty-three patients (17%) underwent reintervention (surgery or percutaneous interventions). Freedom from reintervention at 10 years after repair was 92.3%, 82.1%, and 48.7% for TOF without transannular patch, TOF/TOF-PA with transannular patch and TOF-PA with RVOT tube respectively ( P < 0.001 between each groups). In addition, reintervention rate was significantly different between TOF with transannular patch and TOF-PA with pulmonary trunk ( P < 0.001). In multivariate analysis, the absence of pulmonary trunk ( P = 0.013), pulmonary artery branches stenosis ( P = 0.001), prior palliative surgery ( P < 0.001), and reintervention before discharge ( P < 0.001) were reintervention risk factors. The initial anatomy of pulmonary trunk and branches strongly influences the risk of reintervention in TOF with or without PA. Avoid palliative surgery by directly performing a complete repair in the neonatal period would be a strategical option to improve the prognosis of these patients.