Long-Term Results after Fallot’s Repair

Objectives: The aim of this study was to evaluate the long-term outcome and freedom from pulmonary valve replacement (PVR) after the initial repair of the tetralogy of Fallot (TOF). In Addition, the anatomical variations and surgical techniques were analyzed. Patients were treated with transanular patch (TAP), valve sparing repair (VSR), or conduits from the right ventricle to the pulmonary arteries (RVPA-conduits).

Methods: Hospital records and operative protocols of all operated patients between April 1980 and June 2017 were reviewed. 306 patients were identified and divided into anatomical subgroups according to diagnosis of TOF-pulmonary stenosis, TOF-pulmonary atresia, and TOF-double outlet right ventricle. Of these patients, 91 received PVR after 21.9 ± 1.0 years. Follow-up was 35.6 ± 0.6 years.

Results: Complete case notes of 306 patients were reviewed and analyzed. There were 21 deaths (6.86%), 14 of them being early deaths (4.58%) after primary correction, 4 deaths (1.31%) occurred in association with secondary interventions on the heart. One patient died after a noncardiac operation (0.33%) and there were two late deaths (0.65%). However, during the last 12 years, no early mortality following repair has been observed. Ninety-one patients (30.39%) received PVR with an early mortality of 2.20% (n = 2). There was no late mortality after PVR. There was a significant difference in freedom from reoperation after TAP, VSR, and RVPA-conduits. Freedom from PVR for TAP was 19.1 ± 1.2 years, for VSR 24.6 ± 1.3 years and for RVPA-Conduits 12.1 ± 2.2 years (p < 0.001). Primary palliation (p = 0.004), small Z-scores for the pulmonary valve (p = 0.013), longer time on bypass (p < 0.001), and longer crossclamping times (p = 0.05) were associated with a significant risk of PVR. Presence of a genetic syndrome (p = 0.002) and longer crossclamping times (p = 0.018) showed an increased risk of death.

Conclusion: The repair of TOF has a beneficial long-term prognosis and low mortality. Genetic syndromes and longer crossclamping times were negatively associated with survival. PVR is a frequently needed second operation with a considerable low risk even in the setting with multiple reoperations. Primary palliative procedures, small pulmonary valve Z-scores and longer crossclamp times are associated with earlier reoperations. TAP leads to earlier reoperation, but has not been a significant risk factor for PVR and has no negative impact on survival.

No conflict of interest has been declared by the author(s).