Tetralogy of Fallot in the Current Era

Only few studies have reported long-term outcome of the transatrial-transpulmonary approach in the current era of management of tetralogy of Fallot (ToF). We investigated 15-year outcome of correction via a transatrial-transpulmonary approach in a large cohort of successive patients operated in the 21st century. All infant ToF patients undergoing transatrial-transpulmonary ToF correction between 2000 and 2015 were included (N = 177, 106 male, median follow-up 7.1 (interquartile range 3.0-10.9) years. Data regarding postoperative complications, reinterventions, development of atrial and ventricular arrhythmia, cardiac function, and survival were evaluated. Prior shunting was performed in 10 patients (6%). The transatrial-transpulmonary approach resulted in valve-sparing surgery in 57 patients (32%). Postoperative surgical complications included junctional ectopic tachycardia (N = 12, 7%), pericardial (N = 10, 6%) or pleural effusion (N = 7, 3%), chylothorax (N = 7, 4%), bleeding requiring reoperation (N = 4, 3%), and superficial wound infection (N = 1). Fifty-one patients underwent 68 reinterventions, mainly due to pulmonary restenosis (PS) (N = 57). ToF correction at age <2 months and double outlet or double-chambered right ventricle variants of the ToF spectrum were independent predictors for reintervention. Patients undergoing valve-sparing ToF correction had a significant longer PR-free survival than those with a transannular patch (8.5 [95% confidence interval 6.8-10.3] years vs 1.1 [95% confidence interval 0.8-1.5] years; P < 0.001). Overall mortality was 2.8%; mortality rates were higher in premature/dysmature newborns (0.7% vs 9.5%; P < 0.001). Although the 15-year outcome of the transatrial-transpulmonary approach in terms of postoperative complications and mortality rates is excellent, the high incidence of moderate and severe PR is worrisome. Valve-sparing surgery was associated with a substantially lower incidence of PR, yet was surgically not possible in the majority of patients.