Perioperative Factors Influence the Long-Term Outcomes of Children and Adolescents with Repaired Tetralogy of Fallot

Tetralogy of Fallot (TOF) often carries long-term sequelae following surgical intervention. We hypothesized that early perioperative factors are associated with long-term adverse right ventricular (RV) remodeling, diminished exercise capacity, and increased morbidity. We conducted a retrospective cohort study of patients operated for TOF that underwent cardiac magnetic resonance imaging study (CMR), exercise stress test (EST), and detailed review of past medical history. Outcome variables included measures of RV size, and function, maximal work rate, and oxygen consumption, and interim hospitalizations, surgeries, and catheterizations. Thirty-nine subjects were included. Age at surgical repair was 0.3 +/- 0.3years and age at testing was 9.7 +/- 1.4years. On CMR, there was borderline RV dilation with moderate pulmonary insufficiency (PI) [RF 32% (8; 43)] and normal RV ejection fraction [60% (55; 67)]. On EST, there was low percent-predicted maximal oxygen consumption (77 +/- 20%), and percent-predicted maximal work rate (84 +/- 23%). On multivariable analysis, mechanical ventilation and Blalock-Taussig (BT) shunt prior to complete surgical repair were associated with the number of future hospitalizations. Duration of cardiopulmonary bypass and prior BT shunt were associated with future catheterizations. Prior BT shunt was a predictor of worse RVEF, while duration of mechanical ventilation and use of transannular patch were predictors of worse PI. Longer duration of mechanical ventilation (or LOS) was associated with worse maximal work rate. Surgical and perioperative factors may portend long-term RV remodeling and outcome in TOF. Further studies are warranted to explore these associations and potential underlying mechanisms.