Staged vs primary repair: which is more effective for tetralogy of Fallot with cyanosis?

A study comparing staged (SR) and primary repair (PR) for the management of neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) shows lower early mortality and neonatal morbidity in the SR group. However, cumulative morbidity and reinterventions were more favourable in the PR group.

The authors conducted a balanced multicentre comparison of SR (initial palliation [IP] and subsequent complete repair [CR]) as opposed to PR treatment strategies. They retrospectively reviewed consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 from the Congenital Cardiac Research Collaborative.

Death was the primary outcome, while secondary ones included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anaesthesia, ventilation, and inotrope use; and complication and reintervention rates. Propensity-score adjustment was used to compare outcomes.

A total of 342 patients underwent SR (IP: surgical, n=256; transcatheter, n=86) and 230 underwent PR. Preprocedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more frequent in the SR group (p≤0.01). At median 4.3 years, no significant between-group difference was observed in the risk of death (10.2 percent vs 7.4 percent; p=0.25).

Mortality risk remained similar between groups after adjustment (hazard ratio, 0.82, 95 percent confidence interval, 0.49–1.38; p=0.456), but was favourable in the SR group during early follow-up (<4 months; p=0.041). Component analysis revealed favourable secondary outcomes in the SR group, but cumulative analysis favoured the PR group. Of note, reintervention risk was higher among patients in the SR group (p=0.002).

These findings suggest potential benefits to each strategy, according to the authors.