Contemporary Approach to Managing Young Symptomatic Infants With Tetralogy of Fallot in the United Kingdom

Abstract

Objectives

We set out to determine the characteristics and outcomes of symptomatic infants with tetralogy of Fallot who due to insufficient pulmonary blood flow required transcatheter or surgical intervention in early infancy.

Methods

Five centers in the United Kingdom contributed data for infants born with tetralogy of Fallot who underwent an initial procedure between January 2015 and January 2022. The studied group were infants requiring palliative or reparative procedure at less than three months of age. The primary outcome was survival to one year; secondary outcomes were periprocedural complications, cumulative mechanical ventilation time, length of hospital stay, and need for reintervention.

Results

A total of 535 infants with tetralogy of Fallot were identified during the study period. 96 infants underwent initial palliation (58 right ventricular outflow tract stent, 7 ductal stent, 17 balloon pulmonary valvuloplasty, 9 modified Blalock-Taussig-Thomas shunt, and 5 right ventricular outflow tract patch augmentation). 37 infants underwent primary repair at less than three months of age, 402 infants had primary repair at 3 to 12 months of age. Median age and weight were 26 days and 3.1 kg for initial palliation; 68 days and 4.7 kg for primary repair under three months (P < .001). One year survival post-procedure was 95.7% for initial palliation, and 97.3% for primary repair under three months (P = .55, hazard ratio [HR] 1.97, 95% confidence interval [CI] 0.33-11.67). Five-year freedom from reintervention on the right ventricle-to-pulmonary artery segment was 76% after staged repair and 88% after primary repair under three months (P = .23, HR 1.90, 95% CI 0.75-4.80).

Conclusions

Initial palliation with staged repair remains the predominantly employed approach for the highest risk young infants with tetralogy of Fallot in the United Kingdom. Survival outcomes are excellent; concerns remain regarding increased cumulative costs and associated reintervention rates. Primary repair under three months of age has also proven to be feasible with excellent results, although with individual candidate selection bias.