The Association of Gestational Age and Size with Management Strategies and Outcomes in Symptomatic Neonatal Tetralogy of Fallot

Abstract

In neonatal, symptomatic tetralogy of Fallot (sTOF), data are lacking on whether high-risk groups would benefit from staged (SR) or complete repair (CR). We studied the association of gestational age (GA) at birth and z-score for birth weight (BWz), with management strategy and outcomes in sTOF. California population-based cohort study (2011-2017) of infants with sTOF (defined as catheter or surgical intervention prior to 44 weeks corrected GA) was performed, comparing management strategy and timing by GA and BWz categories. Multivariable models evaluated composite outcomes and days alive and out of hospital (DAOOH) in the first year of life. Among 345 patients (SR = 194; CR = 151), management strategy did not differ by GA or BWz with complete repair defined as prior to 44 weeks corrected gestational age; however, did differ by GA with regard to complete/timely repair (defined as complete repair within first 30 days of life). Full-term and early-term neonates underwent CR 20 (95%CI: - 27.1, - 14.1; p < 0.001) and 15 days (95%CI: - 22.1, - 8.2; p < 0.001) sooner than preterm neonates. Prematurity and major anomaly were associated with mortality or non-cardiac morbidity, while only major anomaly was associated with mortality or cardiac morbidity (OR = 3.5, 95%CI: 1.8,6.7, p < .0001). Full-term infants had greater DAOOH compared to preterm infants (35.2 days, 95%CI: 4.0, 66.5, p = 0.03). LGA infants and those with major anomaly had significantly lower DAOOH. In sTOF, patient specific risk factors such as prematurity and major anomaly were more associated with outcomes than management strategy.

Keywords: Neonatal tetralogy of Fallot; Prematurity; Surgical outcomes.

Fig. 1

Kaplan Meier curves by surgical approach stratified by gestational age category at birth (preterm (≤36 weeks, n = 96); early term (37–38 weeks, n = 97); full-term (>38 weeks, n = 152). Definition used in this analysis: Timely and complete repair = complete repair within 30 days of life. Although not statistically significant, there appeared to be a visual difference suggesting lower survival probability in premature neonates that underwent a timely complete repair compared to those that underwent a delayed (i.e. >30 days of life) or palliative repair (p = 0.07)

Fig. 2

Kaplan Meier curves by surgical approach stratified by birth weight category (SGA = small for gestational age (n = 63); AGA = appropriate for gestational age (n = 264); LGA = large for gestational age (n = 18)). Definition used in this analysis: Timely and complete repair = complete repair within 30 days of life. Although not statistically significant, there appeared to be a visual difference on the Kaplan Meier curve suggesting lower survival probability up to 1 year of life in LGA infants that underwent a timely and complete repair compared to those that underwent a delayed (i.e. >30 days of life) or palliative intervention (p = 0.2)