Introduction:
Symptomatic neonates with tetralogy of Fallot (sTOF) and hypoplastic branch pulmonary arteries (hPA) are at high risk. Management strategies include staged repair (SR) - initial palliation (IP) followed by later complete repair (CR) - or primary repair (PR). A balanced comparison of these approaches is needed in the sTOF neonate with hPA.
Methods:
Consecutive neonates with sTOF and hPA - defined as one hPA z-score <-2.0 with contralateral z-score <0 - who underwent SR or PR from 2005-17 were retrospectively reviewed by the Congenital Catheterization Research Collaborative. Primary outcome was mortality and secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital, ICU and surgical morbidities, and reintervention. Outcomes were compared between treatment strategies using propensity score adjustment (PSA) to account for baseline differences between groups (PR=reference group).
Results:
The cohort comprised 188 neonates including 121 SR (IP: 29 transcatheter; 54 surgery) and 67 PR patients. Presence of pulmonary atresia, lower gestational age and birthweight were more common in the SR cohort. Median right and left hPA z-scores were -2.19 (IQR -2.65, -1.92) and -2.33 (-2.84, -1.99), respectively. There were significant differences in treatment strategy across centers (p<0.01). Overall survival was similar between groups, before (p=0.33) and after PSA (HR 1.91, 0.48-7.7, p=0.36). Reintervention was more common in the SR group overall (HR 1.7, 1.03-2.7, p=0.04), but there was no difference after definitive repair (HR 1.21, 0.71-2.0, p=0.49). Secondary outcomes are shown in Table 1.
Conclusions:
In this multicenter comparison of SR or PR for management of neonates with sTOF and hPA, after PSA, no difference in mortality was found. Neonatal morbidities largely favored the SR group, but cumulative morbidities and reintervention favored the PR group. Further study is needed to determine late impact of these early advantages to SR.