Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot

Abstract

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.

Objectives: This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.

Methods: Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.

Results: The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).

Conclusions: In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy.

Keywords: congenital heart disease; neurodevelopmental outcomes; outcomes; palliation; staged repair.

Conflict of interest statement

Funding Support and Author Disclosures Financial support for this research was derived, in part, from the Kennedy Hammill Pediatric Cardiac Research Fund, the Liam Sexton Foundation, and A Heart Like Ava. Dr. Goldstein has reported consulting relationships with Medtronic, W.L. Gore & Associates, and Edwards Lifesciences; and is an advisory board member for PECA Labs. Dr. Qureshi has reported consulting relationships with W.L Gore & Associates, Edwards Lifesciences, and Abiomed. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Figures

Figure 1:. Study Population.

This cohort diagram depicts which subjects from the original CCRC TOF cohort were included in the current study. In total, 57% of the original CCRC TOF cohort received care at a hospital contributing data to PHIS during a single contemporary period (birth date between 1/2011–12/2017).

Central Figure:. Comparisons of costs associated with Primary and Staged Repair Strategies.

A. This notched box plots depicts the unadjusted observed outcomes: total 18-month cost (left) and cost-per-day-alive (right). The central line depicts the median value, notches the 95% confidence interval of the median, the top and bottom of the box depict the 25th and 75th percentiles, whiskersdepict 1.5 × the interquartile range, and gray circlesdepict individual observations. Data are truncated at the 80th percentile. B. This Forest plot depicts the results of propensity score adjusted analyses. Depicted are the point estimate (diamond) and 95% CI (brackets). The cost ratio between primary repair and staged repair is depicted on the x-axis. The red dashed line marks a cost ratio of 1. The specific cost ratios, confidence intervals, and p-values are reproduced to the right of the graph.