Abstract

Background: Infants with single ventricle heart disease have a 20% mortality rate during the first year of life with most deaths occurring prior to Stage II/Glenn. Although the time after the Stage II procedure is believed to be a lower risk period, significant morbidity and mortality may occur during this time. Methods: Retrospective cohort analysis of infants enrolled in the National Pediatric Cardiology Improvement Collaborative who underwent staged single ventricle palliation from 2016-2021 and survived to Stage II operation. Multivariable logistic regression and classification and regression tree analysis were performed to identify risk factors for the composite of mortality or transplantation between Stage II surgery and the first birthday. Candidate variables (n=75) included sociodemographic, prenatal, birth, anatomic, stage I intra- and post-operative, interstage course, and Stage II operative characteristics. Results: Of the 1455 patients in the cohort who survived to Stage II, the overall event rate was 7% (76 (5%) died and 34 (2%) were referred for transplant). Overall event rates at 30 and 100 days after Stage 2 were 2% and 5%, respectively ( Figure 1A ). Independent risk factors for mortality and transplantation included shunt type and AV valve repair at Stage I, ECMO and reintubation after Stage I, pre-operative weight and ≥ moderate AV valve regurgitation prior to Stage II, Stage II cross-clamp time, and comprehensive Stage II surgery ( Figure 1B). Patient race/ethnicity and socioeconomic status were not associated with worse outcome. Conclusions: Mortality rates after Stage II surgery to 1 year of age remain high ~5%. Some of the risk factors identified have been shown to also predict interstage mortality, whereas others (reintubation, pre-operative weight, and Stage II characteristics) may be unique to the period after Stage II. Further research is needed to understand whether risk factor modification results in improved transplant-free survival.

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