Key result
Major extracardiac anomalies and being small for gestational age predicted 1-year mortality or transplant need in HLHS patients undergoing the Norwood procedure (overall 1-year survival 85.6%).
Why the study?
Mortality risk factors in hypoplastic left heart syndrome remain ambiguous and have mainly focused on independent effects rather than cumulative preoperative risk factors.
Observational (n=99)
Major extracardiac anomalies, being small for gestational age, and aortic atresia are significant preoperative risk factors for mortality or transplant in HLHS patients after the Norwood procedure.
May refine preoperative counseling for HLHS families; leaves open whether targeted interventions improve outcomes.
OBJECTIVES: Several studies have reported mortality risk factors associated with hypoplastic left heart syndrome (HLHS). However, these data are ambiguous and mainly focused on the independent effects of these factors. We examined both the independent and the cumulative effects of preoperative risk factors for poor outcome in patients undergoing the Norwood procedure. Moreover, we studied the risk factors associated with prolonged initial hospital stays in these patients. METHODS: We performed a retrospective national 18-year observational study of preoperative risk factors for 1 year, as well as total follow-up mortality or need for transplant in patients with HLHS (N = 99) born in Finland between 1 January 2004 and 31 December 2021. RESULTS: Overall, one-year survival was 85.6%. In a multivariable analysis, having a major extracardiac anomaly and being small for gestational age were significant predictors of one-year mortality or the need for a transplant. Aortic atresia was a predictor of total follow-up mortality. An analysis of the cumulative effect indicated that the presence of 2 risk factors was associated with higher mortality. CONCLUSIONS: HLHS remains the defect with the highest procedural risks for mortality in paediatric cardiac surgery. From a prognostic point of view, recognition of independent preoperative risk factors as well as the cumulative effect of risk factors for mortality is essential.The results of this study were presented orally at the 55th Annual Meeting of the Association for European Paediatric and Congenital Cardiology, Geneva, Switzerland, 28 May 2022.
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Selenius et al. (2023) conducted an observational in Hypoplastic left heart syndrome (HLHS) (n=99). Preoperative risk factors (major extracardiac anomaly, small for gestational age, aortic atresia) was evaluated on 1-year mortality or need for transplant. Major extracardiac anomalies and being small for gestational age predicted 1-year mortality or transplant need in HLHS patients undergoing the Norwood procedure (overall 1-year survival 85.6%).
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