Risk factors for mortality in patients with hypoplastic left heart syndrome after the Norwood procedure.

Abstract

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 effect of these factors. We examined both the independent and the cumulative effect of preoperative risk factors for poor outcome in patients undergoing the Norwood procedure. Moreover, we studied the risk factors associated with prolonged initial hospital stay in these patients. We performed a retrospective national 18-year observational study of preoperative risk factors for one-year, as well as total follow-up mortality or need for transplant in HLHS-patients (N = 99) born in Finland between 1 January 2004 and 31 December 2021. Overall, one-year survival was 85.6%. In a multivariable analysis, major extracardiac anomaly and being small for gestational age were significant predictors of one-year mortality or need for transplant. Aortic atresia was a predictor of total follow-up mortality. When analyzing the cumulative effect, the presence of two risk factors was associated with higher mortality. 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.