Abstract

The aim of this study was to evaluate experience and predictors of early mortality in patients with hypoplastic left heart syndrome (HLHS)-type defects undergoing Norwood procedure (NP) with right ventricle-to-pulmonary artery (RV-PA) shunt. Between 2001 and 2009, a consecutive series of 229 children with HLHS-type single ventricle underwent NP with application of RV-PA shunt. Demographic, echocardiographic, and clinical perioperative data were retrospectively analyzed. The mean duration of follow-up of survivors was 4.5 ± 2.1 years (60 days to 8.1 years). Follow-up was complete for 92.1% of patients. Major early postoperative complications included sepsis/generalized infection in 40 (17.5%), pericardial effusion in 9 (3.9%), and wound infection in 8 (3.5%). The early (30-day) survival was 87.8% (n = 201). In the late postoperative period, 12 (5.9%) died. Early nonsurvivors were more frequently older than 14 days (P = .045) at initial surgery, had lower operative weight (P = .024), had more frequent associated cardiac (P < .001) and/or extracardiac anomalies (P < .001), and were more likely to have a restrictive interatrial communication before operation (P = .024). Use of the right RV-PA shunt has helped to mitigate some previously described predictors of early death after NP. Longer follow-up will be required to determine whether the RV-PA shunt modification confers an important survival benefit.

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