Mid-term neurodevelopmental outcome in children with HLHS and related anomalies

Abstract

Background: Surgical outcome for HLHS and related anomalies has been improved, and today, more than 80% patients can survive Norwood procedure and reach Fontan completion. However, morbidity rate is still high, and neurological and developmental outcome is unclear. The purpose of this study is to elucidate the mid-term neurodevelopmental outcome in patients with HLHS and related anomalies, and to assess the appropriate surgical strategy. Patients and methods: Twenty-six (26) HLHS patients or its variants who underwent the initial surgical palliation between 2003 and 2011 were enrolled (Neonatal Norwood: 6, Bilateral PAB: 20). Twenty-three (23) patients had reached Fontan completion, and the other 3 BDG patients are awaiting TCPC. Bayley scale at 1-year-old (10), at 3-year-old (11) and WISC-III at 6-year-old (11) have been used for the developmental evaluation since 2008. Six patients took 2 types of examination during the study period. Results: Bayley scale: Although most functions are still delayed in HLHS at 1-year old, they are significantly improved at 3-year-old. There was no difference between neonatal Norwood group (4) and bilateral PAB group (7), but 2 patients in neonatal Norwood group showed low FIQ less than 80. View this table: Results of Bayley scale & WISC-III Conclusions: Mid-term neurodevelopmental outcome in Norwood survivors is acceptable, where patients may catch up with their growth. However, some patients show developmental delay, suggesting the possible worse effect of Norwood circulation in the neonatal period. Further study is required.