Background: Regional low-flow perfusion of the brain is a bypass technique commonly used during stage 1 reconstruction in neonates with hypoplastic left heart syndrome and related variants. The neurodevelopmental outcome of these children is unknown.
Methods: Twenty-nine infants (22 boys, 7 girls) with hypoplastic left heart syndrome or variant requiring single ventricle palliation and aortic arch reconstruction were studied between 1999 and 2004. Mental Developmental Index (MDI) and Psychomotor Developmental Index were assessed using Bayley Scales of Infant Development and correlated with intraoperative and perioperative variables. Results are reported as mean +/- standard deviation.
Results: Average age at stage 1 operation and at bidirectional Glenn was 7 +/- 8 days and 6.0 +/- 2 months, respectively. The MDI was in the low average range (87.7 +/- 13.2). The Psychomotor Developmental Index was in the mildly delayed range (75.2 +/- 14.5). Regional low-flow perfusion was used in 31% (9 of 29 patients), with an average circulatory arrest time of 23.5 +/- 13.4 minutes. Deep hypothermia and circulatory arrest was used as the primary operative strategy in 69% of patients (20 of 29 patients), with an average circulatory arrest time of 44.3 +/- 15.3 minutes (p = 0.003). No differences in MDI or Psychomotor Developmental Index scores were observed between the regional low-flow perfusion and non-regional low-flow perfusion groups (MDI, 88.0 +/- 12.1 versus 87.6 +/- 14.0; p = 0.93, respectively; Psychomotor Developmental Index, 75.5 +/- 15.1 versus 75.0 +/- 14.6; p = 0.93, respectively). Lowest operative temperature (<16 degrees C) and birth order (<2 versus >3) significantly related to MDI (89.6 versus 72.8; p = 0.047).
Conclusions: At 1 year of age, neurodevelopmental outcomes of patients undergoing stage 1 using regional low-flow perfusion were similar to outcomes observed in children exposed to circulatory arrest. The association of birth order and MDI suggests that early intervention may benefit these patients.