Early childhood health, growth, and neurodevelopmental outcomes after complete repair of total anomalous pulmonary venous connection at 6 weeks or younger.

OBJECTIVE: This interprovincial inception cohort study explores early childhood outcomes and their operative and perioperative predictors after total anomalous pulmonary venous connection repair, simple (patent ductus arteriosus, atrial septal defect, or both) or complex (any associated major cardiac anomalies).

METHODS: From 1996 through 2004, a total of 41 consecutive neonates with total anomalous pulmonary venous connection underwent complete repair with deep hypothermic circulatory arrest. Multidisciplinary health and neurodevelopmental outcomes (Bayley Scales of Infant Development II [1993], Mental and Psychomotor Developmental Indices) were assessed at 18 to 24 months of age. Regression analyses explored potentially modifiable predictors of outcome.

RESULTS: Survival after simple total anomalous pulmonary venous connection repair was 31 of 32 (97%); that after complex repair was 3 of 9 (33%). Relative risk of death was higher for complex (21.3) and obstructive (8.4) total anomalous pulmonary venous connections. Those who died had longer deep hypothermic circulatory arrest times (P < .001). For 34 survivors, Mental Developmental Index was 87 +/- 16 and Psychomotor Developmental Index was 89 +/- 13. Among survivors, 24.6% of variability in Mental Developmental Index was explained by its linear relationship with socioeconomic status, whereas 35.5% of variability in Psychomotor Developmental Index was explained by its linear relationship with weight at surgery and highest plasma lactate level after surgery. North American native peoples made up 38% of survivors, a higher than expected number.

CONCLUSION: Mean developmental scores were in the low average range of normative data. Socioeconomic factors predicted mental outcome. High postoperative lactate level on day 1 predicted low motor scores. Other potentially modifiable variables will need to be explored. Further epidemiologic study relating race to occurrence of total anomalous pulmonary venous connection is suggested. Early neurodevelopmental assessment is recommended.