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| Is Cardiac Diagnosis a Predictor of Neurodevelopmental Outcome after Cardiac Surgery in Infancy? | |
J. W. Gaynor1, Marsha Gerdes1, Alex S. Nord2, Judy Bernbaum1, Elaine H. Zackai1, Gil Wernovsky1, Robert R. Clancy1, Patrick J. Heagerty2, Cynthia B. Solot1, Jo Ann D'Agostino1, Nancy B. Burnham1, Donna McDonald-McGinn1, Susan C. Nicolson1, Thomas L. Spray1, Gail P. Jarvik2; 1The Children's Hospital of Philadelphia, Philadelphia, PA; 2University of Washington, Seattle, WA
Methods: Infants with ventricular septal defect (VSD), tetralogy of Fallot (TOF), transposition of the great arteries (TGA) and hypoplastic left heart syndrome (HLHS) in a study of apolipoprotein E (APOE) polymorphisms and ND outcome underwent ND and genetic evaluation at 4 years of age. Domains tested included: cognition; language; speech; memory; executive function; visual-motor, fine motor and academic skills. Results: Testing was completed in 178 patients with normal genetic evaluations: VSD(26), TOF(44), TGA(41) and HLHS(67). There were no differences in gestational age, ethnicity, APOE genotype, socioeconomic status or maternal education among groups. Age at first operation was significantly lower for patients with TGA and HLHS compared to TOF and VSD. Post-operative length of stay (LOS) was significantly longer for HLHS compared to all other groups and for TGA compared to TOF and VSD. HLHS was significantly correlated with use of deep hypothermic circulatory arrest (DHCA) and multiple operations. Mean scores for each domain were within normal limits for all groups. (Figure) Compared to HLHS, patients with TGA had significantly higher scores for cognition, fine motor skills, executive function, and math skills. Compared to HLHS patients with TOF had higher scores for cognition and executive function. There were no significant differences between HLHS and VSD patients for any domain. Significant impairments in at least 1 domain were identified in 8% (2/25) of patients with VSD, 20% (8/41) with TOF, 17% (7/41) with TGA and 18% (12/65) with HLHS. After correction for demographic, pre-operative, and operative variables; there were no significant differences among groups for any domain. Conclusion: Mean scores for ND outcomes are in the normal range for preschool children with no recognized genetic syndromes after surgery for VSD, TOF, TGA, and HLHS. ND outcomes for HLHS are comparable to VSD, TOF and TGA in most domains. The number of children with impairments in at least 1 domain is increased compared to the general population for all groups. Differences do exist among diagnoses for unadjusted outcomes for some domains. However, because of the correlation of diagnosis with factors such as age at surgery, LOS, DHCA, and multiple operations; it is not possible to determine if cardiac diagnosis is causal in its prediction of outcomes or related secondary to these variables. 
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