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J Thorac Cardiovasc Surg 2007;133:1344-1353
© 2007 The American Association for Thoracic Surgery


Surgery for Congenital Heart Disease

Patient characteristics are important determinants of neurodevelopmental outcome at one year of age after neonatal and infant cardiac surgery

J. William Gaynor, MDa,*, Gil Wernovsky, MDb, Gail P. Jarvik, MD, PhDc, Judy Bernbaum, MDd, Marsha Gerdes, PhDe, Elaine Zackai, MDf, Alex S. Nord, BAc, Robert R. Clancy, MDg, Susan C. Nicolson, MDh, Thomas L. Spray, MDa

a Division of Cardiothoracic Surgery, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa
b Division of Pediatric Cardiology, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa
d Division of General Pediatrics, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa
e Division of Psychology, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa
f Division of Genetics, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa
g Division of Neurology, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa
h Division of Cardiothoracic Anesthesiology, The Cardiac Center at The Children’s Hospital of Philadelphia, Philadelphia, Pa
c Department of Medicine (Medical Genetics), University of Washington, Seattle, Wash.

Read at the Eighty-sixth Annual Meeting of The American Association for Thoracic Surgery, Philadelphia, Pa, April 29-May 3, 2006.

Received for publication May 15, 2006; revisions received October 12, 2006; accepted for publication October 25, 2006.

* Address for reprints: J. William Gaynor, MD, Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, 34th and Civic Center Blvd, Suite 8527, Philadelphia, PA 19104. (Email: gaynor{at}email.chop.edu).

Objective: Many studies of neurodevelopmental outcomes after neonatal and infant cardiac surgery have focused on potentially modifiable risk factors for adverse outcomes, primarily intraoperative management strategies and the use of deep hypothermic circulatory arrest. There is increasing evidence that patient-specific factors are more important determinants of outcome.

Methods: We investigated predictors of neurodevelopmental outcomes at 1 year of age after neonatal and infant cardiac surgery in a subgroup of infants enrolled in a prospective study of apolipoprotein E (APOE) genotype and neurodevelopmental outcome. Children with a variety of 2-ventricle cardiac defects repaired with only 1 operation with cardiopulmonary bypass and no more than 1 episode of deep hypothermic circulatory arrest were included. Neurodevelopmental outcomes at 1 year of age included the Bayley Scales of Infant Development-II, which yield 2 indices, the Mental Developmental Index and the Psychomotor Developmental Index.

Results: Two hundred forty-seven infants underwent surgical repair between October 1998 and April 2003 with 1 hospital death and 3 deaths before 1 year of age. Neurodevelopmental evaluation was performed in 188 (77%) of 243 survivors, including 56 patients with tetralogy of Fallot, 39 with transposition of the great arteries with intact ventricular septum, 34 with ventricular septal defects, and 59 with other defects. The median age at operation was 56 days (1–186 days), including 72 (38%) neonates. Confirmed or suspected genetic syndromes were present in 59 (31%) of 188 infants. Deep hypothermic circulatory arrest was used in 67 (35%) infants with a median duration of 34 minutes (1-80 minutes). For the entire cohort, the mean Mental Developmental Index was 90.6 ± 14.9 and the mean Psychomotor Developmental Index was 81.6 ± 17.2. For patients without genetic syndromes, the mean Mental Developmental Index was 93.7 ± 13.6 and the mean Psychomotor Developmental Index was 85.1 ± 14.6. For the entire cohort, predictors of lower scores for both the Mental Developmental Index and Psychomotor Developmental Index were presence of a confirmed or suspected genetic syndrome, lower birth weight, and presence of the APOE {epsilon}2 allele (all P < .04). Black race was associated with higher scores on the Psychomotor Developmental Index (P = .018). Lower nasopharyngeal temperature during cardiopulmonary bypass was associated with a lower score on the Psychomotor Developmental Index (P = .03) and was the only intraoperative factor that was a significant predictor of either the Mental or Psychomotor Developmental Index.

Conclusions: The strongest predictors of a worse neurodevelopmental outcome at 1 year of age were patient-specific factors including presence of a genetic syndrome, low birth weight, and presence of the APOE {epsilon}2 allele. Patient-specific factors eclipsed the use and duration of deep hypothermic circulatory arrest as predictors of worse neurodevelopmental outcomes.



Abbreviations and Acronyms APOE = apolipoprotein E; BCAS = Boston Circulatory Arrest Study; CHD = congenital heart defect; CPB = cardiopulmonary bypass; DHCA = deep hypothermic circulatory arrest; MDI = Mental Developmental Index; NP = nasopharyngeal; PDI = Psychomotor Developmental Index; TGA = transposition of the great arteries; TOF = tetralogy of Fallot; VSD = ventricular septal defect



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