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J Thorac Cardiovasc Surg 2008;135:91-97
© 2008 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Genetic factors are important determinants of neurodevelopmental outcome after repair of tetralogy of Fallot

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

Research presented at the American Heart Association Scientific Sessions 2004, Orlando, Florida, November 7-10, 2004.

Received for publication February 1, 2007; revisions received April 19, 2007; accepted for publication April 26, 2007.

^_* Address for reprints: Ilana Zeltser, MD, Division of Cardiology, The University of Texas at Southwestern Medical Center, Children’s Medical Center at Dallas, 1935 Motor Street, Dallas, TX 75235. (Email: ilana.zeltser{at}childrens.com).

Objective: Adverse neurodevelopmental sequelae are common in children with congenital heart defects. Tetralogy of Fallot is part of the clinical phenotype of many genetic syndromes. We evaluated the determinants of neurodevelopmental outcome in patients with tetralogy of Fallot.

Methods: We performed a subgroup analysis of children with tetralogy of Fallot undergoing complete repair before 6 months of age who were enrolled in a trial assessing apolipoprotein E genotype as a predictor of neurodevelopmental outcome. Assessment included genetic evaluation, neurologic examination, and the Bayley Scales of Infant Development-II, yielding the Mental Developmental Index and Psychomotor Developmental Index.

Results: Sixty children were tested at 1 year of age. A confirmed or suspected genetic syndrome was identified in 18.3%. The mean Mental Developmental Index was 89 ± 13, and the mean Psychomotor Developmental Index was 81 ± 17. Scores for the Mental Developmental Index (76 ± 13 vs 92 ± 11) and Psychomotor Developmental Index (63 ± 13 vs 85 ± 15) were significantly lower for patients with genetic syndromes. The presence of a genetic syndrome was a predictor of lower Mental Developmental Index and Psychomotor Developmental Index (P = .002 and P = .001). The presence of tetralogy of Fallot with pulmonary atresia and the apolipoprotein E 2 allele were predictive of a lower Mental Developmental Index (P = .001 and P = .035). No other preoperative or operative variables were predictive of worse neurodevelopmental outcome.

Conclusions: At 1 year of age after repair of tetralogy of Fallot, most patients had neurodevelopmental scores within the normal range. Genetic syndromes and the apolipoprotein E 2 allele were important risk factors for neurodevelopmental dysfunction and accounted for some interindividual differences in outcome.