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J Thorac Cardiovasc Surg 2005;129:1091-1097
© 2005 The American Association for Thoracic Surgery


Surgery for Congenital Heart Disease

Late neurodevelopmental outcome after repair of total anomalous pulmonary venous connection

Paul M. Kirshbom, MDa,*, Thomas B. Flynn, PhDd, Robert R. Clancy, MDe, Richard F. Ittenbach, PhDf, Diane M. Hartman, RNb, Stephen M. Paridon, MDc, Gil Wernovsky, MDc, Thomas L. Spray, MDb, J. William Gaynor, MDb

a Division of Cardiothoracic Surgery, Emory University, Atlanta, Ga
b Division of Cardiothoracic Surgery, The Childrens Hospital of Philadelphia, Philadelphia, Pa
c Division of Cardiology, The Childrens Hospital of Philadelphia, Philadelphia, Pa
d Division of Psychology, The Childrens Hospital of Philadelphia, Philadelphia, Pa
e Division of Neurology, The Childrens Hospital of Philadelphia, Philadelphia, Pa
f Division of Biostatistics and Epidemiology, The Childrens Hospital of Philadelphia, Philadelphia, Pa.

Received for publication June 8, 2004; revisions received August 10, 2004; accepted for publication August 16, 2004.

* Address for reprints: Paul M. Kirshbom, MD, 1365 Clifton Rd, Suite A2100, Cardiothoracic Surgery, Emory Clinic Bldg. A, At-lanta, GA 30322 (E-mail: paul_kirshbom{at}emoryhealthcare.org).

OBJECTIVE: We sought to define the neurodevelopmental status of school-aged survivors of total anomalous pulmonary venous connection repaired during infancy.

METHODS: All school-aged survivors of total anomalous pulmonary venous connection repair performed at a single institution were eligible. Thirty children returned for neurologic examination and neurodevelopmental testing.

RESULTS: The median age at total anomalous pulmonary venous connection repair was 16 days (range, 1–141 days), and age at testing was 11 years (range, 6–19 years). Pulmonary venous return was supracardiac in 14 patients, infracardiac in 12 patients, cardiac in 3 patients, and mixed in 1 patient. Preoperative obstructed total anomalous pulmonary venous connection was present in 6 patients. Circulatory arrest was used in all repairs, with a median duration of 35 minutes (range, 17–55 minutes). At follow-up, microcephaly (head circumference <5%) was present in 28%. Neuromuscular examination was suspect or abnormal in 27%. Mean Full-scale IQ (95.3 ± 18.5) and Verbal IQ (98.6 ± 20.2) were not different from population norms, but Performance IQ (92.3 ± 16.9) was significantly lower than population norms (P = .02). Fine motor skills and visual-motor coordination were significantly impaired (P < .01 for Grooved Pegboard and Test of Visual-Motor Integration). Patients with total anomalous pulmonary venous connection also had difficulty with tests of attention (Test of Everyday Attention for Children, P < .01), but results of tests of memory function were not significantly different from population norms.

CONCLUSIONS: School-aged survivors of infant total anomalous pulmonary venous connection repair exhibit a significant incidence of neurodevelopmental difficulties. Fine motor function, visual-motor integration, and attention are the most commonly affected domains. Evaluation of these children is indicated to identify those who are at risk for learning disabilities and who could benefit from early intervention.





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