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Right arrow Congenital - acyanotic

J Thorac Cardiovasc Surg 2006;131:792-798
© 2006 The American Association for Thoracic Surgery


Surgery for Congenital Heart Disease

Neurodevelopmental outcome after early repair of a ventricular septal defect with or without aortic arch obstruction

Jonathan R. Kaltman, MD a , * , Gail P. Jarvik, MD, PhD b , Judy Bernbaum, MD c , Gil Wernovsky, MD a , Marsha Gerdes, PhD d , Elaine Zackai, MD e , Robert R. Clancy, MD f , Susan C. Nicolson, MD g , Thomas L. Spray, MD h , J. William Gaynor, MD h

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

Received for publication October 10, 2005; revisions received December 6, 2005; accepted for publication December 12, 2005.

* Address for reprints: Jonathan Kaltman, MD, Division of Cardiology, The Children's Hospital of Philadelphia, 34th and Civic Center Blvd, Philadelphia, PA 19104. (Email: kaltman{at}email.chop.edu).

OBJECTIVES: Cross-sectional studies of intermediate-term survivors of infant cardiac surgery have revealed a high frequency of neurodevelopmental disabilities. Few data exist regarding neurodevelopmental outcome of infants undergoing surgical intervention for a ventricular septal defect. The purpose of this study was to evaluate the neurodevelopmental outcome at 1 year of age of children who had surgical repair in infancy of a ventricular septal defect or a ventricular septal defect with aortic arch obstruction.

METHODS: Children who underwent repair of a ventricular septal defect or single-stage repair of a ventricular septal defect with aortic arch obstruction at less than 6 months of age were assessed at 1 year of age by using the Bayley Scales of Infant Development II, which yields the Mental Development Index and the Psychomotor Development Index, both with an expected mean of 100 ± 15.

RESULTS: At 1 year, 55 patients (ventricular septal defect alone = 36; ventricular septal defect with aortic arch obstruction = 19) returned for evaluation. The mean Mental Development Index was 92.6 ± 11.7, with 3 (5%) patients scoring 70 or less. The mean Psychomotor Development Index was 86.1 ± 16.4, with 10 (18%) patients scoring 70 or less. Patients with a suspected or confirmed genetic syndrome had both a lower Mental Development Index score (P = .011) and a lower Psychomotor Development Index score (P = .001). Mental Development Index and Psychomotor Development Index were independent of anatomic (specifically aortic arch obstruction) and intraoperative factors (specifically deep hypothermic circulatory arrest).

CONCLUSIONS: Neurodevelopmental outcome at 1 year of age was within the normal limits for most patients who underwent repair of a ventricular septal defect or a ventricular septal defect with aortic arch obstruction during infancy.



Abbreviations and Acronyms DHCA = deep hypothermic circulatory arrest; d-TGA = d-transposition of the great arteries; MDI = Mental Development Index; PDI = Psychomotor Development Index; VSD = ventricular septal defect





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