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J Thorac Cardiovasc Surg 2009;138:924-932
© 2009 The American Association for Thoracic Surgery

Congenital Heart Disease

Two-year neurodevelopmental outcomes of infants undergoing neonatal cardiac surgery for interrupted aortic arch: A descriptive analysis

^a Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
^b Department of Genetics, University of Alberta, Edmonton, Alberta, Canada
^c Department of Surgery, University of Alberta, Edmonton, Alberta, Canada
^d Pediatric Rehabilitation Outcomes, Evaluation and Research, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada
^e Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada
^f Post of Biostatistician, Research Methods Team, Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada
^g Western Canadian Complex Pediatric Therapies Follow-up Group (D. Moddemann, Winnipeg, Manitoba, Canada, A. Synnes, Vancouver, British Columbia, Canada, and J. Bodani, Regina, Saskatchewan, Canada)

Received for publication August 20, 2008; revisions received December 17, 2008; accepted for publication March 8, 2009.

^_* Address for reprints: Charlene M. T. Robertson, MD, Room 242, GlenEast, Glenrose Rehabilitation Hospital, 10230 111 Ave, Edmonton, Alberta, Canada, T5G 0B7. (Email: Charlene.Robertson{at}capitalhealth.ca).

Objective: This study determined neurodevelopmental outcomes of survivors of neonatal cardiac surgery for interrupted aortic arch through an interprovincial program and explored preoperative, intraoperative, and postoperative outcome predictors.

Methods: Children who underwent neonatal cardiac surgery for interrupted aortic arch at 6 weeks old or younger between 1996 and 2006 had a multidisciplinary neurodevelopmental assessment at 18 to 24 months old (mental and psychomotor developmental indices as mean ± SD and delay [score <70]). Survivor outcomes were compared by univariate and multivariate analyses and compared between children with and without chromosomal abnormality.

Results: Outcomes were available for all 26 survivors (mortality, 3.7%). Mental and psychomotor developmental indices were 75.8 ± 17.1 and 72.3 ± 16.9, respectively, with significantly lower scores for children with chromosomal abnormalities, which accounted for 29% of the variance in developmental indices. For the remaining 17 children without chromosomal abnormalities, mental and psychomotor developmental indices were 82.7 ± 14.5 and 79.1 ± 14.3, respectively, with deep hypothermic circulatory arrest time and Apgar score at 5 minutes contributing 46% of the variance in mental developmental index.

Conclusions: The neurodevelopmental indices of children who have undergone neonatal cardiac surgery for interrupted aortic arch are below normative values; those of children with chromosomal abnormalities are even lower. For children without a chromosomal abnormality, longer deep hypothermic circulatory arrest times and low Apgar scores predict lower mental developmental indices at 18 to 24 months of age.