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(Circulation. 2001;104:I-127.)
© 2001 American Heart Association, Inc.

Surgery for Congenital Heart Disease

Neurodevelopmental Outcomes in Children After the Fontan Operation

Joseph M. Forbess, MD; Karen J. Visconti, PhD; David C. Bellinger, PhD, MSc; Richard A. Jonas, MD

From the Departments of Cardiovascular Surgery (J.M.F., K.J.V., R.A.J.) and Neuroepidemiology (D.C.B.), Children’s Hospital, and the Department of Surgery (J.M.F., R.A.J.), Harvard Medical School, Boston, Mass.

Reprint requests to Joseph M. Forbess, MD, Department of Cardiovascular Surgery, Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. E-mail forbess{at}cardio.tch.harvard.edu

Background— Previous studies of patients after the Fontan operation have reported IQ scores lower than population norms. In the past decade, changes have occurred both in surgical methods used and in the patient population undergoing Fontan palliation. The present study examined the impact of these changes on neurodevelopmental outcomes after Fontan.

Methods and Results— Neuropsychological tests were administered to 27 five-year-old children after Fontan. Mean age at repair was 2 years 4 months. The present sample was compared with an earlier Fontan group (EFG) of 133 patients who underwent surgery in the 1970s and 1980s. Mean age at repair for the EFG was 7 years 3 months. Compared with EFG, the present study sample was younger at Fontan (P=0.0001) and more likely to have undergone a Norwood procedure (P=0.02), a pre-Fontan bidirectional cavopulmonary anastomosis (P<0.001), and Fontan fenestration (P=0.001). Although mean full-scale, verbal, and performance IQ scores were within 1 SD (15 points) of the population mean of 100 (93±16, 95±15, and 91±17, respectively), mean full-scale and performance IQ scores were significantly lower than this population mean (P=0.03 and P=0.01, respectively).

Conclusions— Compared with a historical cohort of Fontan patients from this institution, a staged approach to Fontan earlier in life is not detrimental to neurodevelopmental outcome. Neurodevelopmental outcomes in children after Fontan are in the normal range, but performance remains lower than the general population.

Key Words: Fontan procedure • follow-up studies • intelligence quotient