(Circulation. 2000;102:883.)
© 2000 American Heart Association, Inc.
Clinical Investigation and Reports |
Cognitive Development After the Fontan Operation
Presented in part at the 68th Scientific Sessions of the American Heart Association, Anaheim, Calif, November 13–16, 1995, and published in abstract form (Circulation. 1995;92[suppl I]:I-121).
From the Departments of Cardiology (G.W., K.G., T.L.G., A.Z.W., J.B., J.W.N.), Neuroepidemiology (K.M.S., D.C.B.), Neurology (A.J.D.), and Cardiac Surgery (R.A.J., J.E.M.), Children’s Hospital and the Departments of Pediatrics (G.W., K.G., T.L.G., A.J.D., J.W.N.) and Surgery (R.A.J., J.E.M.), Harvard Medical School, Boston, Mass.
Background—Patients with a single ventricle have multiple risk factors for central nervous system injury, both before and after the Fontan procedure.
Methods and Results—A geographically selected cohort was invited to undergo standardized testing, including age-appropriate measures of intelligence quotient (IQ) and achievement tests. Historical information was obtained by chart review and patient questionnaires. Of the 222 eligible patients, 133 (59.9%) participated. Median age at testing was 11.1 years (range, 3.7 to 41.0 years), 6.0 years (range, 1.6 to 19.6 years) after surgery. Mean full-scale IQ was 95.7±17.4 (P<0.006 versus normal); 10 patients (7.8%) had full-scale IQ scores <70 (P=0.001). After adjustment for socioeconomic status, lower IQ was associated with the use of circulatory arrest before the Fontan operation (P=0.002), the anatomic diagnoses of hypoplastic left heart syndrome (P<0.001) and "other complex" (P=0.05), and prior placement of a pulmonary artery band (P=0.04). Mean composite achievement score was 91.6±15.4 (P<0.001 versus normal); 14 patients (10.8%) scored <70 (P<0.001). After adjustment for socioeconomic status, independent risk factors for low achievement scores included the diagnoses of hypoplastic left heart syndrome (P=0.004) and "other complex" (P=0.003) or prior use of circulatory arrest (P=0.03), as well as a reoperation with cardiopulmonary bypass within 30 days of the Fontan (P=0.01).
Conclusions—Most individual patients palliated with the Fontan procedure in the 1970s and 1980s have cognitive outcome and academic function within the normal range, but the performance of the cohort is lower than that of the general population.
Key Words: Fontan procedure • intelligence • risk factors • cardiopulmonary bypass
This article has been cited by other articles:
 |
|
 |
|
  C. A. Joynt, C. M.T. Robertson, P.-Y. Cheung, A. Nettel-Aguirre, A. R. Joffe, R. S. Sauve, W. S.G. Biggs, N. J. Leonard, D. B. Ross, I. M. Rebeyka, et al. Two-year neurodevelopmental outcomes of infants undergoing neonatal cardiac surgery for interrupted aortic arch: A descriptive analysis J. Thorac. Cardiovasc. Surg., October 1, 2009; 138(4): 924 - 932. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. T. Mahle, J. W. Newburger, G. P. Matherne, F. C. Smith, T. R. Hoke, R. Koppel, S. S. Gidding, R. H. Beekman III, S. D. Grosse, and on behalf of the American Heart Association Congen Role of Pulse Oximetry in Examining Newborns for Congenital Heart Disease: A Scientific Statement From the American Heart Association and American Academy of Pediatrics Circulation, August 4, 2009; 120(5): 447 - 458. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. T. Mahle, J. W. Newburger, G. P. Matherne, F. C. Smith, T. R. Hoke, R. Koppel, S. S. Gidding, R. H. Beekman III, S. D. Grosse, and on behalf of the American Heart Association Congen Role of Pulse Oximetry in Examining Newborns for Congenital Heart Disease: A Scientific Statement from the AHA and AAP Pediatrics, August 1, 2009; 124(2): 823 - 836. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Sarajuuri, T. Lonnqvist, L. Mildh, I. Rajantie, M. Eronen, I. Mattila, and E. Jokinen Prospective follow-up study of children with univentricular heart: Neurodevelopmental outcome at age 12 months J. Thorac. Cardiovasc. Surg., January 1, 2009; 137(1): 139 - 145. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons J. Am. Coll. Cardiol., December 2, 2008; 52(23): e143 - e263. [Full Text] [PDF]
|
|
|
|
|
|
C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease) Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons J. Am. Coll. Cardiol., December 2, 2008; 52(23): 1890 - 1947. [Full Text] [PDF]
|
|
|
|
|
|
C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons Circulation, December 2, 2008; 118(23): e714 - e833. [Full Text] [PDF]
|
|
|
|
|
|
C. A. Warnes, R. G. Williams, T. M. Bashore, J. S. Child, H. M. Connolly, J. A. Dearani, P. del Nido, J. W. Fasules, T. P. Graham Jr, Z. M. Hijazi, et al. ACC/AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease): Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons Circulation, December 2, 2008; 118(23): 2395 - 2451. [Full Text] [PDF]
|
|
|
|
 |
|
 R. van der Rijken, G. Hulstijn-Dirkmaat, F. Kraaimaat, L. Nabuurs-Kohrman, A. Nijveld, B. Maassen, and O. Daniels Open-heart surgery at school age does not affect neurocognitive functioning Eur. Heart J., November 1, 2008; 29(21): 2681 - 2688. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. G. Ohye, J. W. Gaynor, N. S. Ghanayem, C. S. Goldberg, P. C. Laussen, P. C. Frommelt, J. W. Newburger, G. D. Pearson, S. Tabbutt, G. Wernovsky, et al. Design and rationale of a randomized trial comparing the Blalock-Taussig and right ventricle-pulmonary artery shunts in the Norwood procedure. J. Thorac. Cardiovasc. Surg., October 1, 2008; 136(4): 968 - 975. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. Atallah, I. A. Dinu, A. R. Joffe, C. M.T. Robertson, R. S. Sauve, J. D. Dyck, D. B. Ross, I. M. Rebeyka, and the Western Canadian Complex Pediatric Therapies F Two-Year Survival and Mental and Psychomotor Outcomes After the Norwood Procedure: An Analysis of the Modified Blalock-Taussig Shunt and Right Ventricle-to-Pulmonary Artery Shunt Surgical Eras Circulation, September 30, 2008; 118(14): 1410 - 1418. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Wernovsky The Paradigm Shift Toward Surgical Intervention for Neonates With Hypoplastic Left Heart Syndrome Arch Pediatr Adolesc Med, September 1, 2008; 162(9): 849 - 854. [Full Text] [PDF]
|
|
|
|
|
|
A. J. Shillingford, M. M. Glanzman, R. F. Ittenbach, R. R. Clancy, J. W. Gaynor, and G. Wernovsky Inattention, Hyperactivity, and School Performance in a Population of School-Age Children With Complex Congenital Heart Disease Pediatrics, April 1, 2008; 121(4): e759 - e767. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Wernovsky Improving neurologic and quality-of-life outcomes in children with congenital heart disease: Past, present, and future J. Thorac. Cardiovasc. Surg., February 1, 2008; 135(2): 240 - 242. [Full Text] [PDF]
|
|
|
|
|
|
J. Li, G. Zhang, H. Holtby, A.-M. Guerguerian, S. Cai, T. Humpl, C. A. Caldarone, A. N. Redington, and G. S. Van Arsdell The influence of systemic hemodynamics and oxygen transport on cerebral oxygen saturation in neonates after the Norwood procedure J. Thorac. Cardiovasc. Surg., January 1, 2008; 135(1): 83 - 90. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. E. Creighton, C. M.T. Robertson, R. S. Sauve, D. M. Moddemann, G. Y. Alton, A. Nettel-Aguirre, D. B. Ross, I. M. Rebeyka, and and the Western Canadian Complex Pediatric Therapi Neurocognitive, Functional, and Health Outcomes at 5 Years of Age for Children After Complex Cardiac Surgery at 6 Weeks of Age or Younger Pediatrics, September 1, 2007; 120(3): e478 - e486. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. G. McBride, P. M. Kirshbom, J. W. Gaynor, R. F. Ittenbach, G. Wernovsky, R. R. Clancy, T. B. Flynn, D. M. Hartman, T. L. Spray, R. E. Tanel, et al. Late cardiopulmonary and musculoskeletal exercise performance after repair for total anomalous pulmonary venous connection during infancy J. Thorac. Cardiovasc. Surg., June 1, 2007; 133(6): 1533 - 1539. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 P. A. Karsdorp, W. Everaerd, M. Kindt, and B. J.M. Mulder Psychological and Cognitive Functioning in Children and Adolescents with Congenital Heart Disease: A Meta-Analysis J. Pediatr. Psychol., June 1, 2007; 32(5): 527 - 541. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. S. Goldberg, E. L. Bove, E. J. Devaney, E. Mollen, E. Schwartz, S. Tindall, C. Nowak, J. Charpie, M. B. Brown, T. J. Kulik, et al. A randomized clinical trial of regional cerebral perfusion versus deep hypothermic circulatory arrest: Outcomes for infants with functional single ventricle J. Thorac. Cardiovasc. Surg., April 1, 2007; 133(4): 880 - 887. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. Y. Alton, C. M.T. Robertson, R. Sauve, A. Divekar, A. Nettel-Aguirre, S. Selzer, A. R. Joffe, I. M. Rebeyka, D. B. Ross, and Western Canadian Complex Pediatric Therapies Proje Early childhood health, growth, and neurodevelopmental outcomes after complete repair of total anomalous pulmonary venous connection at 6 weeks or younger J. Thorac. Cardiovasc. Surg., April 1, 2007; 133(4): 905 - 911. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. J. Visconti, D. Rimmer, K. Gauvreau, P. del Nido, J. E. Mayer Jr, I. Hagino, and F. A. Pigula Regional Low-Flow Perfusion Versus Circulatory Arrest in Neonates: One-Year Neurodevelopmental Outcome Ann. Thorac. Surg., December 1, 2006; 82(6): 2207 - 2213. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Gauvreau Hypothesis Testing: Proportions Circulation, October 3, 2006; 114(14): 1545 - 1548. [Full Text] [PDF]
|
|
|
|
 |
|
 M. A. Padula and A. M. Ades Neurodevelopmental Implications of Congenital Heart Disease NeoReviews, July 1, 2006; 7(7): e363 - e369. [Full Text] [PDF]
|
|
|
|
|
|
B. W. McCrindle, R. V. Williams, P. D. Mitchell, D. T. Hsu, S. M. Paridon, A. M. Atz, J. S. Li, J. W. Newburger, and for the Pediatric Heart Network Investigators Relationship of Patient and Medical Characteristics to Health Status in Children and Adolescents After the Fontan Procedure Circulation, February 28, 2006; 113(8): 1123 - 1129. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. E. Mitchell, R. F. Ittenbach, J. W. Gaynor, G. Wernovsky, S. Nicolson, and T. L. Spray Intermediate outcomes after the Fontan procedure in the current era J. Thorac. Cardiovasc. Surg., January 1, 2006; 131(1): 172 - 180. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. L. Dent, J. P. Spaeth, B. V. Jones, S. M. Schwartz, T. A. Glauser, B. Hallinan, J. M. Pearl, P. R. Khoury, and C. D. Kurth Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion J. Thorac. Cardiovasc. Surg., January 1, 2006; 131(1): 190 - 197. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. H. Schultz, G. P. Jarvik, G. Wernovsky, J. Bernbaum, R. R. Clancy, J. A. D'Agostino, M. Gerdes, D. McDonald-McGinn, S. C. Nicolson, T. L. Spray, et al. Effect of congenital heart disease on neurodevelopmental outcomes within multiple-gestation births J. Thorac. Cardiovasc. Surg., December 1, 2005; 130(6): 1511 - 1516. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. L. Dent, J. P. Spaeth, B. V. Jones, S. M. Schwartz, T. A. Glauser, B. Hallinan, J. M. Pearl, P. R. Khoury, and C. D. Kurth Brain magnetic resonance imaging abnormalities after the Norwood procedure using regional cerebral perfusion J. Thorac. Cardiovasc. Surg., December 1, 2005; 130(6): 1523 - 1530. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R Kaulitz and M Hofbeck Current treatment and prognosis in children with functionally univentricular hearts Arch. Dis. Child., July 1, 2005; 90(7): 757 - 762. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. A. Ghaferi and G. M. Hutchins Progression of liver pathology in patients undergoing the Fontan procedure: Chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma J. Thorac. Cardiovasc. Surg., June 1, 2005; 129(6): 1348 - 1352. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. J. Licht, J. Wang, D. W. Silvestre, S. C. Nicolson, L. M. Montenegro, G. Wernovsky, S. Tabbutt, S. M. Durning, D. M. Shera, J. W. Gaynor, et al. Preoperative cerebral blood flow is diminished in neonates with severe congenital heart defects J. Thorac. Cardiovasc. Surg., December 1, 2004; 128(6): 841 - 849. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. A. Connor, R. R. Arons, M. Figueroa, and K. M. Gebbie Clinical Outcomes and Secondary Diagnoses for Infants Born With Hypoplastic Left Heart Syndrome Pediatrics, August 1, 2004; 114(2): e160 - e165. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. W. Gaynor, M. Gerdes, E. H. Zackai, J. Bernbaum, G. Wernovsky, R. R. Clancy, M. F. Newman, A. M. Saunders, P. J. Heagerty, J. A. D'Agostino, et al. Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery J. Thorac. Cardiovasc. Surg., December 1, 2003; 126(6): 1736 - 1745. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. L. Culbert, D. A. Ashburn, G. Cullen-Dean, J. A. Joseph, W. G. Williams, E. H. Blackstone, and B. W. McCrindle Quality of Life of Children After Repair of Transposition of the Great Arteries Circulation, August 19, 2003; 108(7): 857 - 862. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. J. Griffin, T. D. Elkin, and C. J. Smith Academic Outcomes in Children with Congenital Heart Disease Clinical Pediatrics, June 1, 2003; 42(5): 401 - 409. [Abstract] [PDF]
|
|
|
|
 |
|
 H Dittrich, C Buhrer, I Grimmer, S Dittrich, H Abdul-Khaliq, and P E Lange Neurodevelopment at 1 year of age in infants with congenital heart disease Heart, April 1, 2003; 89(4): 436 - 441. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
P. M. Kirshbom, R. J. Myung, J. W. Gaynor, R. F. Ittenbach, S. M. Paridon, W. M. DeCampli, T. R. Karl, and T. L. Spray Preoperative pulmonary venous obstruction affects long-term outcome for survivors of total anomalous pulmonary venous connection repair Ann. Thorac. Surg., November 1, 2002; 74(5): 1616 - 1620. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. M. Forbess, K. J. Visconti, C. Hancock-Friesen, R. C. Howe, D. C. Bellinger, and R. A. Jonas Neurodevelopmental Outcome After Congenital Heart Surgery: Results From an Institutional Registry Circulation, September 24, 2002; 106(12_suppl_1): I-95 - I-102. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A P SALMON Hypoplastic left heart syndrome---outcome and management Arch. Dis. Child., December 1, 2001; 85(6): 450 - 451. [Full Text] [PDF]
|
|
|
|
|
|
J. M. Forbess, K. J. Visconti, D. C. Bellinger, and R. A. Jonas Neurodevelopmental Outcomes in Children After the Fontan Operation Circulation, September 18, 2001; 104 (2009): I-127 - I-132. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. K. Perloff and C. A. Warnes Challenges Posed by Adults With Repaired Congenital Heart Disease Circulation, May 29, 2001; 103(21): 2637 - 2643. [Full Text] [PDF]
|
|