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J Thorac Cardiovasc Surg 2007;133:1524-1532
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Neurodevelopmental and neuroradiologic outcomes in patients with univentricular heart aged 5 to 7 years: Related risk factor analysis

Anne Sarajuuri, MDa,*, Eero Jokinen, MD, PhDb, Riina Puosi, MSca, Marianne Eronen, MD, PhDb, Leena Mildh, MDc, Ilkka Mattila, MD, PhDd, Leena Valanne, MD, PhDe, Tuula Lönnqvist, MD, PhDa

a Department of Neurology, Helsinki University Central Hospital, Helsinki, Finland
b Department of Cardiology, Helsinki University Central Hospital, Helsinki, Finland
c Department of Anesthesiology and Intensive Care Medicine, Helsinki University Central Hospital, Helsinki, Finland
d Department of Surgery, Helsinki University Central Hospital, Helsinki, Finland
e Hospital for Children and Adolescents, and the Helsinki Medical Imaging Center, Helsinki University Central Hospital, Helsinki, Finland.

Received for publication September 18, 2006; revisions received November 24, 2006; accepted for publication December 13, 2006.

* Address for reprints: Anne Maria Hannele Sarajuuri, MD, Helsinki University Central Hospital, Hospital for Children and Adolescents, Department of Child Neurology, Lastenlinnante 2, 00029 HUS, Helsinki, Finland.

Objective: Despite improved survival and neurodevelopmental outcome, children with hypoplastic left heart syndrome and other forms of univentricular heart remain at increased risk for cognitive, motor, and other neurologic deficits.

Methods: We examined 27 children with hypoplastic left heart syndrome or other forms of univentricular heart at a median age of 5.70 years (range 4.99–7.51 years) and performed brain computed tomography or magnetic resonance imaging on 20. Possible risk factors were correlated with outcome.

Results: Mean full-scale IQ among patients with hypoplastic left heart syndrome was 86.7; that among patients with other forms of univentricular heart was 89.1, with both differing significantly from the expected population mean (P = .015 and P = .029, respectively). Cerebral palsy was diagnosed in 1 of 7 patients with hypoplastic left heart syndrome and 2 of 20 with other forms of univentricular heart. Brain computed tomography or magnetic resonance imaging revealed ischemic changes and infarcts or atrophy in 5 of 8 patients who had undergone the Norwood procedure and in 2 of 12 of those who had not (P = .062). Abnormal computed tomographic findings correlated significantly with lower full-scale IQ (P = .045) and verbal IQ (P = .02). In the multiple linear regression model, diuresis the third day after the primary operation and cardiopulmonary bypass time in the bidirectional Glenn operation correlated significantly with the primary outcome of full-scale IQ.

Conclusion: In children with univentricular heart, intellectual and neurologic deficits are common. Perioperative and postoperative risk factors related to the primary phase and bidirectional Glenn operation contribute to these deficits.

Abbreviations and Acronyms BDG = bidirectional Glenn; CPB = cardiopulmonary bypass; CT = computed tomography; DHCA = deep hypothermic cardiac arrest; FIQ = full-scale IQ; HLHS = hypoplastic left heart syndrome; MRI = magnetic resonance imaging; PIQ = performance IQ; TCPC = total cavopulmonary connection; UVH = univentricular heart; VIQ = verbal IQ




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