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J Thorac Cardiovasc Surg 2009;137:139-145
© 2009 The American Association for Thoracic Surgery

Congenital Heart Disease

Prospective follow-up study of children with univentricular heart: Neurodevelopmental outcome at age 12 months

^a Division of Neurology, Department of Paediatric and Adolescent Medicine, Helsinki University Central Hospital, Helsinki, Finland
^b Division of Anesthesiology and Intensive Care Medicine, Department of Paediatric and Adolescent Medicine, Helsinki University Central Hospital, Helsinki, Finland
^c Division of Paediatric Cardiology, Department of Paediatric and Adolescent Medicine, Helsinki University Central Hospital, Helsinki, Finland
^d Division of Surgery, Department of Paediatric and Adolescent Medicine, Helsinki University Central Hospital, Helsinki, Finland

Received for publication January 6, 2008; revisions received April 1, 2008; accepted for publication June 15, 2008.

^_* Address for reprints: Anne Sarajuuri, MD, Department of Child Neurology, Department of Paediatric and Adolescent Medicine, Helsinki University Central Hospital, Lastenlinnantie 2, 00029 HUS, Finland. (Email: Anne.Sarajuuri{at}hus.fi).

Objectives: Despite recent advances in the treatment of children with univentricular heart, their neurodevelopmental outcome remains a major concern.

Methods: This prospective follow-up study evaluated the neurodevelopmental outcome of 23 patients with hypoplastic left heart syndrome, 14 with other forms of univentricular heart, and 46 healthy control subjects at a median age of 12.2 months. The Griffiths Developmental Scale and Alberta Infant Motor Scale served for developmental evaluation.

Results: The mean Griffiths developmental quotient of children with hypoplastic left heart syndrome was significantly less (91.6) than that of control children (106.8, P < .001). Patients with univentricular heart scored significantly lower than control subjects only in the gross motor domain (P = .001) but not in overall development (100.6). Alberta Infant Motor Scale scores were significantly lower in children with hypoplastic left heart syndrome (37.5, P < .001) and univentricular heart (43.5, P = .011) than in control subjects (53.3). In linear regression a diagnosis of hypoplastic left heart syndrome (P = .016), a clinical history of seizure (P = .002), and the highest plasma lactate level after the bidirectional Glenn operation (P = .045) were significantly associated with the developmental quotient.

Conclusions: At age 1 year, the level of development of children with univentricular heart was significantly lower than for control subjects only in motor skills, whereas children with hypoplastic left heart syndrome had a more widespread developmental delay. The diagnosis, a clinical seizure history, and increased plasma lactate levels after the bidirectional Glenn operation emerged as risk factors.

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				A. Eerola, E. Jokinen, H. Sairanen, and J. Pihkala During treatment protocol for univentricular heart serum levels of natriuretic peptides decrease, Eur J Cardiothorac Surg, December 1, 2010; 38(6): 735 - 740. [Abstract] [Full Text] [PDF]