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Ann Thorac Surg 1998;66:523-526
© 1998 The Society of Thoracic Surgeons

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Original articles: cardiovascular

Total cavopulmonary anastomosis (Fontan) in children with Down’s syndrome

^a Division of Cardiology, The Hospital for Sick Children, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada
^b Division of Cardiovascular Surgery, The Hospital for Sick Children, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada
^c Department of Critical Care Medicine, The Hospital for Sick Children, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada
^d Department of Pediatrics, The Hospital for Sick Children, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada
^e Department of Surgery, The Hospital for Sick Children, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada
^f Toronto Congenital Cardiac Centre for Adults, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada

Accepted for publication March 14, 1998.

Address reprint requests to Dr Adatia, Critical Care Medicine and Cardiology, The Hospital for Sick Children, 555 University Ave, Toronto, Ont, Canada M5G 1X8
e-mail: (iadatia{at}sickkids.on.ca)

Background. There is a paucity of information to guide the management of the child with Down’s syndrome and congenital heart disease in whom biventricular repair is precluded.

Methods. Through the cardiology and cardiovascular surgery databases of The Hospital for Sick Children and Toronto Congenital Cardiac Centre for Adults, we identified patients with trisomy 21 and ventricular hypoplasia who had undergone a Fontan procedure (or modification).

Results. Of 533 patients who had undergone a Fontan operation between 1976 and 1997, 4 had trisomy 21. All 4 patients had unbalanced complete atrioventricular septal defect with right ventricular hypoplasia in 3 and left ventricular hypoplasia in 1. Three patients survived, and 1 died of endocarditis. The 3 survivors have done well in the short term and medium term without complications related to the pulmonary vasculature.

Conclusions. We suggest that in appropriately selected patients with trisomy 21 and ventricular hypoplasia who are unsuitable for two or one and a half ventricle repair, the Fontan procedure is not contraindicated and provides short-term and medium-term benefit.

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