Total cavopulmonary anastomosis (Fontan) in children with Down's syndrome.
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.
Campbell
R M
RM
Department of Critical Care Medicine, The Hospital for Sick Children, University of Toronto, Ontario, Canada.
Adatia
I
I
Gow
R M
RM
Webb
G D
GD
Williams
W G
WG
Freedom
R M
RM
D004314:Down Syndrome / Q000150:complications*; D018729:Fontan Procedure / Q000401:mortality; D006330:Heart Defects, Congenital / Q000601:surgery*; D006343:Heart Septal Defects / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011183:Postoperative Complications; D016896:Treatment Outcome