Objective: To evaluate the outcome of patients with UH submitted to the various stages of the Fontan procedure.
Methods: We retrospectively reviewed the registers patients (pts) with UH managed at our centre (1998-2008), and analysed their clinical outcome according to the type of systemic ventricle (G1: 2 ventricles, G2: left, G3: right and G4: indeterminate).
Results: 82 pts. The average present age is 4,3 years-old (2 months -12 years) and 57% are male. The first, second and third stages of the Fontan procedure were done at an average age of 36 days, 11,4 months and 4,4 years, respectively.
G1 (10 pts):1 pt in the first, 3 in the second and 4 in the last stage of the Fontan procedure; two patients was lost. Most patients are doing well, with minimum or no symptoms.
G2 (26 pts):5 pts in the first, 9 in the second and 11 in the last stage of the Fontan procedure; one patient was lost. Most patients are also doing well.
G3 (45 pts):15 pts in the first, 12 in the second and 18 in the last stage of the Fontan procedure; two patients was lost. Seven patients have mild to moderate limitation of activity and/or have mild to moderate cyanosis. Deaths (9) occurred in this group at or after the first stage.
G4 (1 pts): at the second stage and is clinically well.
Conclusion: The overall results of the palliation of patients with univentricular heart are good.Worse prognosis in patients with right type UH.