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- A Azakie, B W McCrindle, L N Benson, G S Van Arsdell, J L Russell, J G Coles, D Nykanen, R M Freedom, and W G Williams.
- Department of Surgery, The Hospital For Sick Children, University of Toronto School of Medicine, Ontario, Canada.
- Ann. Thorac. Surg. 2001 May 1;71(5):1541-6.
BackgroundOutcomes of the Fontan operation in children initially palliated with the modified Norwood procedure are incompletely defined.MethodsFrom August 1993 to January 2000, 45 patients (mean age 2.6 +/- 1.1 years, weight 12.7 +/- 2.8 kg) who were palliated with staged Norwood procedures (hypoplastic left heart syndrome, n = 32; nonhypoplastic left heart syndrome, n = 13) underwent a modified Fontan operation. Preoperative features included moderate/severe atrioventricular valve regurgitation (n = 5, 11%), reduced ventricular function on echocardiography in 11 patients, McGoon index 1.56 +/- 0.38, and pulmonary artery distortion in 18 patients (40%).ResultsA lateral tunnel (n = 16) or an extracardiac conduit (n = 29) connection with fenestration in 38 patients (84%) was used. Concomitant procedures included pulmonary artery reconstruction (n = 24, 53%), atrioventricular valve repair (n = 4, 9%) or replacement (n = 1). Before Fontan, 12 patients (27%) had an intervention to address neoaortic obstruction, and 7 patients required balloon dilation/stenting of the left (n = 5) or right pulmonary artery (n = 5). Intraoperatively, left (n = 5) or right pulmonary artery (n = 1) stenting was performed in 5 patients (11%). On follow-up, 8 patients required additional interventional procedures to address left pulmonary artery narrowing (n = 5), or venous (n = 5) or arteriopulmonary collaterals (n = 1). Perioperative mortality was 4.4% (n = 2). There were 2 late deaths at a mean follow-up of 39 +/- 20 months.ConclusionsIn relatively high-risk patients, midterm results of the Fontan operation for children initially palliated with the Norwood procedure were good. Combined interventional-surgical treatment algorithms can lead to improved outcomes.
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