Total cavopulmonary connections in children with a previous Norwood procedure

Background. Outcomes of the Fontan operation in children initially palliate d with the modified Norwood procedure are incompletely defined. Methods. From 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 pro cedures (hypoplastic left heart syndrome, n = 32; nonhypoplastic left heart syndrome, n = 13) underwent a modified Fontan operation. Preoperative feat ures included moderate/severe atrioventricular valve regurgitation (n = 5, 11%), reduced ventricular function on echocardiography in 11 patients, McGo on index 1.56 +/- 0.38, and pulmonary artery distortion in 18 patients (40% ). Results. A lateral tunnel (n = 16) or an extracardiac conduit (n = 29) conn ection with fenestration in 38 patients (84%) was used. Concomitant procedu res included pulmonary artery reconstruction (n = 24, 53%), atrioventricula r valve repair (n = 4, 9%) or replacement (n = 1). Before Fontan, 12 patien ts (27%) had an intervention to address neoaortic obstruction, and 7 patien ts required balloon dilation/stenting of the left (n = 5) or right pulmonar y artery (n = 5). Intraoperatively, left (n = 5) or right pulmonary artery (n = 1) stenting was performed in 5 patients (11%). On follow-up, 8 patient s required additional interventional procedures to address left pulmonary a rtery 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. Conclusions, In relatively high-risk patients, midterm results of the Fonta n operation for children initially palliated with the Norwood procedure wer e good. Combined interventional-surgical treatment algorithms can lead to i mproved outcomes. (Ann Thorac Surg 2001;71:1541-6) (C) 2001 by The Society of Thoracic Surgeons.