Background. The historic outcome of the Fontan procedure in children with single ventricle and heterotaxy syndrome has been poor, and in the current era it has been incompletely described. Methods. From January 1993 through April 2000, 30 patients (age range, 5.3 ± 3.6 years) had total cavopulmonary connection for single ventricle and isomerism and heterotaxy syndrome. Right atrial isomerism and heterotaxy (n = 19) or left atrial isomerism and heterotaxy (n = 11) were associated with a morphologic right ventricle (n = 26), a common atrioventricular valve (n = 25), pulmonary atresia (n = 9) or stenosis (n = 17), anomalous pulmonary venous drainage (partial, n = 7; total, n = 11; obstructed, n = 4), and interrupted inferior vena cava (n = 13). A previous systemic to pulmonary artery shunt had been performed in 21 patients, and 3 patients had previous pulmonary artery banding. The Fontan procedure was staged in all but 3 patients at a median age of 12 months (range, 3 to 114 months). Before the Fontan procedure, atrial arrhythmia was present in 7 patients (23%). Results. An extracardiac conduit was constructed in 22 patients (median diameter, 20 mm; range, 16 to 27 mm), and a lateral tunnel was constructed in 4 patients. Associated procedures included pulmonary artery augmentation (n = 17), repair of anomalous pulmonary venous drainage (n = 7), and repair of a regurgitant atrioventricular valve (n = 2). The mean cardiopulmonary bypass time was 134 ± 57 minutes. Cardioplegic cardiac arrest was used in 12 patients for a mean duration of 62 ± 28 minutes. There were four hospital deaths (13%), three that occurred in children having concomitant repair of anomalous pulmonary venous drainage (previously undetected in one). Thirteen children (41%) developed early postoperative atrial arrhythmias, 11 of the 13 children required temporary pacing. There was one late death. Follow-up (3.4 ± 2.7 years) was available on 24 of 25 survivors. All but 2 patients were in sinus rhythm. Conclusions. Early and midterm outcomes after the Fontan procedure in this patient group improved and may be further ameliorated by detecting and repairing associated obstruction to pulmonary venous flow and performing atrioventricular valvuloplasty before the Fontan procedure.
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