Staged Fontan operation for children with heterotaxy syndrome, bilateral ductus arteriosus, and nonconfluent pulmonary artery

We report two cases of successful Fontan operation in children with heterotaxy syndrome associated with univentricular physiology and absent and nonconfluent central pulmonary arteries with both distal pulmonary arteries directly connected to the ipsilateral ductus arteriosus. After unilateral systemic-pulmonary shunt, the central pulmonary artery was reconstructed with a polytetrafluoroethylene prosthetic graft concomitantly with bidirectional cavopulmonary shunt. Finally, extracardiac total cavopulmonary connection was performed as an off-pump procedure. Children with bilateral ductus arteriosus and a nonconfluent pulmonary artery with univentricular physiology present a particular challenge in regard to completing Fontan operations. Careful attention should be directed at ensuring balanced growth of the bilateral distal pulmonary arteries. When planning reconstruction of the central pulmonary artery with a prosthesis, late reconstruction may be beneficial, as it enables utilization of a larger-caliber graft, obviating the need for replacement during a subsequent Fontan operation.