Complex double-outlet right ventricle repair in a neonate with complete tracheal agenesis

underline the relative greater frequency of this complication in heterotaxy syndrome when associated with anomalous pulmonary and systemic venous connections, which can make surgical repair complicated with the need to create a tortuous and potentially obstructive pathway. Despite this concern, a recent report from our institution 5 has shown excellent outcome with cavopulmonary connection in patients with heterotaxy syndrome and anomalous pulmonary venous return. This is likely due to improved surgical technique and use of extracardiac conduit in cases with complex atrial and venous anatomy (18%). None of the 3 patients had evidence of obstruction at the pulmonary venous pathway early after the Fontan procedure, as determined by 2-dimensional echocardiography. Pulmonary venous obstruction developed slowly through a period of several months to years. In patient 2, pulmonary venous obstruction developed 4 months after device closure of the fenestration, suggesting that the additional scarring from device implantation contributed to the obstruction. The obstruction was due to fibrotic tissue at either the interatrial septum or in the interatrial course of the pulmonary veins (patient 2). To prevent this complication, and to ensure a wide open pulmonary venous outflow, particularly in patients with mitral atresia or stenosis, we currently enlarge the communication between the pulmonary veins and the right atrium by unroofing the coronary sinus posteriorly into the left atrium, in addition to resection of the interatrial septum. In conclusion, late onset obstruction of pulmonary venous pathway after lateral tunnel cavopulmonary connection is a rare but serious complication that may be prevented by unroofing the coronary sinus at the time of Fontan procedure.