Urgency of operation in infracardiac total anomalous pulmonary venous connection

Because the tendency for pulmonary venous obstruction in the infracardiac type of total anomalous pulmonary venous connection may be partially dependent on the connection of the descending vein to the portal vein, the inferior vena cava, or one of their tributary vessels, we reviewed our surgical experience with various subtypes of infracardiac total anomalous pulmonary venous connection.

The urgency of operation in 4 neonates with infracardiac total anomalous pulmonary venous connection was reviewed.

Two patients with pulmonary venous obstruction in whom the descending vein connected to the portal vein were operated on immediately with successful outcome. One patient who had become critically ill after the ductus venosus had closed died before operation could be undertaken. One patient in whom the descending vein connected to the left hepatic vein was operated on electively with successful outcome.

In hemodynamically stable patients with no clinical or echocardiographic signs of pulmonary venous obstruction, some form of differentiation with regard to urgency of operation may be appropriate. When the descending vein connects to the inferior vena cava or a hepatic vein, the operation may be performed on a semi-elective basis. In contrast, when the descending vein connects to the portal vein or the ductus venosus, operation should generally not be delayed because of the high likelihood of obstruction.