Surgical repair and postoperative course of an infant with infracardiac total anomalous pulmonary venous connection, cor triatriatum sinistrum and transposition of the great arteries.

The combination of a d-transposition of the great arteries, cor triatriatum sinistrum and a total anomalous pulmonary venous connection of the infracardiac type is a very rare condition. Up to now, one surgical repair in an adolescent with transposed great arteries and total anomalous pulmonary venous drainage of the supracardiac type has been reported. In this paper, an infant with the above mentioned cardiovascular malformation is presented. The common pulmonary vein drained into the inferior vena cava and was obstructed. There were arborisation abnormalities in both lungs with mild pulmonary hypertension. The infant has been successfully operated upon at the age of 6 months and a weight of 4.5 kg. The membrane within the left atrium was resected, the common pulmonary vein was anastomosed to the left atrium and a Mustard procedure was performed. During the first 6 postoperative weeks, the infant had problems with adaptation. There was both a transient ballooning of the Mustard patch with significant obstruction of the pulmonary venous drainage and a delayed pulmonary recovery. Two months later, the patch was straightened and the child could be discharged from hospital. After 12 months, the child died from an infection of the airways.