Paediatrics: Total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection

All blood returning to the heart (systemic and pulmonary) returns to the right atrium, and an obligatory patent foramen ovale or ASD is necessary for survival. The pulmonary veins form a single channel and join the systemic venous circulation through either a supracardiac superior vena cava (SVC), intra- or intracardiac IVC connection.

Clinical features

Infants usually present in the first few days of life with varying degrees of obstruction, cyanosis, and congestive cardiac failure. The presentation will depend on the degree of obstruction to the pulmonary venous return.

Treatment

This is often a surgical emergency and anastomosis of the common pulmonary channel to the left atrium, with the closure of ASD, and interruption of connections to the systemic venous circuit is required.