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 sys-temic venous circulation through either a
supracardiac superior vena cava (SVC), intra- or infracardiac IVC connection.
Infants usually present in the
first few days of life with varying degrees of obstruction, cyanosis, and
congestive cardiac failure. Presentation will depend on the degree of
obstruction to the pulmonary venous return.
This is often a surgical emergency
and anastomosis of the common pulmo-nary channel to the left atrium, with
closure of ASD, and interruption of connections to the systemic venous circuit