Extracardiac Fontan in apicocaval juxtaposition.

Extracardiac total cavopulmonary connection between the inferior vena cava and right pulmonary artery has become the most popular Fontan modification nowadays, yielding good results. However, certain situs anomalies along with systemic venous drainage variations give rise to an issue about the best way for extracardiac conduit routing. Three cases of apicocaval juxtaposition are described. The situs was inversus with the apex and inferior vena cava on the same side (left), whereas the superior vena cava (Glenn) was located on the right in all cases. The literature is revisited and technical options are discussed. Surgery was carried out with excellent short- and midterm results.