Fig. 5.

Fontan circulation from first to final stages of total cavo-pulmonary connection, illustrated in the hypoplastic left heart syndrome. Illustration of untreated hypoplastic left heart syndrome is to the left, followed by the three surgical operations: Step 1, a shunt (homograft-patch, orange) is created from the single right ventricle to the pulmonary artery and the atrial septal defect is enlarged. Step 2, a bidirectional cavo-pulmonary anastomosis is created while the shunt is removed. In the third and final step, the extracardiac conduit in Gore-Tex (yellow) is placed as a total cavo-pulmonary anastomosis, creating the Fontan circulation. An animation of the blood flow in the different surgical stages during the myocardial contraction can be seen in the supplementary material. Illustrations created by Michael Bjaanes in collaboration with the Department of Pediatric Cardiology at Oslo University Hospital. Reprint of modified version with permission from [2]