Fontan Procedure

The Fontan procedure is the last of the staged operations for children with a single working ventricle, such as hypoplastic left heart syndrome or tricuspid atresia. After the earlier Glenn (or hemi-Fontan) has sent blood from the upper body to the lungs, the Fontan adds the blood from the lower body, so all returning blue blood flows passively into the lungs and the single ventricle pumps only to the body. Francis Fontan first did this kind of repair in 1968 for tricuspid atresia, and the operation has been changed many times since. It is usually done when the child is about 2 to 3 years old. Today there are two main types. In the lateral tunnel Fontan, a patch of PTFE is sewn inside the right atrium to make a tunnel from the inferior vena cava up to the pulmonary arteries. In the extracardiac Fontan, now the choice in many centers, the inferior vena cava is cut from the heart and joined to the pulmonary artery with a PTFE tube graft outside the heart. The extracardiac version can often be done on a warm, beating heart without clamping the aorta, and sometimes without bypass at all; other surgeons use deep cooling and a short period of circulatory arrest. Many surgeons add a small hole (fenestration) between the pathway and the atrium to keep the heart filled after surgery; it often closes on its own or can be closed later with a device. After a Fontan, fluid around the lungs is common, and clots, rhythm problems and poor flow through the lungs are risks. Central lines are avoided to reduce clotting. For the scrub, the case starts with a repeat sternotomy and turns on having the right graft size, the aortic punch for the fenestration, and fine polypropylene ready.