What is Fontan associated liver disease?
Fontan-associated liver disease is a hepatic disorder arising from hemodynamic changes and systemic venous congestion following Fontan surgery. The histological changes produced in the liver are similar but not equivalent to those seen in other forms of cardiac liver disease.
How long does a Fontan last?
Since the Fontan procedure requires open heart surgery, your toddler will be on a heart-lung machine to temporarily take over blood circulation and breathing. The procedure will last about five hours.
What happens after Fontan?
What Happens After the Fontan Procedure? Children who have the Fontan procedure usually spend 1 to 2 weeks in the hospital to recover. They get round-the-clock care and monitoring. They also get medicines to help the heart and improve blood flow.
What is Fontan palliated?
The Fontan operation involves routing of systemic venous return directly into pulmonary circulation bypassing the subpulmonary ventricle. This is an effective palliation in patients with complex congenital heart disease involving single ventricle physiology.
How does a Fontan procedure affect the liver?
Cardiac Center Patients who undergo the Fontan operation as children for a complex congenital heart defect are at risk of developing progressive liver fibrosis, a buildup of fibrous deposits, as a result of the circulation created by the surgery, according to a new study.
Why does Fontan cause liver damage?
Systemic venous pressure elevation caused by passive pulmonary blood flow results in elevated systemic venous pressure, causing liver congestion. In addition, cardiac output and cardiac index are diminished, and as a result, zone 3 hepatocytes may be compromised by decreased oxygen delivery to centrilobar cells.
How successful is Fontan surgery?
Conclusions: Over 80% of patients who survive Fontan surgery will be alive at 20 years. Developing late sequelae including protein losing enteropathy, ventricular dysfunction or requiring a pacemaker predict a higher risk of late death.
What does the Fontan procedure correct?
The Fontan procedure refers to any surgical procedure that leads to systemic flow of venous blood to the lungs without passing through a ventricle. In 1971, Fontan and Baudet (1) described a surgical procedure for repair of tricuspid atresia that built on experimental and clinical research from the 1940s.
Why is Fontan procedure done?
Why is the Fontan procedure done? The Fontan is done so that almost all the blue blood coming back from the body goes to the lungs. After this stage, most children are much “pinker” because now nearly all of the blood pumped out to the body goes to the lungs first.
When is a Fontan procedure performed?
The Fontan procedure is the third stage of the repair. It is done when the child is between 18 months and 2 years of age. A heart catheterization is done before the Fontan.
What is liver fibrosis?
Liver fibrosis is the excessive accumulation of extracellular matrix proteins including collagen that occurs in most types of chronic liver diseases. Advanced liver fibrosis results in cirrhosis, liver failure, and portal hypertension and often requires liver transplantation.