What is a normal Fontan pressure?
Typical pressures in a well compensated young patient with a Fontan circulation are a CVP (mPAP) of 12 mm Hg and atrial pressure of 5 mm Hg, so giving a transpulmonary pressure gradient of about 7 mm Hg.
What is a failing Fontan?
The Australian and New Zealand Fontan Registry (ANZFR) defines Fontan failure as the occurrence of death, heart transplant, protein-losing enteropathy (PLE), plastic bronchitis or New York Heart Association (NYHA) Functional class III or IV at follow-up.
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.
What is high Fontan pressure?
High systemic venous pressure is the essence of the Fontan paradox where a higher than normal systemic venous pressure is necessary to maintain pulmonary blood flow. In failing Fontan, this systemic venous hypertension is transmitted to the venous and the lymphatic circulation.
Why does Fontan cause liver damage?
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.
How long does Fontan heart last?
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 is FALD with the liver?
Fontan-associated liver disease (FALD) is a major non-cardiac determinant of mortality following the Fontan procedure. • The incidence of liver cirrhosis and hepatocellular carcinoma (HCC) increases with the duration of the Fontan circulation.
How long can you live with Fontan surgery?
The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure.
What is FALD medical term?
What does bridging fibrosis mean?
A term for the presence of bands of varying thickness (the wider the bands, the more advanced the disease) of fibrous tissue and collagen seen by low-power light microscopy, which span (connect) portal spaces and/or centrilobular spaces in patients with evolving cirrhosis.