How does a Fontan circulation work?

The Fontan procedure involves redirecting blood flow from the lower body to the lungs. In the Fontan procedure: The inferior vena cava (IVC) is disconnected from the heart and routed directly to the pulmonary artery. Usually a large tube called a “conduit” is added to make the connection.

What are Fontan patients?

The Fontan procedure is used in pediatric patients who possess only a single functional ventricle, either due to lack of a heart valve (e.g. tricuspid or mitral atresia), an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome or hypoplastic right heart syndrome), or a complex …

How many Fontan patients are there?

The worldwide population of patients with Fontan circulation grew to an estimated 50 000 to 70 000 patients in 2018, with 40% of patients >18 years of age.

What does the Fontan procedure do?

The Fontan procedure refers to any surgical procedure that leads to systemic flow of venous blood to the lungs without passing through a ventricle.

When is Fontan done?

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 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.

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 when a Fontan fails?

Late Fontan failure may present insidiously over years. It is a failure of medical management to interpret the absence of overt symptoms or ascites as evidence of optimal haemodynamic status in the functionally univentricular circulation.

What age is Fontan for?

What is a failed Fontan?

What is a Fontan fenestration?

Key points. Fenestration closure after a Fontan operation is a procedure to close the hole between your child’s heart and the tunnel that takes oxygen-poor blood from the body to the lungs. The procedure is done in the heart catheterization lab.

What is the essence of the Fontan circulation?

The essence of the Fontan circulation • only 1 functional ventricle systemic ventricle • no subpulmonary ventricle • Systemic venous return flows, without a pumping ventricle, into the lungs The essence of the Fontan circulation • only 1 functional ventricle systemic ventricle • no subpulmonary ventricle

What do you need to know about the Fontan procedure?

Identify potential complications of Fontan circulation. The Fontan procedure refers to any surgical procedure that leads to systemic flow of venous blood to the lungs without passing through a ventricle.

How old does a child have to be to have a Fontan circulation?

This circuit has growth potential and can therefore be created in children as young as 1 year; it leaves a minimal amount of atrial tissue exposed to high pressure, which in time may cause atrial arrhythmia.

When to use the univentricular Fontan heart procedure?

The univentricular Fontan procedure may be considered for patients with cardiac malformation and a single functional chamber, which usually results from a dysfunctional heart valve or an absent or inadequate pumping chamber.