What is the Fontan?

The Fontan is a series of three surgeries performed for different types of heart abnormalities where there is only one functional pumping chamber. The goal of the surgery is to separate the blue and red blood and make the heart do only one job which is to pump the red blood to the body. This is achieved by redirecting oxygen-poor blood from the lower part of the body towards the pulmonary artery and lungs. This allows all the oxygen-poor blood to bypass the heart and flow directly into the pulmonary artery and lungs, while oxygen-rich blood from the lungs enters the heart and is pumped to the rest of the body.

Before the procedure

Testing will be done to determine if a child is ready for a Fontan procedure. This will include ECGs, echocardiograms, a cardiac catheterization procedure, and in some cases, a cardiac CT or MRI.

Surgery

During this surgery, the inferior vena cava (IVC), a large vein that drains the lower part of the body, is connected to the pulmonary artery. This allows all of the oxygen-poor blood to drain directly into the lungs and only oxygen-rich blood is received by the single ventricle to pump to the rest of the body.

Adult and Adolescent Management

Infants who do not undergo surgical correction or receive a heart transplant have a significantly reduced chance of survival.

The Fontan circulation is a special kind of circulation that affects other organs if it is not functioning well. It can affect many organ systems such as the brain, lungs, liver, gut and kidneys. Abnormal heart rhythms can also develop. Lifelong follow up with a cardiologist is required. Regular monitoring allows the cardiologist to assess the heart's function, identify any potential issues, and provide appropriate care and intervention as needed. This includes bloodwork, ECGs, Holter monitors, echocardiograms, exercise stress tests, cardiac catheterization procedures and imaging such as a CT or MRI.  

Children with this heart condition may experience limitations in exercise tolerance compared to individuals with normal hearts. Growth and development may be affected. Neurodevelopmental issues such as intellectual disability or mental health conditions such as ADHD have been observed. 

Adolescents and adult females should inform their cardiologist of any plans of pregnancy. The use of contraception should be discussed because some maintenance medications can interact with oral contraceptive pills increasing the risk of stroke. The pregnancy itself puts a lot of strain on the heart, but a healthy pregnancy with a Fontan circulation is possible.