Children who are born with either just one ventricle or a ventricle that is underdeveloped will have to undergo a type of open heart surgery called the Fontan. What exactly are single ventricle defects and why do those who have the Fontan procedure need to be concerned about liver disease?

Single Ventricle Defects

One out of every 100 babies is born with a congenital heart defect (CHD), making it the most common birth defect in the United States. A type of CHD called single ventricle defects is rare and affects only five out of every 100,000 babies. Some of the most common types of single ventricle defects include:

• Hypoplastic left heart syndrome (HLHS)
• Hypoplastic right heart syndrome (HRHS)
• Double outlet right ventricle (DORV)
• Double inlet left ventricle (DILV)
• Tricuspid atresia

When there is just one fully functioning ventricle present, it means the heart only has one chamber that is able to pump oxygen-rich blood to the rest of the body. Most babies born with a single ventricle defect will have difficulty breathing and might also have something called cyanosis, which means there is a bluish tint to the lips, fingernails, and skin.

Treatment for Single Ventricle Defects

Babies born with a single ventricle defect will have to undergo three surgical procedures, which includes a shunt, the Glenn operation, and the Fontan operation. As the child grows into an adult, they will most likely need to have a revision to the Fontan so that adjustments can be made in accordance to their growth.

Some Fontan patients will also need to get a pacemaker implanted and will most likely have to take certain prescription medications. One of the most common side effects of the Fontan is liver complications.

Liver Complications in Fontan Patients

One of the main reasons so many Fontan patients end up with liver disease is because higher pressures in the Fontan circulation, along with decreased cardiac output, will cause congestion in the liver. For this reason, it is imperative for those who have had the Fontan to have their liver checked on a regular basis. This can be done in the following ways:

• Abdominal imaging: This may include having a CT scan or an MRI to check for physical abnormalities of the liver.
• Bloodwork: This is to check for increased levels of gamma-glutamyl transferase (GGT) or abnormal protein and albumin levels.

It is highly recommended that babies born with a single ventricle defect see a pediatric cardiologist that understands the Fontan and liver complications following the procedure. For more information, contact a group such as DirectImaging.