Treatment for Your Child’s Hypoplastic Ventricle

In a normal heart, oxygen-poor blood is pumped to the lungs from the right ventricle. Oxygen-rich blood is pumped to the body from the left ventricle.

: Stage III

Your child has a defect in the heart called a hypoplastic ventricle. This means that the ventricle is either too small or absent. The most common treatment is heart surgery. It is often done in three stages. The surgery does not fully repair the heart problem. But it can relieve symptoms. And it can increase your child’s chances to live a more normal life. This sheet helps you understand the surgery that is done during Stage III of treatment. Your child’s doctor can tell you more as needed. 

The Goals of Heart Surgery for a Hypoplastic Ventricle

• Stage I. Make the single working ventricle the main pumping chamber of the heart. This will let it send oxygen-rich blood to the body.

• Stage II. Decrease the workload of the single ventricle.

• Stage III. Separate the circulation of blood in the heart. This is so oxygen-poor blood and oxygen-rich blood don’t mix.

Stage III: The Fontan Procedure

The Fontan procedure sends oxygen-poor blood from the lower body directly to the lungs.

This surgery is done when your child is 2-3 years old. Your child may stay in the hospital for 5-14 days. This part of the surgery is done to separate the circulation of blood in the heart. This is so oxygen-poor blood does not mix with oxygen-rich blood in the single ventricle. The doctor will do one of these two repairs:

• Intracardiac Fontan. The inferior vena cava (IVC) is a large blood vessel. It brings oxygen-poor blood to the right atrium. A tube is built in the right atrium to make a baffle (tunnel). This is so that blood flows from the IVC to the pulmonary artery. This sends blood straight to the lungs to get oxygen. The tunnel may need a small hole. This is to stop pressure from building up in the lungs right after surgery.

• Extracardiac Fontan. The IVC is sewn into a tube that is put outside the heart. The tube goes straight to the pulmonary artery. This lets oxygen-poor blood flow straight to the lungs to get oxygen.