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Treatment for Your Child’s Hypoplastic VentricleFront view cross section of heart showing atria on top and ventricles on bottom showing aorta, pulmonary artery, mitral valve, aortic valve, left atrium, left ventricle, right atrium, right ventricle, tricuspid valve, pulmonary valve, superior vena cava, and inferior vena cava. Arrows on right side of heart show oxygen-poor blood pumping to lungs. Arrows on left side of heart show oxygen-rich blood pumped to body.: Stage III

Your child has a defect in the heart called a hypoplastic ventricle. This means that 1 of the ventricles is either too small or absent. The most common treatment is heart surgery. It is often done in 3 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 provide blood to both the lungs and the body. 

  • Stage II. Decrease the workload of the single ventricle. This reduces the mixing of oxygen-poor and oxygen-rich blood. 

  • Stage III. Separate the circulation of blood in the heart. This is so very little oxygen-rich and oxygen-poor blood mix.

Risks and Possible Complications of Heart Surgery

Risks and possible complications include:

  • Arrhythmia (abnormal heart rhythm)

  • Problems in the lungs

  • Infection

  • Bleeding

  • Problems with the nervous system, such as seizure or stroke

  • Abnormal buildup of fluid around the heart or lungs

Stage III: The Fontan Procedure

Front view cross section of heart showing Fontan procedure for hypoplastic left ventricle. Superior vena cava is connected to pulmonary artery. Tube outside heart is connected to inferior vena cava at bottom and superior vena cava at top. Arrows show blood flowing from left atrium to right ventricle, then being pumped out aorta.

This surgery is done when your child is 2 to 3 years old. Your child may stay in the hospital for 1 to 2 weeks. 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 within the right atrium to make a baffle (tunnel). This is so that blood flows from the IVC directly to the pulmonary artery. This sends blood straight to the lungs to get oxygen. Sometimes, a small hole is left in the tunnel (fenestration). This hole acts like a pop-off valve, preventing pressure from building up in the lungs too much 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. A fenestration may also be used in this case.

When to Call the Doctor

Call the doctor right away if your child has any of the following:

  • Increased redness, draining, swelling, or bleeding at the incision site

  • Fever 100.4°F (38°C) or higher

  • Irritable

  • Trouble feeding, poor appetite, or not gaining weight 

  • Belly pain

  • Tiredness

  • Shortness of breath

  • Cough that won’t go away

  • Nausea or vomiting that continue

  • Irregular heartbeat

  • Your child doesn't seem to be improving


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