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Treatment for Your Child’s Single Ventricle Heart Defect: Stage III Surgery

Front 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.

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.

 

Your child has a single ventricle type of heart defect. 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 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 single ventricle heart defects

  • 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.

What are the risks and complications

Risks and possible complications include:

  • Infection
  • Bleeding
  • Problems with the nervous system, such as seizure or stroke
  • Abnormal heart rhythm (arrhythmia)
  • Abnormal buildup of fluid around the heart or lungs
  • Problems in the lungs
  • Damage to the nerves that control the vocal cords or the diaphragm

 

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.

The Fontan procedure sends oxygen-poor blood from the lower body directly to the lungs without being pumped by the ventricle.

 

This surgery is done when your child is 2 or older. 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 2 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.
  • Extra-cardiac 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 should I call my child's healthcare provider

Call your child's provider right away if any of these occur after surgery:

  • Increased redness, draining, swelling, or bleeding at the incision site
  • Belly pain
  • Fever of 100.4°F (38°C) or higher, or as directed by your child's healthcare provider
  • Trouble feeding/eating, poor appetite, or weight loss 
  • Shortness of breath or difficulty breathing
  • Color changes (gray, pale or blue) of the skin, lips or fingernails
  • Cough that won’t go away
  • Increased tiredness or irritability
  • Prolonged nausea or vomiting
  • Irregular heartbeat
  • No noticeable improvement or you don't think your child is getting better

 

 The Fontan procedure sends oxygen-poor blood from the lower body directly to the lungs without being pumped by the ventricle.

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