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Treatment for Your Child’s Hypoplastic Ventricle: Stage I

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 heart problem that includes a hypoplastic ventricle. The most common treatment is heart surgery. This is often done in 3 stages. Treatment is complicated. It requires careful management of your child’s health. And treatment does not fix your child’s heart problem. But it can ease symptoms and increase your child’s chances to live a more normal life. You and your child’s healthcare provider have decided that the benefits of this surgery outweigh any risks. This sheet helps you understand the surgery that is done during the first stage. Your child’s cardiologist or surgeon can answer your questions and tell you more as needed.

The goals of heart surgery for a hypoplastic ventricle

Stage I. Make the one working ventricle the main pumping chamber of the heart. This provides stable blood flow to both the lungs and the body.

Stage II. Ease the workload of the one ventricle. This reduces the mixing of oxygen-poor and oxygen-rich blood.

Stage III. Separate the circulation of blood in the heart. This means 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

  • Problems with the vocal cords and diaphragm

  • Infection

  • Bleeding

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

  • Abnormal buildup of fluid around the heart or lungs

Stage I: The Norwood procedure

Front view cross section of heart showing Norwood procedure. Patch widens aorta (neoaorta), atrial septum is removed, and shunt goes from artery branching from aorta to pulmonary artery. Arrows show blood flowing from left atrium to right ventricle and mixed blood going from right ventricle to aorta. Some blood from aorta goes through shunt to pulmonary artery.
The Norwood procedure makes the right ventricle the main pumping chamber of the heart. The ventricle can then provide blood flow to the entire body. The surgery also makes sure blood to and from the lungs goes to the right ventricle.
The first stage of surgery is called the Norwood procedure. It is generally done within the first week after birth. Your child may need to stay in the hospital for 3 to 4 weeks. Your child will need this surgery if the aorta or other left heart structures are too small or missing. This includes hypoplastic left heart syndrome. The surgery rebuilds the heart so that the right ventricle is used as the main pumping chamber. Blood can then be pumped from the right ventricle to the rest of the body to deliver oxygen. During the surgery, the surgeon does the following:

  • Atrial septectomy. The surgeon removes the wall dividing the two upper heart chambers (atrial septum). This allows oxygen-rich blood from the left atrium to mix with oxygen-poor blood in the right atrium.

  • Reconstruction of aorta. The surgeon divides the main pulmonary artery. This artery, along with patch material, is used to rebuild the aorta. This is called the neoaorta or “new” aorta. Blood from the right ventricle can then be pumped through the pulmonary valve to the new aorta. This sends blood from the right ventricle directly to the body instead of to the lungs.

  • Placement of shunt (tube). The surgeon creates a new pathway to send blood to the lungs. This is because the main pulmonary artery has been used to rebuild the aorta. So the surgeon places a shunt. It connects an artery branching from the aorta to the pulmonary artery. This allows a controlled amount of blood to reach the lungs. As an alternative, the surgeon may use a technique called the Sano method instead of the shunt. In this case, the surgeon places a tube from the right ventricle to send blood directly to the pulmonary arteries that are connected to the lungs.

When to call the healthcare provider

After any of these surgeries, call your child's healthcare provider right away if your child has any of the following:

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

  • Fever of 100.4°F (38°C) or higher, or as directed by your child's healthcare provider

  • Trouble feeding, poor appetite, or not gaining weight

  • Irritable

  • Tiredness

  • Shortness of breath

  • Cough that won’t go away

  • Nausea or vomiting that continue

  • Irregular heartbeat

  • Your child doesn't seem to be getting better

Online Medical Reviewer: Ayden, Scott, MD
Online Medical Reviewer: Bass, Pat F III, MD, MPH
Last Review Date: 10/1/2016
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