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.

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 complex. It requires careful management of your child’s health. And treatment does not repair your child’s heart problem. But it can relieve symptoms and increase your child’s chances to live a more normal life. You and your child’s doctor have decided that the benefits of this surgery outweigh any risks. This sheet helps you understand the surgery that is done during stage I. 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 1 working ventricle the main pumping chamber of the heart. This provides blood to both the lungs and the body.

Stage II. Decrease the workload of the 1 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

  • 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 first stage of surgery is called the Norwood Procedure. It is generally done within the first week after birth. A hospital stay of 3 to 4 weeks may be needed. Your child will need this procedure if the aorta or other left heart structures are too small or absent. This includes hypoplastic left heart syndrome. The procedure 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 procedure, the surgeon performs the following:

  • Atrial septectomy. The atrial septum (wall dividing the 2 upper chambers) is removed. This allows oxygen-rich blood from the left atrium to mix with oxygen-poor blood in the right atrium.

  • Reconstruction of aorta. The main pulmonary artery is divided. It is used, along with patch material, 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). A new pathway must be created to send blood to the lungs. This is because the main pulmonary artery has been used to rebuild the aorta. So, a shunt is placed. 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 in place of the shunt. In this case, a tube is placed from the right ventricle to send blood directly to the pulmonary arteries that are connected to the lungs.

When to Call the Doctor

After any of these surgeries, 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

  • 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 improving