How is tricuspid atresia treated?

Tricuspid atresia has potentially fatal complications, including not getting enough oxygen to the body’s tissues, and a high red blood cell count, which may cause clots that can lead to stroke or heart attack. These complications can be avoided with prompt treatment.

While medications can help babies who have tricuspid atresia, this condition is usually treated with surgery. Surgeries for tricuspid atresia include:

  • Balloon septostomy: This is a surgery which creates an atrial septal defect (or widens an existing one). This procedure may be required soon after the child’s birth if the child is very blue as a life-saving measure. One of the following two surgical operations may then follow depending upon the condition of the child.
    • Modified BT shunt: In a cyanosed (blue) child, an artificial tube is placed between a big artery in the chest and arteries of the lungs to increase the blood flow to the lungs and improve the oxygen supply to the body. This is done by an open operation usually within the first few days of life.
    • Pulmonary artery banding: In a child who has heart failure due to excessive blood flow to the lungs, a flexible band is placed surgically around the pulmonary artery to restrict the blood flow to the lungs. This surgery is required within days to weeks after birth.
  • The Glenn procedure: This procedure, done between four and six months of birth, is one in which the surgeon connects the superior vena cava to the pulmonary artery. Blood can then flow directly to the lungs (instead of the heart) and pick up oxygen.
  • The Fontan procedure: During this surgery, the surgeon forms a path that allows oxygen-poor blood that is going back to the heart to flow directly into the arteries that transport blood into the lungs. Most children with tricuspid atresia do not have the Fontan procedure until they are at least 2 years of age. Some children are not good candidates for this surgery.

What follow-up care is required after treatment?

Your baby will need lifelong follow-up care so that a cardiologist can monitor his or her health. The cardiologist can let you know whether your child has to continue taking preventive antibiotics before procedures such as dental care. Sometimes, the cardiologist will recommend that your child limit physical activity that is too vigorous.

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