Treatments and drugs

By Mayo Clinic Staff

There isn't currently a way to replace the defective tricuspid valve. Treatment for tricuspid atresia involves surgery to ensure adequate blood flow through the heart and into the lungs, allowing your baby's body to receive the proper amount of oxygen-rich blood. Often, this requires more than one surgical procedure. Medications also may be given before surgery.

Surgery

Your child will likely need more than one surgical procedure to correct tricuspid atresia. Some of these procedures are called palliative surgeries, because they're done as a temporary fix to immediately increase blood flow. Following are some of the procedures babies with tricuspid atresia may require:

  • Atrial septostomy. This procedure creates or enlarges the opening between the heart's upper chambers (atria) to allow more blood to flow from the right atrium to the left atrium.
  • Shunting. Creating a bypass (shunt) from the main blood vessel leading out of the heart (aorta) to the pulmonary arteries allows for adequate blood flow to the lungs.

  • Surgeons generally implant a shunt during the first four to eight weeks of life. However, babies usually outgrow this shunt and may need another surgery to replace it.

  • Pulmonary artery band placement. If your baby has too much blood flowing to the lungs from the heart, a surgeon may place a band around the pulmonary artery to reduce the amount of blood flowing through the pulmonary artery.
  • Glenn procedure. When babies outgrow the first shunt, they often require the Glenn procedure — a surgery that sets the stage for the more permanent corrective surgery, called the Fontan procedure.

    Doctors usually perform the Glenn procedure when a child is between 3 and 6 months old. Doctors remove the first shunt, and then connect one of the large veins that normally returns blood to the heart (the superior vena cava) to the pulmonary artery instead. This allows oxygen-poor blood to flow directly to the lungs. The procedure reduces the workload on the left ventricle, decreasing the risk of damage to it.

  • Fontan procedure. This surgery is the standard treatment of tricuspid atresia. However, most children with tricuspid atresia don't undergo the Fontan procedure until they are at least 2 years old.

    During a Fontan procedure, the surgeon creates a path for the oxygen-poor blood in a blood vessel that returns blood to the heart (the inferior vena cava) to flow directly into the pulmonary arteries. The pulmonary arteries then transport the blood into the lungs.

    Doctors sometimes leave an opening between the pathway and the right atrium.

Medication

Before surgery, your child's cardiologist may recommend that your child take the medication prostaglandin to help widen (dilate) and keep open the ductus arteriosus.

Follow-up care

To monitor his or her heart health, your baby will need lifelong follow-up care with a cardiologist who specializes in congenital heart disease.

Your child's cardiologist will tell you whether your child needs to continue taking preventive antibiotics before dental and other procedures. In some cases, your child's cardiologist may recommend limiting vigorous physical activity.

The short- and intermediate-term outlook for children who have a Fontan procedure is generally promising. Outcomes for those having surgery later in life are generally less promising. A variety of complications may occur over time and sometimes require additional procedures.

If the circulation system created by the Fontan procedure fails, then a heart transplant may be necessary. Talk to your child's doctor about his or her specific situation.

Aug. 15, 2015