Treatment

There's no way to replace a tricuspid valve in tricuspid atresia. Treatment involves surgery to ensure enough blood flow through the heart and into the lungs.

Often, this requires more than one surgery. Medications to strengthen the heart muscle, lower blood pressure and rid the baby's body of excess fluid and supplemental oxygen to help the baby breathe also might be given before surgery.

Medications

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

Surgeries or other procedures

Some of the procedures used to treat tricuspid atresia are a temporary fix to increase blood flow (palliative surgeries). Procedures that might be needed include:

• Atrial septostomy. Rarely, a balloon is used to create or enlarge the opening between the heart's upper chambers to allow more blood to flow from the right atrium to the left atrium.

• Shunting. This creates a bypass (shunt) from a main blood vessel leading out of the heart to the blood vessel leading to the lungs (pulmonary artery), which improves oxygen levels.

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

• Pulmonary artery band placement. If your baby has a ventricular septal defect and too much blood flowing to the lungs from the heart, a surgeon might place a band around the pulmonary artery to reduce the flow.

• Glenn operation. When babies outgrow the first shunt, they often require this surgery, which sets the stage for the more permanent corrective surgery, called the Fontan procedure.

  Doctors usually perform the Glenn operation when a child is between 3 and 6 months old. Doctors remove the first shunt, then connect one of the large veins that normally returns blood to the heart (the superior vena cava) to the pulmonary artery instead.

  This procedure allows blood to flow directly to the lungs and reduces the workload on the left ventricle, decreasing the risk of damage to it.

• Fontan procedure. A variation of this standard treatment of tricuspid atresia is usually done when the child is 2 to 5 years old. In general, the surgeon creates a path for the blood that's returning to the heart (the inferior vena cava) to flow directly into the pulmonary arteries, which then transport the blood into the lungs.

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

Follow-up care

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

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

The short- and intermediate-term outlook for children who have a Fontan procedure is generally promising. A variety of complications can occur over time and require additional monitoring and procedures.

Failure of the circulation system created by the Fontan procedure might make a heart transplant necessary.