Treatments and drugs

By Mayo Clinic Staff

There's currently no 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 can be done.

Surgery

Your child will likely need more than one surgical procedure to correct tricuspid atresia. Some of these procedures are called rescue 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 typically 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.
  • Glenn procedure. When babies outgrow the first shunt, they often require 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. It connects one of the large veins that normally return blood to the heart (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 surgery, the surgeon creates a path for the oxygen-poor blood returning to the heart to flow directly into the pulmonary arteries, which then transport the blood into the lungs.

Medication

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

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 worse. 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.

Nov. 08, 2012