Babies born with tricuspid atresia don’t have a tricuspid valve — the valve between the right atrium and the right ventricle. This causes the right ventricle not to develop or to be very small. Tricuspid atresia repair starts immediately with treatment including surgery.

Tricuspid atresia repair starts with medication to keep the ductus arteriosus open. This blood vessel between the aorta and the pulmonary artery is open before birth, when the baby gets oxygen from the mother instead of from the lungs. Once the baby is born, this connection is supposed to close.

Keeping the ductus arteriosus open with medications is a temporary measure, providing a path for delivering oxygen-rich blood to the body that would have disappeared if the ductus were allowed to close.

Tricuspid atresia repair can involve one of several complex procedures that require experience, skill and training. Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, is the leading provider of pediatric heart care in Louisville and Southern Indiana.

The tricuspid atresia repair procedure chosen by the cardiothoracic surgeons at Norton Children’s Heart Institute will depend on variations around the heart’s structure — how the child’s heart has developed despite the defect.

Variations can include:

• An atrial septal defect (ASD), a hole between the two atria, allowing blood from both circulations to mix.
• A ventricular septal defect (VSD), a hole between the two ventricles, allowing blood from both circulations to mix.
• Transposition of the great vessels (TGA), a reversal of the aorta and pulmonary artery. In this case, blood reaches the lungs through the pulmonary artery connection to the left ventricle, instead of the right ventricle. But the only path for oxygenated blood to get out to the body is through the ductus arteriosus, atrial septal defect or the ventricular septal defect.
• Pulmonary atresia or stenosis, narrowing or complete closure of the pulmonary valve, affecting blood flow to the lungs.
• Hypoplastic aortic arch or coarctation of the aorta, a narrowing of the aortic arch or a part of the aorta itself.
• Total anomalous pulmonary venous return (TAPVR), where instead of the pulmonary veins returning to the left atrium, they abnormally return to a right-sided venous structure.
• Other variations not listed here also can exist.

Tricuspid Atresia Repair Variations

Tricuspid atresia repair treatment will be based on the child’s unique needs. The treatment usually includes three surgeries that take place at various ages. Newborn treatment often is based on how much blood flow is occurring to the lungs and body:

• A child that has too little blood flow to the lungs or body will need immediate treatment. For a newborn (less than 1 to 2 weeks old), a medicine called prostaglandin can be given through an IV to reopen the connection between the pulmonary artery and aorta (ductus arteriosus) and improve blood flow.
• A child with too little blood flow to the lungs will need surgery to create a connection between the arteries to the body, and the arteries to the lungs. This surgery is called a modified Blalock-Taussig shunt. During this surgery, a small tube is placed between the artery to the arm (subclavian artery) and the arteries to the lungs (pulmonary artery).
• If a child has too much blood flow to the lungs (tricuspid atresia with a large ventricular septal defect), the blood flow will need to be reduced to protect the lungs from damage. This is done by surgically placing a band around the pulmonary artery so that blood flow to the lungs is controlled.
• If the child has inadequate blood flow through the aorta (tricuspid atresia with ventricular septal defect and transposition of the great arteries), blood from the normal-size left ventricle will need to be routed to the aorta. This usually means reconstructing the aorta. This is called the Norwood procedure. Placing a modified Blalock-Taussig shunt can then create pulmonary blood flow.

No matter which surgery is performed, children with tricuspid atresia will need more heart surgeries:

• Bidirectional Glenn procedure: This surgery is done when the child is 3 to 6 months old. The superior vena cava, a vein carrying deoxygenated blood into the heart, is detached from the heart and connected directly to the pulmonary artery. The Blalock-Taussig shunt is removed. This allows blood from the upper body to flow directly to the lungs to pick up oxygen without having to be pumped by the heart. It also keeps blood that already has oxygen from returning to the lungs, so the heart doesn’t have to do unnecessary work. At this point, blood returning from the lower body through the inferior vena cava is still going directly back to the body without first going through the lungs. Because of this, the child will continue to have low oxygen levels and may still appear blue (cyanosis).
• Fontan procedure: The third surgery to improve blood circulation is usually done when the child is between ages 2 and 4. This procedure involves connecting the inferior vena cava directly to the pulmonary artery, forcing all blood returning from the body to pass through the lungs and pick up oxygen before being pumped back out to the body. This procedure will lead to better oxygen levels in the blood, which will diminish signs of cyanosis, and the child’s skin and lips should no longer appear blue.

Tricuspid Atresia Complications and Follow-up Care

The results for the three-stage tricuspid atresia repair surgeries are generally good. Expected survival rates for children who complete all three stages of surgery are 75% to 95%, depending on the factors unique to each child and the care they receive.

Long-term quality of life following the Fontan operation is usually good. Currently, the oldest patients who have had this procedure are age 30 and older. However, complications can occur, including heart rhythm issues that require medication or pacemaker placement. Lung issues also can occur, including a decrease in blood oxygen levels. Digestive issues can occur due to protein loss in the stool, which results in swelling and water retention. Some patients, despite doing well after surgeries, may need a heart or lung transplant later in life.

Why Choose Norton Children’s Heart Institute

• Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
• The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
• The Adult Congenital Heart Association has accredited Norton Children’s Heart Adult Congenital Heart Disease Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
• More than 17,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
• Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
• The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.