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