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Tricuspid valve atresia is a congenital heart defect in which the heart’s tricuspid valve doesn’t develop. This means that blood can’t flow from the heart’s right atrium (upper receiving chamber) to the right ventricle (lower pumping chamber) as it should. Because of this, the right ventricle does not properly develop prior to birth. Therefore, the child will have a single functioning left ventricle as their only way to pump blood to their body and lungs.
As the leading providers of pediatric heart care in Louisville and Southern Indiana, cardiothoracic surgeons at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, are experienced in successfully treating tricuspid valve atresia.
The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the skills and experience to provide a pinpoint diagnosis and develop a customized treatment plan for you and your child.
In a heart with tricuspid valve atresia, solid tissue sits between the right atrium and the right ventricle instead of the tricuspid valve. Blood can’t move through the tricuspid valve and the wall separating the right and left sides of the heart does not form completely, creating a ventricular septal defect (VSD). In most babies with tricuspid valve atresia, the heart has two holes — an atrial septal defect (ASD), a hole between the right atrium and the left atrium — and a VSD, a hole between the right ventricle and left ventricle.
Oxygen-poor blood passes through the right atrial hole into the left atrium, where it mixes with oxygen-rich blood. The left ventricle pumps most of the mixed blood out to the body, but some pushes through the VSD and the right ventricle to the lungs.
Treatment for tricuspid valve atresia can improve the baby’s condition, but it can’t make the heart work like one without a defect. A child born with tricuspid valve atresia will regularly see a pediatric cardiologist throughout childhood and as an adult.
A baby born with tricuspid atresia often has serious symptoms soon after birth because blood flow to the lungs is much lower than normal.
A newborn with tricuspid atresia usually will:
Tricuspid atresia happens while the heart is forming very early in pregnancy. No one knows why the valve doesn’t grow normally.
A baby is more likely to have tricuspid atresia if:
Having one or more risk factors doesn’t mean that a baby will have tricuspid atresia. Tricuspid atresia can happen without any risk factors.
Tricuspid atresia sometimes is seen on ultrasound scans before birth. A fetal echocardiogram can give more information and help the delivery team plan treatment.
A screening pulse oximeter test usually is done on all newborns right after birth using a light on a fingertip or toe. If tricuspid atresia isn’t found before birth, this test will show that the baby’s blood is not carrying as much oxygen as expected. The neonatal team will then do other tests such as a bedside echocardiogram to find the diagnosis.
Treatment for tricuspid atresia 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 surgical treatment for tricuspid atresia 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 usually is 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.