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.

Tricuspid Valve Atresia Symptoms

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:

• Have bluish skin (cyanosis)
• Breathe fast
• Have problems feeding
• Get tired quickly when feeding
• Be less active than most babies

Tricuspid Atresia Diagnosis

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:

• The baby has Down syndrome (trisomy 21)
• Either parent has a congenital heart defect
• The mother had a rubella (German measles) infection or other viral infection during pregnancy
• The mother has poorly controlled diabetes or lupus (an autoimmune disease)
• The mother used certain antiacne or antiseizure medicines during pregnancy

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.

Tricuspid Atresia Treatment

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:

• A child with 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 defectand 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 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.

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.