Heart and Blood Conditions

Tricuspid Atresia

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    • For appointments in Seattle, Bellevue, Everett, Federal Way, Olympia, Tri-Cities and Wenatchee, call 206-987-2515.
    • For appointments in Tacoma and Silverdale, call 253-272-1812.
    • For appointments in Alaska, call 907-339-1945.
    • For appointments in Montana, call 406-771-3223.
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    • Seattle Children's Main Campus: 206-987-2515
    • Bellevue Clinic and Surgery Center: 425-454-4644
    • Everett: 425-304-6080
    • South Clinic in Federal Way: 253-838-5878
    • Olympia: 360-459-5009
    • South Sound Cardiology Clinics: 253-272-1812
    • Tri-Cities (Richland): 509-946-0976
    • Wenatchee: 509-662-9266
    • Pediatric Cardiology of Alaska: 907-339-1945
    • Pediatric Cardiology of Montana: 406-771-3223
    • Seattle Children's doctors provide many cardiac services at regional sites throughout the Pacific Northwest. See our complete list of Heart Center locations.

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    • If you are a provider, fax a New Appointment Request Form (NARF) (PDF) (DOC) to 206-985-3121 or 866-985-3121 (toll-free).
    • No pre-referral work-up is required for most conditions. If you have already done testing such as an EKG, Holter monitor or echocardiogram, please fax this information as well as relevant clinic notes and the NARF to 206-985-3121 or 866-985-3121 (toll-free).
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    • View our complete Heart Center Referral Information (PDF).

What is tricuspid atresia?

Tricuspid atresia (pronounced try-CUSP-id ah-TREE-sha) is a birth defect in which a baby’s heart does not have a tricuspid valve.

In normal hearts, the tricuspid valve is the door that allows blood to flow from the right atrium, one of the receiving chambers of the heart, into the right ventricle, 1 of the pumping chambers of the heart. The right ventricle then pumps the blood through the pulmonary artery to the lungs to pick up oxygen.

In babies with tricuspid atresia, the tricuspid valve does not form. Blood is not able to travel directly from the right atrium to the right ventricle.

These babies typically pump some blood to their lungs by a different route. Even so, the oxygen levels in the blood are lower than normal, which can make them look blue (cyanotic). In addition, the right ventricle often does not grow the way it should and is smaller than normal.

Symptoms usually begin within the first few hours after the baby is born. In some cases, it may take a few days for symptoms to appear.

Most babies with tricuspid atresia need treatment in the first days or weeks of life.

  • Tricuspid atresia is one of the more complex forms of congenital heart defects.

    In babies with tricuspid atresia, the only way for blood to get out of their right atrium is through a hole in the atrial septum, the wall separating the right atrium from the left atrium. This is called an atrial septal defect (ASD). Most babies with tricuspid atresia also have a hole in the ventricular septum, the wall between the 2 ventricles. This is called a ventricular septal defect (VSD).

    With tricuspid atresia, oxygen-poor (blue) blood comes from the body into the right atrium and crosses the atrial septal defect into the left atrium, where it mixes with oxygen-rich (red) blood. This mixed (purple) blood then flows into the left ventricle, which pumps it through the aorta to the brain and body. If a VSD exists, some of the blood crosses through the hole and is pumped to the pulmonary artery.

    Some of the blood that enters the aorta is channeled across a blood vessel called the ductus arteriosus. This vessel joins the aorta to the pulmonary artery and provides another way for some blood to get to the lungs.

    The ductus arteriosus usually closes soon after birth. In newborns with tricuspid atresia, the ductus arteriosus may be the only way for blood to reach their lungs. For this reason, a medicine called prostaglandin is sometimes started immediately after birth to prevent the ductus arteriosus from closing.

Tricuspid Atresia at Seattle Children’s

  • U.S. News & World Report consistently ranks Seattle Children’s cardiology and heart surgery program as one of the best in the country. With more than 40 pediatric cardiologists, we have experience diagnosing and treating every kind of heart problem.

    Our heart team has treated many children with tricuspid atresia. We have extensive experience with the treatment these patients require, including cardiac catheterizations and surgery. Children with tricuspid atresia receive compassionate, comprehensive care through our Single Ventricle Program.

    Seattle Children’s outcomes for surgeries to reconfigure the heart’s circulation system are among the best in the nation.

    We have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery.

    Seattle Children’s has been treating children since 1907. Our team members are experts in their fields and in meeting the unique needs of children. For example, the doctors who give your child anesthesia (sedation) are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.

  • When you come to Seattle Children’s, a team of people will take care of your child. Along with your child’s heart doctor (cardiologist), you are connected with other providers, such as newborn specialists (neonatologists), nurses, child life specialists, social workers and others, if their expertise is needed.

    We work together to meet all of your child’s health needs and help your family through this experience. We’ll discuss treatments in ways you can understand and involve you in every decision.

Symptoms of Tricuspid Atresia

Most babies with tricuspid atresia show symptoms during the first few hours of life. However, in some babies, symptoms do not appear until a few days or weeks after birth.

Symptoms of tricuspid atresia may include:

  • Murmur (the sound of blood moving in the heart)
  • Bluish skin tone (cyanosis)
  • Fast breathing (tachypnea)
  • Working hard to breathe
  • Poor feeding
  • Poor weight gain or growth

Diagnosing Tricuspid Atresia

To diagnose this condition, your doctor will examine your baby and use a stethoscope to listen to their heart. Doctors can often hear a heart murmur — the sound of blood moving in the heart in a way that’s not normal.

The doctor will ask for details about any symptoms your child has, their health history and your family health history.

Your child will need an echocardiogram test (an ultrasound of the heart) so the doctor can see how their heart works.

Your child may need other tests. These will likely include:

Treating Tricuspid Atresia

For babies with tricuspid atresia, your doctor will likely suggest some procedures and treatments right away to improve your baby’s blood flow. Other procedures may be done later, such as open-heart surgery. Most babies can be helped with surgery.

We provide complete care for children with tricuspid atresia through our Single Ventricle Program. You’ll be coached on how to do home monitoring and provided with 24-hour support should problems arise. This allows us to identify small issues before they become serious problems.

Treatment Options for Tricuspid Atresia

  • Your doctor may give your baby a drug (prostaglandin) to help keep the ductus arteriosus from closing and to help the blood circulate.

  • Your baby will likely need several surgeries to improve their blood flow. The exact procedures and timing depend on your child’s body and symptoms. The surgeries are done in stages during the first few years of life. The surgeries that may be needed are:

    Blalock-Taussig shunt

    Prostaglandin is the medicine that is used to prevent the ductus arteriosus from closing. The open (patent) ductus arteriosus is a good short-term solution to providing blood to the lungs.

    However, a more permanent connection is often needed. This is called a Blalock-Taussig (BT) shunt. The BT shunt is a small plastic tube that connects two parts of the heart. It serves as a stable connection between the aorta and the pulmonary artery. During this surgery, a small tube is sewn to connect the blood to the body with the blood to the lungs. This permanently replaces the ductus arteriosus.

    If a BT shunt is needed, this surgery is performed during the newborn period. After this surgery, the oxygen levels in the body will be lower then normal, usually around 80%.

    Bidirectional Glenn

    The bidirectional Glenn (sometimes referred to as the hemi-Fontan) is a surgery that redirects the superior vena cava (the large vein draining blue blood from the upper part of the body) from the heart to the lungs. Normally, the superior vena cava drains into the heart.

    After the surgery, the blue blood will drain directly to the lungs without being pumped to the heart. This surgery cannot be done in newborns because the blood pressure in the lungs is too high. This is usually performed around 3 to 6 months of age when the blood pressure in the lungs has reached its normal low level. After this surgery, the oxygen in the body will still be lower than normal, usually around 80%.

    Fontan

    The final planned surgery in repairing tricuspid atresia is called the Fontan operation. In this surgery, the inferior vena cava (the large vein that carries the blue blood returning from the lower part of the body) is redirected from the heart to the lungs. Normally, the inferior vena cava drains into the heart.

    After this surgery, all the blue blood returning from the body flows directly to the lungs rather than being pumped by the heart. When the Fontan surgery is completed, the flow to the lungs is completely separated from the flow to the body and the oxygen saturation is essentially normal, usually greater than 95%.

    Although these surgeries are the most common, the exact surgeries your child needs will vary based on their diagnosis and body. The surgical team at Seattle Children’s will discuss the specific surgeries needed for your child in more detail.

Transitioning to Adult Care

As your child with tricuspid atresia grows into adulthood, we partner with the Adult Congenital Heart Disease Program through the University of Washington to provide a smooth transition to adult cardiologists who specialize in caring for patients with childhood heart disease. This ensures that your child has complete care throughout their lifetime.

Contact Us

Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.