Tricuspid Atresia

From heart.org. ©2009, American Heart Association, Inc.

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

With tricuspid atresia, blue (oxygen-poor) blood comes from the body into the right atrium. Then, it passes through the ASD into the left atrium, where it mixes with red (oxygen-rich) blood. This mixed (purple) blood then flows into the left ventricle, which pumps it through the aorta to the brain and body. If the baby has a VSD, some of the mixed blood passes through the VSD into the right ventricle, which pumps it through the pulmonary artery to the lungs.

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

Usually, the ductus arteriosus 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, doctors may start giving a medicine called prostaglandin right after birth to keep the ductus arteriosus open.

Some babies with tricuspid atresia have other conditions that also affect blood flow through their heart. These may include pulmonary stenosis or transposition of the great arteries. Both of these conditions can complicate the diagnosis and affect the type of surgery a baby needs.

Keeping the ductus arteriosus open with medicine is a good short-term way to get blood to the lungs. But often, babies need a more lasting connection. This is called a Blalock-Thomas-Taussig (BTT) shunt.

The BTT shunt is a small plastic tube that connects the aorta (blood supply to the body) and the pulmonary artery (blood supply to the lungs). A surgeon sews the tube in place, or a shunt can be placed in a cardiac catheterization procedure.

If your baby needs a shunt, we do this soon after birth — typically in the first 1 to 2 weeks. After this surgery, the oxygen levels in the body will still be lower than normal, usually around 80%.

The bidirectional Glenn is a surgery that changes blood flow from a large vein called the superior vena cava. Normally, this vein carries blue (oxygen-poor) blood from the upper part of the body into the heart, which pumps the blood to the lungs. After the surgery, the blue blood flows to the lungs without going through the heart first.

Newborns cannot have this surgery because the blood pressure in their lungs is too high. We usually do this surgery around age 3 to 6 months, when the blood pressure in the lungs has reached its normal low level. After this surgery, the oxygen level in the body will still be lower than normal, usually around 80%.

This surgery is sometimes called the hemi-Fontan.

The final planned surgery for tricuspid atresia is called the Fontan operation. This surgery changes blood flow from a large vein called the inferior vena cava. Normally, this vein carries blue blood from the lower part of the body into the heart, which pumps it to the lungs. After the surgery, all the blue blood returning from the body flows to the lungs without going through the heart first.

When the Fontan surgery is done, the flow of blood to the lungs is completely separate from the flow to the body. The oxygen level in the body is normal, usually greater than 95%. After your child has a Fontan procedure, they get coordinated, ongoing, team-based care through our Fontan Clinic.

• The Heart Center team includes more than 40 pediatric cardiologists who diagnose and treat every kind of heart problem. We have treated many children with tricuspid atresia.
• Our doctors and surgeons are experts in the treatments your child may need, including cardiac catheterizations and surgery. Seattle Children’s outcomes for surgeries to change the flow of blood to the heart and lungs are among the best in the nation.
• Children with tricuspid atresia receive comprehensive care through our Single Ventricle Program.
• We also have a pediatric cardiac anesthesia team and Cardiac Intensive Care Unit for children who need them. General anesthesia is a medicine we give to people before surgery so they are fully asleep during the procedure.
• Your child’s team includes other experts from Seattle Children’s based on their needs, like neonatologists and pulmonologists.

• Most developing babies with tricuspid atresia are diagnosed before birth. If this happens, Seattle Children’s Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for any care your baby may need.
• Your child’s treatment plan is custom-made. We plan and carry out their treatment based on the specific details of their heart condition. We closely check your child’s needs to make sure they get the care that is right for them at every age.
• We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.

• We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
• Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
• Our Child Life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
• Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make your experience as smooth as possible.
• Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
• Read more about the supportive care we offer.