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.
Tricuspid Atresia Treatment Options
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 may need cardiac catheterization using a balloon to enlarge the opening between their atria.
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 (BT) 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%.
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%.
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 the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.