Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
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Monroe Carell Jr.
Children's Hospital
at Vanderbilt
2200 Children's Way
Nashville, TN 37232


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Tricuspid Atresia

 

What is Tricuspid Atresia?

The tricuspid heart valve allows oxygen-poor blood returning from the body to move from the right atrium to the right ventricle where it is pumped to the lungs.

In Tricuspid Atresia, the tricuspid valve fails to form. As a result, blood cannot pass into the right ventricle and into the lungs. This also results in underdeveloped pulmonary arteries and right ventricle. The only way for blood to pass through the heart is through a hole in the wall between the right and left atria. This hole is known as an Atrial Septal Defect (ASD). The oxygen-poor blood passes through the ASD to the left atrium where it mixes with the oxygen-rich blood returning from the lungs and then gets pumped through the left ventricle to the body. The child may appear slightly blue or “cyanotic” due to this mixing of blood.

How do we diagnose Tricuspid Atresia?

Patients with tricuspid atresia may present with cyanosis, trouble eating, or respiratory concerns. The diagnosis is made by an echocardiogram.

How do we treat Tricuspid Atresia?

Treatment for tricuspid atresia is usually a staged surgical approach involving different surgeries throughout the first two years of the child's life. If there is a large amount of blood flow to the lungs, the child may require a pulmonary artery band that will restrict the flow of blood to the lungs. If there is too little blood going to the lungs, the child may require a Blalock-Taussig (BT) shunt. It connects the subclavian artery to one of the pulmonary arteries. This allows more blood to flow to the lungs and may help the pulmonary arteries grow, and may also decrease the cyanosis.

The second procedure is usually the Glenn shunt, typically done between six to 12 months of age. This connects the superior vena cava directly to the pulmonary artery. It allows blood from the upper body to go directly to the lungs and bypass the heart.

The third stage is called the Fontan procedure. This is usually done around two years of ag. It connects the inferior vena cava to the pulmonary arteries. At this point, all of the blood returning from the body goes directly to the lungs without going through the heart. The heart is now a single pump, taking blood from the lungs and pumping it out to the body.


Last Edited: June 30, 2016
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