Tricuspid Atresia
Before you begin reading about this heart defect, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
- What is tricuspid atresia?
- What are the symptoms?
- How is it diagnosed?
- What are the treatment options?
- What kind of follow-up care is required?
What is tricuspid atresia?
The right side of the normal heart receives oxygen-poor blood/blue 
Tricuspid Atresia
Enlarge blood from the body’s veins and pumps it to the lungs to receive oxygen. The oxygen-rich blood/pink blood returns from the lungs to the left side of the heart, which pumps the blood to the body. The tricuspid valve is the opening between the right atrium (the upper chamber) and the right ventricle (the lower chamber) A heart with tricuspid atresia is characterized by poorly developed right heart structures and:
- has no tricuspid valve
- has a smaller-than-normal right ventricle/hypoplastic right ventricle.
- Children with tricuspid atresia always have an atrial septal defect, a hole between the right atrium and left atrium, so that oxygen-poor and oxygen-rich blood (blue and pink blood) mix inside of the heart. They usually also have a ventricular septal defect, a hole between the right ventricle and left ventricle.
Tricuspid atresia is often associated with pulmonary stenosis or narrowing of the pulmonary valve, or pulmonary atresia where the pulmonary valve is completely closed. Tricuspid atresia can also be associated with transposition of the great arteries, where the aorta, the large artery that carries blood to the body, is connected to the small right ventricle.
Tricuspid atresia is a single-ventricle lesion, because the heart has only one functioning ventricle (the left ventricle).
What are the symptoms?
The symptoms include:
- cyanosis or blue or purple tint to lips, skin and nails
- heart murmur – the heart sounds abnormal when a doctor listens with a stethoscope
- shortness of breath
- difficulty feeding
- poor weight gain
- fatigue
- abnormal shape of the fingertips (“clubbing”) in older children
How is it diagnosed?
Tricuspid atresia may be diagnosed before birth, with fetal echocardiogram. Our Fetal Heart Program can prepare a plan for delivery and care immediately after birth.
Tricuspid atresia is usually diagnosed a few hours or days after birth. Pediatricians from other hospitals refer newborns to the Cardiac Center when they notice symptoms and signs such as a “blue baby with a heart murmur.” Pulse oximetry is a painless way to monitor the oxygen level of the blood.
Some or all of these tests may be required for diagnosis:
- chest X-ray
- blood tests
- electrocardiogram (EKG) – this test shows the electrical activity of the heart
- echocardiogram (also called “echo” or ultrasound) – sound waves create an image of the heart. This test usually confirms the diagnosis.
- cardiac catheterization – a thin tube is inserted into the heart through a vein and/or artery in either the leg or through the umbilicus (“belly button”)
What are my child’s treatment options?
Your baby will be admitted to the Tabas Cardiac Intensive Care Unit (CICU). The baby may require oxygen, and a medication called prostaglandin to maintain adequate oxygen level in the blood. Prostaglandin is an intravenous medication that keeps open the connection between the pulmonary artery (the artery that normally carries blue blood to the lungs to receive oxygen) and the aorta (the artery that carries pink blood to the body). This connection, called PDA or patent ductus arteriosus, is open in the fetus, and closes soon after birth. When the PDA closes, some babies with tricuspid atresia get quite blue/cyanosed. An infusion of prostaglandin can re-open the PDA and is a life saving intervention. Not all babies with tricuspid atresia require prostaglandin.
If the baby has labored breathing or poor effort, he or she may need help with a breathing machine or ventilator. It is not uncommon for babies to have poor respiratory effort or apnea while on prostaglandin infusion.
At least two and possibly three surgeries will be required.
- Babies who require prostaglandin to maintain adequate oxygen level will require surgery soon after birth. The surgery involves creation of a “shunt,” which is a tube that connects one of the branches of the aorta and the pulmonary artery, and thus replaces the PDA. This operation is called the Blalock-Taussig shunt or BT shunt. Many babies with tricuspid atresia are well enough to be discharged home soon after birth. However, some of these babies may require the “shunt” operation at a few weeks of life if the level of oxygen in their blood is decreasing.
Some babies with tricuspid atresia are too “pink” or have too much blood-flow to the lungs, and will require an operation called “pulmonary artery banding” to narrow the pulmonary artery and regulate blood flow to the lungs. Babies with tricuspid atresia and transposition of great arteries may require the “Norwood operation” if the aorta is too small (see hypoplastic left heart syndrome).
- The second operation, called the hemi-Fontan/Glenn operation usually occurs within six months of birth. During this surgery, the superior vena cava, one of the two large veins attached to the heart to return deoxygenated or blue blood from the upper half of the body, is disconnected from the heart and attached to the pulmonary artery. During this operation, the surgeon also removes the BT shunt. After this operation, deoxygenated or blue blood from the upper body goes to the lungs without passing through the heart.
- The third operation, called the Fontan, occurs at approximately one-and-a-half to three years of age. During this surgery, the inferior vena cava, the other large vein that returns deoxygenated blood to the heart from the lower half of the body, is disconnected from the heart and attached to the pulmonary artery. This means that deoxygenated or blue blood from the whole body goes to the lungs without passing through the heart.
After these operations, deoxygenated blood (blue blood) flows to the lungs without passing through the right side of the heart. The Cardiac Center team will explain the surgical procedures to you in more detail, based on your child’s heart anatomy.
Of historical interest, the Fontan operation was performed for the first time in patients with tricuspid atresia.
What kind of follow-up care will my child need?
Through 18
Children with tricuspid atresia require life-long care by a cardiologist. Many remain on medications for life. Additional surgeries may be required.
Our pediatric cardiologists follow patients until they are young adults, coordinating care with the primary care physicians.
Into Adulthood
It’s important that your child continue to see a cardiologist as an adult. We will help with the transition to an adult cardiologist.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children’s Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.
Because of enormous strides in medicine and technology, today many children with heart conditions go on to lead healthy, productive lives as adults.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Chitra Ravishankar, MD
Date: August 2009
Contact Us
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267-426-9600
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