What is tricuspid atresia?
The tricuspid valve is the opening between the right atrium (the upper chamber) and the right ventricle (the lower chamber). In a normal heart, the right side receives oxygen-poor blood (blue blood) from the body’s veins. It pumps the blue blood to the lungs to receive oxygen. The oxygen-rich blood (red blood) returns from the lungs to the left side of the heart, which pumps the blood to the body.
A heart with the congenital heart disease tricuspid atresia is characterized by poorly developed right heart structures and has:
- No tricuspid valve
- A smaller-than-normal right ventricle/hypoplastic right ventricle
- An atrial septal defect (a hole between the right atrium and left atrium) so oxygen-poor and oxygen-rich 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 in children is often associated with pulmonary stenosis or narrowing of the pulmonary valve. Tricuspid atresia can also be associated with transposition of the great arteries, where the aorta — the large artery that carries blood to the body — arises from the small right ventricle, and the pulmonary artery (lung artery) arises from the large left ventricle.
Tricuspid atresia is a single-ventricle lesion, because the heart has only one functioning ventricle (the left ventricle).
Signs and symptoms of tricuspid atresia
Tricuspid atresia symptoms in children include:
- Cyanosis: a 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
- Abnormal shape of the fingertips (“clubbing”) in older children
Testing and diagnosis of tricuspid atresia
Tricuspid atresia is usually diagnosed a few hours or days after birth. Pediatricians from other hospitals refer newborns to the Cardiac Center when the baby shows symptoms or signs that may indicate a heart problem, such as a blue tint to the skin or a heart murmur.
Some or all of these tests may be required for diagnosis of tricuspid atresia in children:
- 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”)
Treatment for tricuspid atresia
Your baby will be admitted to the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU), where he will receive round-the-clock attention from a team of dedicated cardiac critical care medicine specialists. Your baby may require oxygen and a medication called prostaglandin to maintain an adequate oxygen level in the blood. Prostaglandin is an intravenous medication that keeps 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 red blood to the body) open.
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 turn quite blue. An infusion of prostaglandin can re-open the PDA and is a life-saving intervention. Not all babies with tricuspid atresia require prostaglandin.
If your baby has labored breathing or poor effort, he or she may need 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 — the large vein attached to the heart to return deoxygenated or blue blood from the upper half of the body — is closed off or disconnected from the heart and attached to the pulmonary artery. During this operation, the surgeon also closes the BT shunt. After the procedure, deoxygenated or blue blood from the upper body goes directly to the lungs without passing through the heart.
The third operation, called the Fontan, occurs at approximately 1 1/2 to 3 years of age. During this surgery, blood from the inferior vena cava — the large vein that returns deoxygenated blood to the heart from the lower half of the body — is diverted directly 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 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.
Outlook for tricuspid atresia
Because of enormous strides in medicine and technology, today many children born with tricuspid atresia go on to lead healthy, productive lives as adults.
Between the Norwood and Glenn operations
Though early outcomes for patients with single ventricle heart defects after staged reconstruction have improved dramatically, the period between the Norwood procedure and the Glenn operation remains a very vulnerable time for infants. CHOP created the Infant Single Ventricle Monitoring Program to focus on the care and monitoring of infants with single ventricle heart defects between the first and second reconstructive surgeries.
Through age 18
Children with tricuspid atresia require life-long care by a cardiologist. Many remain on medications for life. Additional surgeries may be required.
As “single ventricle survivors” get older, doctors are recognizing that, while some do fine, many experience complications, including lung, liver and gastrointestinal diseases. The Cardiac Center at CHOP created the Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program, to bring together doctors from different specialties to care for single ventricle survivors.
In addition, as a group, children with complex congenital heart defects who have had open heart surgery as infants are at a higher risk for neurodevelopmental issues when compared to children without congenital heart defects. The Cardiac Center at CHOP created the Cardiac Kids Developmental Follow-up Program to provide evaluation, screening and clinical care for children with complex congenital heart disease who are at risk for neurodevelopmental problems.
Our pediatric cardiologists follow your child until he is a young adults, coordinating care with his primary care physicians.
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 Children’s Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.
Reviewed by Paul M. Weinberg, MD, FAAP, FACC