Overview
Diagnosis
Tricuspid atresia may be diagnosed during a routine pregnancy ultrasound before birth. Proper prenatal care is important throughout pregnancy.
After birth, a healthcare professional examines the baby and listens to the heart and lungs. Signs that may indicate tricuspid atresia include blue or gray skin, difficulty breathing, or an irregular heart sound called a murmur, caused by changes in blood flow to and from the heart.
Tests to diagnose tricuspid atresia may include:
• Echocardiogram – Uses sound waves to create moving images of blood flow through the heart and valves. It can show a missing tricuspid valve and other heart abnormalities.
• Electrocardiogram (ECG or EKG) – Records the electrical activity of the heart and detects irregular heart rhythms.
• Pulse oximetry – A sensor on a hand or foot measures oxygen levels in the blood.
• Chest X-ray – Shows the condition of the heart and lungs, including heart size and fluid buildup.
• Cardiac catheterization – A thin catheter is inserted into a blood vessel and guided to the heart. Dye injected through the catheter allows X-ray imaging of the heart chambers and measures pressures. This test is rarely needed for diagnosis but may be used before surgery.
Treatment
There is no way to replace the tricuspid valve in tricuspid atresia. Treatment focuses on improving blood flow through the heart and lungs, managing symptoms, and performing necessary surgeries.
Medications
Medications may be used to:
• Strengthen the heart muscle
• Lower blood pressure
• Remove excess fluid from the body
Supplemental oxygen may help the baby breathe better. Before surgery, prostaglandin may be given to keep the ductus arteriosus open.
Surgeries or procedures
Babies with tricuspid atresia often need multiple heart surgeries, some as temporary fixes before permanent correction:
• Shunting – Creates a new pathway for blood to flow to the lungs, improving oxygen levels. Usually placed in the first two weeks of life and may need replacement later.
• Glenn procedure – Connects a large vein directly to the lung artery, reducing strain on the heart’s lower left chamber. Prepares for the Fontan procedure.
• Fontan procedure – Usually done between ages 2 and 5, it directs most blood that would go to the right heart into the pulmonary artery.
• Pulmonary artery band placement – Reduces blood flow from the heart to the lungs if a ventricular septal defect is present.
• Atrial septostomy – Rarely, a balloon enlarges the opening between the heart’s upper chambers to improve blood flow.
Follow-up care
Babies and adults with tricuspid atresia need lifelong follow-up with a cardiologist trained in congenital heart conditions. Regular checkups are essential to monitor for complications such as heart failure. Many children treated for tricuspid atresia grow up to lead full, active lives.
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