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Tricuspid Atresia

In a baby with a normal heart, the right side of the heart pumps oxygen-poor blood from the heart to the lungs through the pulmonary artery. The oxygen-rich blood that comes back from the lungs is then pumped to the rest of the body through the aorta.

In babies with tricuspid atresia, the tricuspid valve that controls blood flow from the right atrium to the right ventricle is missing. Blood cannot get to the right ventricle and out to the lungs. As a result, the body is not getting enough oxygen. The right ventricle may be underdeveloped. The main pulmonary artery may also be small. Since the blood cannot directly flow from the right atrium to the right ventricle, it must use other, less efficient routes.

Babies born with tricuspid atresia often have other congenital heart defects like an atrial septal defect, a hole between the right and left atria, or a ventricular septal defect, a hole between the right and left ventricles. These defects allow oxygen-rich blood to mix with oxygen-poor blood so that oxygen-rich blood has a way to get pumped to the rest of the body. But this blood flow is inefficient.

Some babies with tricuspid atresia can also have other heart defects, including transposition of the great arteries.

What happens once you have tricuspid atresia?

Babies born with tricuspid atresia may have ashen or bluish-looking skin color, called cyanosis, because their blood doesn’t carry enough oxygen.

Additional symptoms include: poor feeding, problems breathing, and extreme sleepiness.

What causes tricuspid atresia?

The causes of heart defects, such as tricuspid atresia, among most babies are unknown. Some babies have heart defects because of changes in their genes or chromosomes. Heart defects may also be caused by the environment, what the mother eats or drinks, or certain medicines she uses.

What types of tests are used to diagnosis tricuspid atresia?

Tricuspid atresia may be diagnosed during pregnancy or soon after a baby is born.

During pregnancy, tricuspid atresia might be seen during a fetal echocardiogram specifically for the baby’s heart and major blood vessels during the pregnancy. This test can show problems with the structure of the heart and how the heart is working with this defect.

Babies born with tricuspid atresia will show symptoms such as bluish skin or problems breathing. Using a stethoscope, a doctor can hear a heart murmur (an abnormal “whooshing” sound caused by blood not flowing properly) or other sounds that may indicate a heart problem.

If a doctor suspects that there might be a problem, the doctor can request one or more tests:

  • Echocardiogram: This ultrasound can show problems with the structure of the heart.

  • Cardiac catheterization: This test involves looking at the inside of the heart with a catheter (thin tube) and measuring the blood pressure and oxygen.

  • Electrocardiogram (EKG): This test measures the electrical activity of the heart.

  • Pulse oximetry: A pulse oximeter, with sensors placed on the baby’s skin, estimates the amount of oxygen in a baby’s blood.

What types of treatments and procedures are used to treat tricuspid atresia?

Some babies and children will need medicines to help strengthen the heart muscle, lower their blood pressure, and help the body get extra fluid.

Because your baby may not be feeding well, a special high-calorie formula might be prescribed. Some babies become extremely tired while feeding and might need to be fed through a feeding tube.

One or more surgeries may be needed to increase blood flow to the lungs and restore heart function.

  • Shunt procedure: Surgeons create a bypass (shunt) from the aorta to the main pulmonary artery, allowing blood to get to the lungs. If the aorta is small, the surgeon will also enlarge the aorta.

  • Banding: Sometimes, there is too much blood flowing to the lungs. The surgeon may place a band around the artery going to the lungs (main pulmonary artery) to control the blood flow to the lungs. This is usually a temporary procedure.

  • Septostomy: A septostomy creates or enlarges the atrial septal defect, the hole between the right and left upper chambers (atria).

  • Bi-directional Glenn procedure: This procedure creates a direct connection between the main pulmonary artery and the superior vena cava, the vessel returning oxygen-poor blood from the upper part of the body to the heart.

  • Fontan procedure: This procedure usually is done sometime around two years of age. Doctors connect the main pulmonary artery and the inferior vena cava, the vessel returning oxygen-poor blood from the lower part of the body to the heart, allowing the rest of the blood coming back from the body to go to the lungs.

What can I do to support my health when I have tricuspid atresia?

Infants who have these surgeries can not be cured, but treatment can help. They might still have lifelong complications. If the heart becomes weak after treatment, an infant might need a heart transplant. An infant or child who receives a heart transplant will need to take medicines for the rest of their life.

Babies born with tricuspid atresia will need regular follow-up visits with a cardiologist (heart doctor) to monitor their progress. As adults, they may need more surgery or medical care for other possible problems.

Why choose Presbyterian for tricuspid atresia treatment?

Presbyterian’s Pediatric and Congenital Cardiology team has many different options to help you manage your or your child’s heart condition. The team performs various diagnostic tests and procedures to help form an accurate diagnosis and create individualized treatment plans. Depending on the type of heart condition your child has and its underlying cause, the team can recommend a wide variety of treatment options. Our pediatric cardiologists, pediatric interventional cardiologists, and pediatric cardiovascular surgeons work closely together for cases in which cardiac repair or surgery is the best treatment option.