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Tricuspid atresia

Tricuspid atresia is a heart defect present at birth (congenital) in which one of the valves (tricuspid valve) between two of the heart's chambers isn't formed. Instead, there's solid tissue between the chambers.

If your baby is born with tricuspid atresia, blood can't flow through the heart and into the lungs to pick up oxygen as it normally would. The result is the lungs can't supply the rest of your baby's body with the oxygen it needs. Babies with tricuspid atresia tire easily, are often short of breath and have blue-tinged skin.

Tricuspid atresia is treated with surgery. Most babies with tricuspid atresia who have surgery will live well into adulthood, though follow-up surgeries are often needed.

Symptoms Causes Risk factors Complications Prevention

Tricuspid atresia symptoms become evident soon after birth, and can include:

  • Blue tinge to the skin and lips (cyanosis)
  • Difficulty breathing (dyspnea)
  • Tiring easily, especially during feedings
  • Slow growth

Some babies with tricuspid atresia may also develop symptoms of heart failure, including:

  • Fatigue and weakness
  • Shortness of breath
  • Swelling (edema) in the legs, ankles and feet
  • Swelling of the abdomen (ascites)
  • Sudden weight gain from fluid retention
  • Irregular or rapid heartbeat

When to see a doctor

Tell your doctor if you notice any of the above symptoms in your child.

Tricuspid atresia occurs during fetal growth when your baby's heart is developing. While some factors, such as heredity or Down syndrome, may increase your baby's risk of congenital heart defects, such as tricuspid atresia, the cause of congenital heart disease is unknown in most cases.

The normal-functioning heart

Your heart is divided into four chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout the body — your heart uses its left and right sides for different tasks. The right side moves blood to the lungs. In your lungs, oxygen enriches the blood, which then circulates to your heart's left side. The left side of the heart pumps blood into a large vessel called the aorta, which circulates the oxygen-rich blood to the rest of your body. Valves control the flow of blood into and out of the chambers of your heart. These valves open to allow blood to move to the next chamber or to one of the arteries, and they close to keep blood from flowing backward.

When things go wrong

In tricuspid atresia, the right side of the heart can't properly pump blood to the lungs because the tricuspid valve, located between the upper right chamber (atrium) and the lower right chamber (ventricle), is missing. Instead, a solid sheet of tissue blocks the flow of blood from the right atrium to the right ventricle. As a result, the right ventricle is usually very small and underdeveloped (hypoplastic).

Blood instead flows from the upper right chamber to the upper left chamber through a hole in the wall between them (septum). This hole is either a heart defect (atrial septal defect) or an enlarged natural opening (foramen ovale) that is supposed to close soon after birth. If a baby with tricuspid atresia doesn't have an atrial septal defect, the baby may need a procedure to create this opening.

Once the blood flows from the right atrium to the left atrium, the heart's left side must pump blood both to the rest of the body and to the lungs. The blood reaches the lungs from the left side of the heart through another natural opening between the right and left sides (ductus arteriosus) that is supposed to close soon after birth. When necessary, this passageway is kept open using medication in babies with tricuspid atresia.

Some babies with tricuspid atresia have another heart defect — a hole between the lower two chambers (ventricular septal defect). In these cases, blood can flow through the hole and into the right ventricle, which pumps it to the lungs.

In most cases, the exact cause of a congenital heart defect, such as tricuspid atresia, is unknown, but several factors may increase the risk of a baby being born with this condition:

  • A mother who had German measles (rubella) or another viral illness during early pregnancy
  • A parent who has a congenital heart defect
  • Drinking alcohol during pregnancy
  • A mother who has poorly controlled diabetes
  • Use of some types of medications during pregnancy, such as the acne drug isotretinoin (Claravis, Sotret) and some anti-seizure medications
  • The presence of Down syndrome, a genetic condition that results from an extra 21st chromosome

The most significant complication of tricuspid atresia is a lack of oxygen to your baby's tissues (hypoxemia). This can be life-threatening.

Complications later in life

Although treatment greatly improves the outcome for babies with tricuspid atresia, they may still have the following complications later in life, even after surgery:

  • Formation of blood clots that may lead to a clot blocking an artery in the lungs (pulmonary embolism) or to a stroke
  • Easily tiring when participating in sports or other exercise
  • Heart rhythm abnormalities (arrhythmias)

In most cases, congenital heart defects, such as tricuspid atresia, can't be prevented. If you have a family history of heart defects or if you already have a child with a congenital heart defect, a genetic counselor and a cardiologist experienced in congenital heart defects can help you look at possible risks associated with future pregnancies.

Some steps you can take that might reduce your baby's risk of heart and other birth defects in pregnancy include:

  • Get adequate folic acid. Take 400 micrograms of folic acid daily. This amount, which is often already in prenatal vitamins, has been shown to reduce brain and spinal cord defects, and folic acid may help prevent heart defects, too.
  • Talk with your doctor about medication use. Whether you're taking prescription or over-the-counter drugs, an herbal product or a dietary supplement, check with your doctor before using them during pregnancy.
  • Avoid chemical exposure, whenever possible. While you're pregnant, it's best to stay away from chemicals, including cleaning products and paint, as much as you can.
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