Tricuspid Atresia

Heart With Tricuspid Atresia

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Atresia is defined as the congenital absence of a normal opening. In the case of tricuspid atresia, the tricuspid valve is not present resulting in a lack of communication between the right atrium and the right ventricle. Consequently, the right ventricle is small and underdeveloped. An associated ASD allows blood to be diverted from the right to the left atrium. Survival depends on the presence of a VSD or on the ductus arteriosus remaining open after birth in order for blood to reach the lungs.

Corrective surgery is done in a series of three stages. The first stage, performed in the first days of life, is known as a Shunt Operation. During this procedure, a small tube (shunt) is placed between the aorta and the branch pulmonary artery. This helps increase blood flow to the lungs. The second and third stages are collectively known as the Fontan Procedure. The second procedure, performed at approximately six months of age, creates a Bi-directional Cavo Pulmonary Connection (BCPC).

This involves connecting the superior vena cava to the right pulmonary branch artery. The BCPC allows the deoxygenated blood of the upper half of the body to flow directly to the lungs. The shunt placed during the first stage operation is also ligated at this time. The third and final stage is performed at approximately 12 months of age. During this procedure, the deoxygenated blood of the lower half of the heart is directed to the lungs. This is done by channeling the blood of the inferior vena cava through the right atrium to the right branch pulmonary artery.


What Is Normal Cardiac Anatomy?

When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

 
Learn More About Normal Cardiac Anatomy

Heart With Normal Cardiac Anatomy

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When your child has a congenital heart defect, there's usually something wrong with the structure of his or her heart's structure.

The heart is composed of four chambers. The two upper chambers, known as atria, collect blood as it flows back to the heart. The two lower chambers, known as ventricles, pump blood with each heartbeat to the two main arteries (the pulmonary artery and the aorta). The septum is the wall that divides the heart into right and left sides. The atrial septum separates the right and left atria; likewise, the ventricular septum separates the two ventricles.

There are four valves that control the flow of blood through the heart. These flap-like structures allow blood to flow in only one direction. The tricuspid and mitral valves, also known as the atrioventricular valves, separate the upper and lower chambers of the heart. The aortic and pulmonary valves, also known as the arterial valves, separate the ventricles from the main arteries. Oxygen-depleted blood returns from the body and drains into the right atrium via the superior and inferior vena cavas. The blood in the right atrium then passes through the tricuspid valve and enters the right ventricle.

Next, the blood passes through the pulmonary valve, enters the pulmonary artery, and travels to the lungs where it is replenished with oxygen. The oxygen-rich blood returns to the heart via the pulmonary veins, draining into the left atrium. The blood in the left atrium passes through the bicuspid, or mitral, valve and enters the left ventricle.

Finally, the oxygen-rich blood flows through the aortic valve into the aorta and out to the rest of the body.

Tetralogy of Fallot

Tetralogy of Fallot (fah-LO) is a combination of problems caused by a birth defect in the structure of the heart that changes the way blood flows through the heart.

Each year, 4 out of every 10,000 babies born in the United States have the condition, which was named after the French doctor who first described it in the late 1800s, Étienne Fallot. About 10% of all babies born with a heart problem have tetralogy of Fallot (TOF).

The four related problems that together make up tetralogy of Fallot are:

  1. Ventricular septal defect (VSD), which is a hole in the septum, or wall, separating the two lower chambers, or ventricles, of the heart. The septum acts as a barrier that prevents blood from both sides of the heart from mixing together. But when there is a VSD, blood high in oxygen from the left ventricle can mix with blood low in oxygen from the right ventricle. VSD is the defect that can lead to the other problems associated with TOF.
  2. Pulmonary stenosis, a narrowing or thickening of the valve that connects the right ventricle to the pulmonary artery, a blood vessel that carries low-oxygen blood from the heart to the lungs, where the blood receives more oxygen and then returns to the heart. With pulmonary stenosis, the heart has to work harder than normal to pump blood to the lungs. Often, the amount of blood reaching the lungs is below normal.
  3. Right ventricular hypertrophy (hi-PER-truh-fee), which is a thickening of the muscular wall of the right ventricle. The thickened wall can block the flow of blood through the pulmonary valve, which allows blood from the heart to flow into the lungs.
  4. An "overriding aorta," which means the artery that carries high-oxygen blood to the body is out of place and arises above both ventricles, instead of just the left ventricle, as in a healthy heart. This allows some blood that is low in oxygen to flow into the aorta and out to the body, instead of to the pulmonary artery, which would normally take it to the lungs to pick up oxygen.

As a result of these defects, too little blood goes to the lungs. The low-oxygen blood then circulates to the rest of the body and too little oxygen reaches the body tissues.

If tetralogy of Fallot goes untreated, a child may have:

  • dizziness, fainting, or seizures
  • a higher risk of developing endocarditis, an infection of the inner layer of the heart
  • high pressure in the right side of the heart that can cause an irregular heartbeat, called an arrhythmia

A child whose TOF is not repaired might need to limit participation in competitive sports and other physical activities. Many infants who have surgery to correct the defect do very well, participate in normal kid activities, and live to adulthood.

Causes

Scientists have not yet identified a specific cause for tetralogy of Fallot in all cases, but they believe genetics play a role. Someone born with TOF is more likely to have a child or sibling with it.

Mothers who get rubella (German measles) or other viral illnesses during their pregnancies are at a higher risk of giving birth to babies with TOF. Other risk factors and conditions include poor nutrition, alcohol abuse, uncontrolled diabetes, and the mother's age (over 40).

Most of the time, a child with TOF does not have any other birth defects. But children who have certain genetic disorders, such as Down syndrome, often have congenital heart defects, including TOF.

Certain environmental factors, such as air pollution, also may increase a mother's chances of having a baby with TOF.

Signs

One of the most common signs of tetralogy of Fallot is cyanosis (a blue or purple tint to the baby's skin, lips, and fingernails). Healthy babies can sometimes also have a bluish skin tone around the mouth or around the eyes from prominent veins under the skin. This is perfectly normal. If a baby's lips and tongue look pink, you generally don't have to be concerned because babies who have low oxygen levels in the blood usually have blue lips and tongues in addition to bluish skin.

A child with TOF might have sudden episodes of deep cyanosis, called "Tet spells," during crying or feeding. Older children who have Tet spells will often instinctively squat down, which helps to stop the spell.

Other signs include:

  • heart murmur
  • fussiness
  • easy tiring with exertion
  • difficulty breathing
  • fatigue (tiredness)
  • rapid heartbeat (palpitations)
  • fainting
  • "clubbing," where the skin or bones around the tips of fingers are widened or rounded

Diagnosis

Your doctor may use several tests to find out if your child has tetralogy of Fallot, including:

  • pulse oximeter: a small sensor that clips onto the fingertip, toe, or ear and measures how much oxygen is in the blood
  • electrocardiogram (or EKG): a test that records the electrical activity of the heart
  • echocardiogram, or "echo": an ultrasound picture of the heart structures (chambers, walls, and valves). It records the motion of the blood through the heart and can measure the direction and speed of blood flow within the heart structures.
  • chest X-ray
  • cardiac catheterization: a thin, flexible tube called a catheter is inserted into the heart, usually through a vein in the leg or arm, and provides information about the heart structures as well as blood pressure and blood oxygen levels within the heart chambers. Sometimes a device will be inserted into the heart or blood vessels through the heart catheter.

Treatment

Tetralogy of Fallot is repaired through open-heart surgery soon after birth or later in infancy, depending on the baby's health and weight and severity of defects and symptoms.

The two surgical options are:

  1. Complete repair: the surgeon widens the passageway between the right ventricle and the pulmonary artery to improve blood flow to the lungs. The ventricular septal defect is patched to stop the mixing of high-oxygen blood with low-oxygen blood between the ventricles.

    These repairs also fix the two remaining defects (overriding aorta and right ventricular hypertrophy). Because the right ventricle doesn't have to work as hard to pump blood into the lungs, the thickness of the ventricle wall will decrease. And the patched VSD prevents blood with low oxygen from flowing into the aorta.
  2. Temporary or palliative surgery: minor repairs are made to improve blood flow to the lungs. This usually only occurs when the baby is too weak or small to undergo full surgery. In the temporary surgery, the surgeon creates a secondary route for blood to travel to the lungs for oxygen. This is done by placing a small tube, called a shunt, between a large artery branching off the aorta and the pulmonary artery.

    Later when the baby grows stronger, the full repair is performed.

Most babies born with tetralogy of Fallot do very well and survive to adulthood, but require regular follow-up with a heart specialist.

Reviewed by: Gina Baffa, MD
Date reviewed: October 24, 2016