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Tricuspid Atresia

Definition :
Congenital heart defects are problems in the heart present at birth. Tricuspid atresia is a rare congenital heart defect in which there's no valve between two of the heart's four chambers — the upper and lower right chambers. In addition, the lower right chamber is very small.

If your baby is born with tricuspid atresia, blood cannot flow from the body through the heart and into the lungs to pick up oxygen as it normally would. Because the path through the right side of the heart is blocked, blood flow to the lungs is dependent on holes in the walls between the heart's right and left sides.

As a result of tricuspid atresia, the lungs usually don't receive enough blood to supply the rest of the body with the oxygen it needs. This can cause shortness of breath and make babies who have tricuspid atresia tire easily.

Surgery to restore normal blood flow to the lungs is necessary in children with tricuspid atresia. Often, this requires several procedures in the child's first three or four years of life. With advances in care over the last several decades, the outlook for babies born with tricuspid atresia is better now than in the past.

Causes :
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 tricuspid atresia, in most cases the cause is unknown.

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.

Holes in the walls
In tricuspid atresia, the right side of the heart cannot 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, blood 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 usually closes soon after birth. If a baby with tricuspid atresia does not have an atrial septal defect, his or her doctor will give the baby medication to keep the foramen ovale open.

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 usually closes soon after birth. As with the foramen ovale, this passageway is kept open using medication in babies with tricuspid atresia. (When the foramen ovale and the ductus arteriosus are open, they are referred to as being "patent.")

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.

Risk Factor :
While the exact cause of tricuspid atresia is unknown, several factors may increase the risk of a baby being born with this condition. These include :

  • A mother who had German measles (rubella) or another viral illness during early pregnancy
  • A parent who has a congenital heart defect
  • Excessive alcohol consumption during pregnancy
  • A mother who has diabetes
  • Use of some types of medications during pregnancy, such as the acne drug isotretinoin (Accutane) and lithium (Eskalith), which is used to treat bipolar disorder

Children born with Down syndrome, a genetic condition resulting from an extra 21st chromosome, also are at increased risk of having tricuspid atresia and other congenital heart defects.

When to seek medical advice :
Seek medical help if you notice that your baby has the following signs or symptoms of tricuspid atresia :

  • Bluish color of the skin and lips (cyanosis)
  • Difficulty breathing (dyspnea)
  • Tiring easily, especially during feedings
  • Slow growth

Also, contact your doctor if your baby develops any of the following signs and symptoms of congestive heart failure :

  • Fatigue and weakness
  • Persistent cough or wheezing with white or pink blood-tinged phlegm
  • Swelling (edema) in the legs, ankles and feet
  • Swelling of the abdomen (ascites)
  • Sudden weight gain from fluid retention
  • Decreased alertness
  • Irregular or rapid heartbeat
Symptoms :
Seek medical help if you notice that your baby has the following signs or symptoms of tricuspid atresia :
  • Bluish color of the skin and lips (cyanosis)
  • Difficulty breathing (dyspnea)
  • Tiring easily, especially during feedings
  • Slow growth

Also, contact your doctor if your baby develops any of the following signs and symptoms of congestive heart failure :

  • Fatigue and weakness
  • Persistent cough or wheezing with white or pink blood-tinged phlegm
  • Swelling (edema) in the legs, ankles and feet
  • Swelling of the abdomen (ascites)
  • Sudden weight gain from fluid retention
  • Decreased alertness
  • Irregular or rapid heartbeat

Diagnosis :
Before birth
Because of advances in ultrasound technology, it's possible for a baby to be diagnosed with tricuspid atresia before he or she is born (in utero). Doctors can identify the condition on a routine ultrasound exam during pregnancy.

After birth
After your baby is born, his or her doctor may suspect a heart defect, such as tricuspid atresia, if your baby has a bluish cast to his or her skin or is having trouble breathing. Your baby's doctor may also suspect a heart defect if he or she hears a heart murmur — an abnormal whooshing sound caused by turbulent blood flow.

Doctors typically use an echocardiogram to diagnose tricuspid atresia. This test uses high-pitched sound waves that bounce off your baby's heart to produce moving images your baby's doctor can view on a video screen.

In a baby with tricuspid atresia, the echocardiogram reveals the absence of a tricuspid valve and a smaller than normal right ventricle. Because this test tracks blood flow, it can also measure the amount of blood moving through holes in the walls between the right and left sides of the heart. In addition, an echocardiogram can identify associated heart defects, such as an atrial septal defect or a ventricular septal defect.

Complications :
Potential complications of untreated tricuspid atresia include :

  • Lack of oxygen to tissues (hypoxemia). Tricuspid atresia may result in your baby's tissues getting too little oxygen, a condition that can be life-threatening.
  • Increased red blood cell count (polycythemia). If your baby has tricuspid atresia, his or her body may begin to produce a greater than normal number of red blood cells. This can interfere with normal blood flow, which may lead to blood clots, a heart attack or a stroke.

Complications later in life
Babies who have surgery to treat tricuspid atresia may have the following complications later in life :

  • 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)
  • Abnormal loss of protein from the digestive tract (protein-losing enteropathy)

Creating a tricuspid valve for a heart that doesn't have one isn't possible at this time. So instead of correcting the heart's anatomy, treatment for tricuspid atresia involves surgery to ensure adequate blood flow through the heart and into the lungs.

Before surgery, your baby's doctor may recommend that he or she take the medication prostaglandin to help widen (dilate) the blood vessels and keep the ductus arteriosus and the foramen ovale open.

Often, children with tricuspid atresia require more than one surgery. The goal of these surgeries is to create sufficient blood flow into and out of the heart and lungs, allowing your baby's body to receive the proper amount of oxygen-rich blood.

Fontan procedure. A surgery called the Fontan procedure is the best option for treatment of tricuspid atresia. During a Fontan surgery, the surgeon creates a path for the oxygen-poor blood returning to the heart to flow directly into the pulmonary arteries, which then pump the blood into the lungs. During this surgery, the surgeon will also close any holes between the chambers (septal defects) your baby's heart may have.

Most children with tricuspid atresia are not good candidates for the Fontan procedure until they are between 2 and 4 years old. Until then, other procedures and surgeries are often necessary to stabilize their medical condition and ensure proper growth.

The so-called rescue surgeries your baby may need before the Fontan procedure depend on how much blood is reaching his or her lungs. Following are some of the procedures babies with tricuspid atresia may require :

  • Atrial septostomy. This procedure creates or enlarges the opening between the heart's upper chambers (atria) to allow more blood to flow from the right atrium to the left atrium.
  • Pulmonary artery banding. Although the procedure is uncommon, your baby may require pulmonary artery banding if too much blood is being pumped to the lungs through a ventricular septal defect. Placing a band around the pulmonary artery limits the amount of blood that can flow through it. Banding protects the pulmonary artery from damage caused by excessive blood flow.
  • Shunting. Creating a bypass (shunt) from the main blood vessel leading out of the heart (aorta) to the pulmonary arteries allows for adequate blood flow to the lungs. Surgeons typically implant a shunt in the first six to eight weeks of life. However, babies usually outgrow this shunt in about six months. Your baby will then need another surgery to replace it.
  • Glenn procedure. When babies outgrown the first shunt, they often require a surgery that sets the stage for the Fontan procedure. Doctors usually perform the Glenn procedure when a child is about 6 months old. It connects one of the large veins that return blood to the heart (superior vena cava) to the pulmonary artery. This allows oxygen-poor blood to flow directly to the lungs. The procedure reduces the workload on the left ventricle, decreasing the risk of damage to it.

After surgery
To monitor his or her heart health, your baby will need lifelong follow-up care with a heart doctor (cardiologist) who specializes in congenital heart disease. Your child's cardiologist may recommend that your child limit physical activity and take antibiotics before dental procedures to prevent infections.

The long-term outlook for children who have a Fontan procedure isn't clear. In some cases, the circulation system created during the procedure may fail. In such instances, a heart transplant may be necessary.

In most cases, 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, before future pregnancies talk with a genetic counselor and a cardiologist experienced in congenital heart defects.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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