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.
Tricuspid atresia symptoms include:
Most babies who have tricuspid atresia show symptoms within the first week after birth.
Some babies with tricuspid atresia may also develop symptoms of heart failure, including:
When to see a doctor
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
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. (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.
Chambers and the valves of the heart
A normal heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood ...
In tricuspid atresia, blood can't flow from the right atrium to the right ventricle because the valve between them is missing. This condition often includes a smaller than normal right ventricle, as ...
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:
Prompt treatment helps avoid potentially fatal complications of tricuspid atresia, including:
Complications later in life
Preparing for your appointment
A congenital heart defect such as tricuspid atresia is often identified within the first week of life. Whether problems are first noted in the hospital or later by you and your primary care doctor, eventually your baby will be seen by a cardiologist who has experience in treating congenital heart defects.
Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared for your appointment with the cardiologist. Here's some information to help you get ready, and what to expect from your cardiologist.
What you can do
Preparing a list of questions can help you ensure that you cover all of the points you want to. For a congenital heart defect, some basic questions to ask your child's cardiologist include:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask additional questions during your appointment.
What to expect from your doctor
Tests and diagnosis
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.
Treatments and drugs
There's currently no way to replace the defective tricuspid valve. Treatment for tricuspid atresia involves surgery to ensure adequate blood flow through the heart and into the lungs, allowing your baby's body to receive the proper amount of oxygen-rich blood. Often, this requires more than one surgical procedure.
However, most children with tricuspid atresia don't undergo the Fontan procedure until they are at least 2 years old.
Some children with tricuspid atresia aren't good candidates for the Fontan procedure. If that is the case, your child's doctors will discuss other options, including the possibility of a heart transplant.
The short- and intermediate-term outlook for children who have a Fontan procedure is generally promising. Outcomes for those having surgery later in life are generally worse. A variety of complications may occur over time and sometimes require additional procedures. If the circulation system created by the Fontan procedure fails, then a heart transplant may be necessary. Talk to your child's doctor about his or her specific situation.
Lifestyle and home remedies
If your baby's born with tricuspid atresia, it may seem that almost all your time is spent at the hospital or at a doctor's office. But there will be time spent at home, as well. Here are some tips for caring for your child at home.
Coping and support
Caring for a baby with a serious heart problem, such as tricuspid atresia, can be challenging and stressful. Here are some strategies that may help make it easier:
Although every circumstance is different, remember that many children with congenital heart defects, such as tricuspid atresia, grow up to lead enjoyable lives.
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:
Last Updated: 2010-07-21
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