Tricuspid atresia, a condition you’re born with, is a heart problem in which your tricuspid valve doesn’t exist. Without this valve, blood can’t flow normally from your upper to lower chambers on the right side of your heart. Multiple surgeries at different ages help a baby’s heart work better. After surgeries, many people live into adulthood.
Tricuspid atresia is a congenital (present at birth) heart defect that occurs when the tricuspid valve of the heart doesn’t form. The tricuspid valve is normally between two chambers on the right side of your heart, the right atrium (upper chamber) and right ventricle (lower chamber).
In tricuspid atresia, you have solid tissue instead of the tricuspid valve. This sheet of tissue blocks blood flow from the right atrium to the right ventricle, where blood would normally go. Because of this blockage, the right ventricle usually is small and underdeveloped. The other valve on the right side between the right ventricle and the lungs can also be underdeveloped (pulmonary valve).
Healthcare providers put cases of tricuspid atresia into different categories. Up to 80% of people have Type I. Types I and II also have three subcategories based on other heart defects present.
Someone with tricuspid atresia can’t get enough blood flowing through their heart and into their lungs, where it would get oxygen. Because of this, their lungs can’t provide enough oxygen to the rest of their body.
In a baby born with tricuspid atresia, blood flows from the upper right chamber (right atrium) to the upper left chamber (left atrium) of the heart through a hole in the septum, the wall between the chambers. This hole is always present during fetal life (foramen ovale) but sometimes the hole is big and becomes a heart defect (atrial septal defect).
In some babies with tricuspid atresia, there’s an additional hole between their heart’s two lower chambers (ventricular septal defect). Blood can flow through this hole and into the right ventricle, which will pump blood into their lungs.
When blood is flowing through these unnatural routes, blood high in oxygen blends with blood low in oxygen. In a normal heart, the two types of blood don’t mix.
Yes. Tricuspid atresia is one of the serious heart defects that healthcare providers consider critical congenital heart defects. This type of heart disease usually requires care in an intensive care unit with experience in complex congenital heart disease at birth. Frequently, a heart surgery is needed before the baby goes home. Fatal complications occur without diagnosis and treatment.
Both of these heart valve issues have to do with the tricuspid valve. With tricuspid atresia, there’s no tricuspid valve. With Ebstein’s anomaly, the tricuspid valve is there but doesn’t work right.
Tricuspid atresia is rare but is one of the more common complex congenital (present at birth) heart diseases. About 1 out of 10,000 babies born has tricuspid atresia, regardless of gender.
People are more likely to get tricuspid atresia or another congenital heart disease if they have Down syndrome or a parent who had a congenital heart defect.
Other risk factors include having a birthing parent who:
In most cases, babies with tricuspid atresia have symptoms within a week of birth.
A baby with tricuspid atresia can show the following symptoms:
In addition, some babies with this condition can develop heart failure symptoms. These include:
Although tricuspid atresia has known risk factors, the exact causes for it are unknown. Congenital heart diseases develop while a fetus is in the uterus and heart development is taking place.
Healthcare providers can diagnose tricuspid atresia before or after your baby’s birth. Ultrasound allows them to make a diagnosis before birth. After your child is born, their provider can sometimes hear a heart murmur through their stethoscope.
Healthcare providers usually diagnose tricuspid atresia with an echocardiogram. An echocardiogram is an ultrasound that uses high-pitched sound waves that bounce off your child’s heart to produce moving images on a video screen. The echocardiogram tracks the flow of blood and can show:
Because an ultrasound uses harmless sound waves to create images, your provider can use it to check your child’s heart before birth. A fetal echocardiogram gives a better view if a more general ultrasound shows possible tricuspid atresia. In developed countries, providers diagnose most babies before birth.
Other diagnostic tests (after birth) include:
In babies who have tricuspid valve atresia, medication at birth can help keep their patent ductus arteriosus open. This extra blood vessel, which normally closes after birth, lets blood flow from their aorta to their pulmonary artery. This provides an alternate way to send blood to your baby’s lungs to get oxygen. Without a tricuspid valve, the normal route doesn’t work.
Medicines may also help your baby’s:
Healthcare providers usually treat this condition with surgery shortly after a baby’s birth. Your baby will then have more surgery later. Surgeries for tricuspid atresia include:
This surgery, done through cardiac catheterization, widens an existing one to help get more oxygen-rich blood out into your baby’s body. If your child is very blue, they may need this soon after birth as a life-saving measure. This procedure is rarely needed in this heart condition
Depending on your baby’s condition, they may then have one of the following two surgical operations:
This procedure, done between four and six months of birth, is one in which the surgeon connects your child’s superior vena cava to their pulmonary artery. Blood can then flow directly to their lungs without passing through their heart and pick up oxygen.
During this surgery, the surgeon forms a path that allows oxygen-poor blood that is going back to the heart to flow directly into the arteries that transport blood into the lungs. After this procedure, blood with oxygen and blood without oxygen stay separated like they should. Most children with tricuspid atresia don’t have the Fontan procedure until they are at least 2 years of age.
The riskiest time for your baby is the first few months of life before the Glenn procedure, but complications can happen after any surgery for tricuspid atresia.
No, tricuspid atresia isn’t curable. However, surgeries can help a baby’s heart work better and allow them to live past childhood. If the surgeries don’t help or their heart stops working properly, heart transplant can be done.
Although healthcare providers don’t know the exact cause of tricuspid atresia, they do know that it happens before birth. If you’re pregnant or planning to become pregnant, you may reduce your baby’s risk of having tricuspid valve atresia and other complex heart diseases in these ways:
Your baby will need surgeries and lifelong follow-up care so that a cardiologist can monitor their health. Your child will have to take preventive antibiotics before procedures such as dental care. Sometimes, they’ll recommend that your child limit physical activity that’s too demanding.
Most babies with tricuspid atresia won’t reach their first birthday without an operation. However, most children who have the surgeries live to be adults. One study showed a 61% survival rate 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although a heart transplant may be needed at an earlier age.
With today’s medical advances, many people with tricuspid atresia are living into adulthood. They’re moving from the care of a pediatric cardiologist to one who cares for adults. Adults who’ve had surgery for tricuspid atresia can have children, but their pregnancies are in the high-risk category.
In general, the short-term and intermediate-term outlook for children who have surgery is promising. However, the surgery isn’t a cure.
The outlook usually is worse for children who have surgery later in life. If Fontan surgery fails, your child may need a heart transplant.
Home monitoring can help give your baby a better outcome between surgeries. Home monitoring you can do yourself for your baby includes:
Your child will need regular appointments with a cardiologist throughout their life. How often they have office visits depends on their age and treatment stage. Regular checkups help your child’s provider catch any complications that may be developing. After your child has a Fontan procedure, they’ll see their cardiologist at least once a year.
Your child’s cardiologist also may do tests, which can include:
A note from Cleveland Clinic
Tricuspid atresia is a complex condition that normally requires surgery. Don’t be afraid to ask your baby’s healthcare provider about anything you don’t understand. Because of medical advances, the outlook for babies with this condition is better than in the past. Many children with tricuspid atresia are growing up to be adults with tricuspid atresia.
Last reviewed by a Cleveland Clinic medical professional on 06/28/2022.
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