Tricuspid atresia is a congenital (present at birth) heart defect that occurs when the tricuspid valve of the heart is not properly formed. The tricuspid valve lies between two of the chambers of the heart (the right atrium and right ventricle). In tricuspid atresia, there is solid tissue instead of the tricuspid valve. This sheet of tissue blocks blood flow from the right atrium to the right ventricle. Because of this blockage, the right ventricle usually is small and underdeveloped.
When tricuspid atresia is present, a sufficient amount of blood is not able to flow through the heart and into the lungs, where it would obtain oxygen as the blood in a normal heart would. As a result, the lungs of a baby with this defect are not able to provide the rest of the body with its required oxygen.
In a baby who is 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. The hole can be a heart defect (atrial septal defect) or an enlarged natural opening (foramen ovale). The foramen ovale should close soon after a baby is born. If there is no atrial septal defect, a surgeon may need to create a foramen ovale to allow blood flow.
In some babies with tricuspid atresia, there is a hole between the heart’s two lower chambers (ventricular septal defect). Blood can flow through this hole and into the right ventricle, which will pump the blood into the lungs.
Risk factors are traits that make a person more likely to develop a disease or condition. Risk factors for tricuspid atresia include:
Although there are known risk factors for tricuspid atresia, the exact causes are not known. Congenital heart diseases develop while the baby is in the womb and heart development is taking place.
In most cases, babies born with tricuspid atresia have symptoms within a week after being born. A baby with tricuspid atresia can show the following symptoms:
In addition, some babies with this condition can develop heart failure symptoms. These include:
Tricuspid atresia is usually diagnosed with an echocardiogram. An echocardiogram uses high-pitched sound waves that bounce off the child’s heart to produce moving images that the doctor can see on a video screen. The echocardiogram tracks the flow of blood and can show:
Tricuspid atresia has potentially fatal complications, including not getting enough oxygen to the body’s tissues, and a high red blood cell count, which may cause clots that can lead to stroke or heart attack. These complications can be avoided with prompt treatment.
While medications can help babies who have tricuspid atresia, this condition is usually treated with surgery. Surgeries for tricuspid atresia include:
Your baby will need lifelong follow-up care so that a cardiologist can monitor his or her health. The cardiologist can let you know whether your child has to continue taking preventive antibiotics before procedures such as dental care. Sometimes, the cardiologist will recommend that your child limit physical activity that is too vigorous.
In general, the short-term and intermediate-term outlook for children who have surgery is promising, though the surgery does not cure the child. The outlook usually is worse, however, for children who have surgery later in life. If the surgery fails, the child may need a heart transplant.
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or healthcare provider. Please consult your healthcare provider for advice about a specific medical condition. This document was last reviewed on: 09/27/2019