Tricuspid atresia is a heart defect present at birth (congenital) in which a valve (tricuspid valve) between two of the heart's chambers isn't formed. Instead, there's solid tissue between the chambers, which restricts blood flow and causes the right lower heart chamber (ventricle) to be underdeveloped.
A baby, child or adult with tricuspid atresia can't get enough oxygen through its body. People with this condition tire easily, are often short of breath and have blue-tinged skin.
Tricuspid atresia is treated with multiple surgeries. Most babies with tricuspid atresia who have surgery live well into adulthood, though follow-up surgeries are often needed.
Source: http://www.mayoclinic.com
Tricuspid atresia symptoms become evident soon after birth, and can include:
Some babies or older people with tricuspid atresia also develop symptoms of heart failure, including:
Tell your doctor if you notice any of the above symptoms in yourself or your child.
Source: http://www.mayoclinic.com
Tricuspid atresia occurs during fetal heart development. Some genetic factors, such as Down syndrome, might increase your baby's risk of congenital heart defects such as tricuspid atresia, but the cause of congenital heart disease is usually unknown.
Your heart is divided into four chambers â the right atrium and right ventricle and left atrium and left ventricle. The right side of the heart moves blood to the lungs, where it picks up oxygen before it circulates to your heart's left side. The left side 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 your heart. These valves open to allow blood to move to the next chamber or one of the arteries, and they close to keep blood from flowing backward.
In tricuspid atresia, the right side of the heart can't pump enough blood to the lungs because the tricuspid valve is missing. A sheet of tissue blocks the flow of blood from the right atrium to the right ventricle. As a result, the right ventricle is usually small and underdeveloped (hypoplastic).
Blood instead flows from the right atrium to the left atrium through a hole in the wall between them (septum). This hole is either a heart defect (atrial septal defect) or an enlarged natural opening that's supposed to close soon after birth (patent foramen ovale or patent ductus arteriosus). A baby with tricuspid atresia might need medication to keep the natural opening from closing after birth or surgery to create an opening.
Many babies born with tricuspid atresia have a hole between the ventricles (ventricular septal defect). In these cases, some blood can flow through the hole between the left ventricle and the right ventricle, and then blood is pumped to the lungs through the pulmonary artery.
However, the valve between the right ventricle and the pulmonary artery (pulmonary valve) might be narrowed, which can reduce blood flow to the lungs. If the pulmonary valve isn't narrowed and if the ventricular septal defect is large, too much blood can flow to the lungs, which can lead to heart failure.
Some babies may have other heart defects as well.
Source: http://www.mayoclinic.com
Because of advances in ultrasound technology, doctors can usually identify tricuspid atresia on a routine ultrasound exam during gestation.
Your baby's doctor might suspect a heart defect, such as tricuspid atresia, if your newborn has blue-tinged skin or is having trouble breathing.
Another indication is hearing a heart murmur, an abnormal whooshing sound caused by blood not flowing properly, when listening to your baby's heart during a physical exam.
If tricuspid atresia is suspected, your baby's doctor might order tests including:
Source: http://www.mayoclinic.com
A life-threatening complication of tricuspid atresia is a lack of oxygen to your baby's tissues (hypoxemia).
Although treatment greatly improves the outcome for babies with tricuspid atresia, complications can develop later in life, including:
Source: http://www.mayoclinic.com
Congenital heart defects such as tricuspid atresia usually aren't preventable. If you have a family history of heart defects or a child with a congenital heart defect, a genetic counselor and a cardiologist experienced in congenital heart defects can help you look at 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:
Source: http://www.mayoclinic.com
Here are some tips for caring for your child with tricuspid atresia:
Strive for good nutrition. Your baby might not be getting enough calories because of tiring during feeding and an increased need for calories. It's often helpful to give your baby frequent, small feedings.
Breast milk is an excellent source of nutrition, but formula works well, too. Your doctor might prescribe a special high-calorie formula.
Preventive antibiotics. Your or your child's cardiologist will likely recommend preventive antibiotics be taken before certain dental and other procedures to prevent bacteria from infecting the inner lining of the heart (infective endocarditis).
Practicing good oral hygiene â brushing and flossing teeth, getting regular dental checkups â also helps prevent infection.
If you're an adult with tricuspid atresia, you need to be seen regularly throughout your life by a doctor trained in adult congenital heart conditions. Your doctor is likely to recommend regular tests to evaluate your condition at these appointments.
Your doctor might recommend that you take preventive antibiotics before certain dental or medical procedures to prevent infective endocarditis.
Ask your doctor about what activities are best for you, and if there are sports or activities that you should limit or avoid.
Women with tricuspid atresia who are considering pregnancy should talk to a doctor who specializes in adult congenital heart diseases as well as a maternal-fetal medicine specialist. If you do become pregnant, it's best to see a doctor who specializes in pregnancies in women with congenital heart disease.
For women who have had a Fontan procedure, pregnancy will be considered high-risk. Some women, such as those with a history of heart failure, will be discouraged from becoming pregnant.
Source: http://www.mayoclinic.com
Caring for a baby with a serious heart problem such as tricuspid atresia can be challenging. Here are some strategies that might help:
Remember that many children with congenital heart defects, such as tricuspid atresia, grow up to lead full lives.
Source: http://www.mayoclinic.com
In most cases, the cause of a congenital heart defect, such as tricuspid atresia, is unknown. However, several factors might increase the risk of a baby being born with a congenital heart defect, including:
Source: http://www.mayoclinic.com
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