What is tricuspid atresia?
Tricuspid atresia is a congenital heart defect in which the valve (tricuspid valve) does not form between the two chambers of the heart. Instead, there is optic tissue between the chambers, restricting blood flow and preventing the development of the lower right heart chamber (ventricle).
A baby, child, or adult with tricuspid atresia may not be able to get enough oxygen through their body. This condition is more common in people with tires, shortness of breath, and bluish skin. Tricuspid atresia can be treated with multiple surgeries. Most children with surgical tricuspid atresia live well into adolescence, although more surgeries may be necessary.
Who gets tricuspid atresia?
A child is more likely to have tricuspid atresia:
- The baby has Down syndrome (trisomy 21)
- Parents have congenital heart defects
- The mother has a rubella infection (German measles) or another viral infection during pregnancy
- Maternal diabetes or lupus (an autoimmune disease) are not adequately controlled
- The mother uses some acne medications or cesarean section during pregnancy.
But having one or more risk factors does not mean that the baby will have tricuspid atresia. Tricuspid atresia occurs without any risk factors.
What are the risk factors for tricuspid atresia?
Risk factors can cause a person to develop symptoms of a disease or condition. Risk factors for tricuspid atresia:
- Down’s Syndrome
- Parents with congenital heart disease
- Who is a mother
- Having a viral illness (including German measles) in early pregnancy.
- You drank too much alcohol during pregnancy.
- There is diabetes that is not adequately controlled.
- Some medications, such as cesarean section or acne medications, were used during pregnancy.
What are the symptoms of tricuspid atresia?
In most cases, babies born with tricuspid atresia show symptoms within a week of birth. A baby with tricuspid atresia shows the following symptoms:
- Blue skin and lips (cyanosis)
- Difficulty feeding
- Shortness of breath and rapid breathing
- Slow growth
- Heart murmur (abnormal heart sounds)
Also, some children with this condition can develop symptoms of heart failure. In addition to:
- Rapid weight gain due to fluid retention
- Swelling in the abdomen
- Swelling of the legs, ankles, and feet.
- Weakness and fatigue
- Irregular or rapid heartbeat
Causes of tricuspid atresia
Tricuspid atresia is an abnormal form of congenital heart disease. It affects 5 out of every 100,000 live births. One in five people with this condition will also have other heart problems. Normally, blood flows from the body into the right atrium, then through the tricuspid valve into the right ventricle and into the lungs. If the tricuspid valve does not open, blood will not flow from the right atrium to the right ventricle. Due to a problem with the tricuspid valve, the blood eventually does not enter the lungs. This is where you go to pick up oxygen (get oxygenated).
Instead, the blood passes through a hole between the right and left atria. In the left atrium, it mixes with oxygen-rich blood and returns from the lungs. This oxygen-depleted, oxygen-rich blood mixture is pumped into the body from the left ventricle. This makes the oxygen level in the blood lower than normal. In those with tricuspid atresia, the lungs receive blood through a hole between the right and left ventricles (described above) or through a fetal vessel called the ductus arteriosus. The ductus arteriosus connects the pulmonary artery (artery to the lungs) to the aorta (the main artery in the body). It occurs when a baby is born but usually shuts down immediately after birth.
How is tricuspid atresia diagnosed?
She suffers from a prenatal ultrasound exam for tricuspid atresia. Otherwise, symptoms appear immediately after birth. A pediatric cardiologist or neonatologist will help you care for your child. These are doctors who are specially trained to treat problems in children. One or more healthcare providers will examine your child. They will listen to your baby’s heart and lungs with a stethoscope and notice any abnormal sounds from the heart (heart murmur).
Congenital heart disease tests may include:
- Pulse oximetry: A simple test that uses a small probe to check the amount of oxygen in the blood.
- Lab tests: Blood and urine samples are reviewed.
- Chest x-ray: Chest radiography often shows visible changes with tricuspid atresia.
- Electrocardiogram (ECG): An ECG records the electrical activity of the heart. Shows abnormal rhythms and detects heart muscle tension.
- Echocardiogram (Echo): It uses resonant sound waves to create a moving image of the heart and heart valves. Tricuspid atresia is diagnosed based on the detection of abnormal heart structures.
- Cardiac catheterization: This procedure provides very detailed information about the internal structures of the heart. Your child will be given medicine to rest (intoxicated). Then a thin, flexible tube (catheter) is inserted into your baby’s groin. Guide the heart. Blood pressure and oxygen measurements are taken in the chambers of the heart, pulmonary artery, and aorta. Contrast dye is also injected to see the structures inside the heart more clearly. Cardiac catheterization is not often done to diagnose this condition, but it can be done before surgery and in the early treatment of the defect.
Treatment of tricuspid atresia
In tricuspid atresia, there is no way to replace the tricuspid valve. Treatment includes surgery to ensure adequate blood flow through the heart and into the lungs. This often requires more than one surgery. Medications may also be given before surgery to strengthen the heart muscle, lower blood pressure, and remove excess fluid and associated oxygen from the baby’s body.
Some methods used to treat tricuspid atresia include temporary relief (palliative surgery) to increase blood flow. Required procedures:
- Atrial septostomy: This creates or widens the opening between the upper chambers of the heart to allow more blood to flow from the right atrium to the left.
- Maneuvers: Creates a shunt (shunt) from the main blood vessel leading from the heart to the blood vessel leading to the lungs (pulmonary artery), which improves oxygen levels. Surgeons usually implant a shunt in the first two weeks of life. However, children usually outgrow this shunt and may need another surgery to replace it.
- Placement of a pulmonary artery band: If your baby is flowing more blood from the heart to the lungs, a surgeon may place a band around the pulmonary artery to reduce the flow.
- Operation Glen: When children outgrow the first bypass, they often need this surgery, which sets the stage for a more permanent corrective surgery called the Fontan procedure. Doctors usually perform a glen operation when the baby is between 3 and 6 months old. Doctors first remove the shunt and then connect it to the pulmonary artery instead of one of the large veins (superior vena cava) that returns blood to the heart. This procedure allows blood to flow directly to the lungs and reduces the workload on the left ventricle, thus reducing the risk of damage.
- Fonton approach: Variation from this standard treatment of tricuspid atresia usually occurs in children 2 to 3 years of age. Typically, the surgeon creates a path for blood returning to the heart (inferior vena cava) to flow directly to the pulmonary arteries, which then carry the blood to the lungs. Sometimes doctors leave an opening between the pathway and the right atrium (fenestration).
Before surgery, your pediatric cardiologist may recommend that your child take prostaglandins.
Lifestyle and home remedies
Here are some tips for caring for your child with tricuspid atresia:
- Strive for good nutrition: Your baby may not get enough calories due to fatigue and high-calorie intake while eating. It is often helpful to give your baby small, frequent feedings.
- Breast milk is an excellent source of nutrition, but the formula works well too. Your doctor may prescribe a special high-calorie formula.
- Preventive antibiotics: Your cardiologist or pediatrician may recommend preventive antibiotics before certain dental and other procedures to prevent a bacterial infection of the lining of the heart (infective endocarditis).
- Following good oral hygiene (brushing and floating, getting regular dental checkups) can also help prevent infection.
- Be active: Encourage regular play and activities as much as you or your child can tolerate or have enough opportunities to relax as recommended by your doctor. Being active can help you maintain your or your child’s heart health.
- Continue with regular medical and pediatric care: Children with congenital heart defects, as well as standard vaccinations, are encouraged to get vaccinated against influenza, pneumonia, and respiratory syncytial virus infections. Your child must take all prescription medications.
- Continue with other appointments with yourself or your pediatrician. Your baby needs at least yearly appointments with a doctor trained in congenital heart conditions. Your pediatrician will likely recommend several tests to evaluate your / your child’s heart condition.
Adults with tricuspid atresia
If you are an adult with tricuspid atresia, you should see a trained doctor throughout your life regularly for heart conditions that present with childbirth in adulthood. During these appointments, your doctor may recommend routine tests to assess your condition. Your doctor may recommend that you take preventive antibiotics before certain medical or dental procedures to prevent infective endocarditis. Ask your doctor what activities are best for you and what sports or activities to limit or avoid.
Tricuspid atresia: Results
- This tiered approach is generally good for children with tricuspid atresia, as three individuals can survive 75 to 95 percent in three stages, depending on the exact deformities and surgery.
- Long-term quality of life after Fontan operation is now better in patients older than 30 years. However, many problems can arise. These include heart rhythm problems that require medication or pacemaker placement.
- Patients also experience lung and lung problems, leading to decreased oxygen in the blood or digestive problems caused by loss of protein in the stool, inflammation, and water retention. For minority patients, heart, lung, and lung transplants may be necessary later in life.