Tricuspid atresia is a specific form of congenital heart defects that involves the deformation of the tricuspid valve in the heart. In some cases the valve is completely missing.
Tricuspid Atresia Causes, Incidence, And Risk Factors
A healthy heart functions with blood flowing into the right atrial valve, through the right ventricle, and then into the lungs. It is oxygenated in the lungs and then reenters the heart and is dispersed through the body. The opening between the right atrial and right ventricle is referred to as the tricuspid valve. Those born with tricuspid atresia have a closed or severely narrowed tricuspid valve, and the blood within the body cannot enter the lungs properly to gain the oxygen needed for proper health.
- The condition occurs in five out of every one hundred thousand births.
- It can occur on its own or with other congenital defects.
- Tricuspid atresia develops during the formation of the heart while still in the womb and is present at birth.
As is the case with most congenital birth defects, the exact causes of tricuspid atresia are largely a mystery. Several different risk factors do exist, however, and are worth understanding if you plan on having a child.
- Some genetic conditions carry a higher risk of tricuspid atresia and other congenital heart defects. Down’s syndrome, for instance, seems to carry a higher rate of congenital defects with it.
- Hereditary factors also play a role, and babies with a parent or close family member who has a congenital heart defect may be more likely to develop tricuspid atresia.
- Smoking during pregnancy can greatly increase the risks of your child developing a congenital defect.
- Alcohol and drug use while pregnant can also have serious consequences and increase the odds that your child is born with tricuspid atresia.
- Some congenital heart defects have been linked to contracting rubella during pregnancy.
- Uncontrolled diabetes during pregnancy can increase the chances of tricuspid atresia.
- Some anti-seizure medications and some anti-acne medications have been linked to congenital defects as well.
Tricuspid Atresia Symptoms
There are several symptoms of tricuspid atresia that will be noticeable in your child. Most of these will be noticed shortly after birth by the physician.
- Shortness of breath
- Rapid heart rates
- Cyanosis, which is a bluish or purplish tint to the skin
- Poor feeding
Most of these symptoms are caused by the lack of oxygen in your baby’s bloodstream. Without proper levels of oxygen reaching the body, serious issues can arise. Once your doctor notices any of these conditions, they will take the next step and confirm their diagnosis.
Tricuspid Atresia Diagnosis
Because many congenital heart defects present themselves with the same symptoms, and due to the fact that several different issues could be present at once, your child’s physician will have to run various tests in order to uncover just which type of congenital defect your baby is suffering from. While a simple stethoscope will often unveil a heart murmur or arrhythmia, further tests will have to be undertaken. These will include one or more of the following in most cases.
- X-Ray – This simple procedure will let doctors take a closer look at your child’s chest, including the size and shape of the heart.
- MRI – This advanced scan is often only utilized after a corrective surgery but is sometimes employed to take a detailed look at your child’s heart structure.
- Echocardiogram – An echocardiogram is one of the most common types of tests used to uncover heart defects. It uses sound to deliver an image of the heart, including its structure and the blood flow within it. It will allow doctors to check for the presence of a tricuspid valve and to locate any additional deformities that could be present.
- EKG – This test will help doctors monitor the heart rhythm and electrical impulses of your heart.
- Cardiac Catheterization – This involves the insertion of a tube into your child’s blood vessel in an effort to check the strength and pressure of the heartbeat.
These tests will usually be more than enough for doctors to confirm a diagnosis of tricuspid atresia, or decide if a different congenital defect is present.
Your child will likely be placed in an intensive care unit and given oxygen through a ventilator. After this is completed, a few steps for treatment will follow.
- Medication known as prostaglandin E1 will be administered. This drug helps to maintain proper blood flow into the lungs which in turn helps improve oxygen levels throughout the body.
- Surgery will follow shortly after diagnosis and will most likely involve multiple surgeries over the course of several months or years.
- First will be a procedure to place a shunt into the heart which will allow regular blood flow between the two valves.
- At around four to six months of age, the next procedure will take place. It involves the connection of blue veins directly to the pulmonary artery.
- Between eighteen months and three years of age, the final procedure will occur. This will be a continuation of the second and the remainder of the veins carrying blue blood will be attached to the pulmonary artery.
- In short, the surgery allows the heart to skip the step of pumping blood through the lungs.
- Later surgeries may need to occur, often in the patient’s twenties or thirties, to correct any irregular heartbeats or other issues that could arise.
The earlier the surgeries are completed the better the outlook will be. While death by sudden arrhythmia is still a concern, the prognosis is still very positive following successful surgeries. Limited physical activity may be recommended and regular exams for the remainder of a patient’s life will be needed. There may be complications that arise later in life, so vigilance is needed at all times.
Numerous complications could occur, even after corrective surgeries. These can be quite serious and include the following.
- Fluid in the lungs or abdomen
- Rapid or irregular heart beats
- Blockage of the shunt
- Chronic diarrhea
- Neurological complications
- Sudden death
Contacting Medical Help for Tricuspid Atresia
Although your doctor will likely notice symptoms before you leave the hospital after birth, there is the chance that symptoms will present later. Contact your doctor immediately if you notice that your child has trouble eating, difficulty breathing, or is turning blue or purple.
There is no surefire way to prevent tricuspid atresia. Avoidance of drugs, alcohol, and cigarettes during your pregnancy will help tremendously. Regular screenings can help determine if your child is at risk for developing a problem, and steps can be taken to ensure that the hospital staff is prepared to deal with any complications during birth.