When your baby is born, does it sometimes feel like it's hard to breathe? Does it sometimes look like the skin is turning blue? It's normal to feel very scared when you see something like that. Today we are going to talk about a congenital heart disease that shows these symptoms. This is called TAPVR (Total Anomalous Pulmonary Venous Return) . Although the name may seem a bit complicated, let's understand it simply.
What is TAPVR? To put it simply...
Think about it, our bodies get oxygen from the lungs, right? So, from these lungs, oxygenated blood (let's call this "good blood") is brought to the heart by veins , a type of blood vessel. Normally, this "good blood" should come to the left atrium on the left side of the heart. From there, the blood is pumped to provide oxygen to the entire body.
However, in a baby with TAPVR, instead of connecting to the left atrium of the heart, this pulmonary vein connects to the wrong place, which is the right atrium . The right atrium normally receives "bad blood" that has been used up by the body, which is low in oxygen. Now the "good blood" that is full of oxygen mixes with this "bad blood." What happens then? The amount of oxygen in the blood that is supposed to go to the whole body decreases. This is a very dangerous and life-threatening condition for a baby. This is a congenital heart disease.
Almost all babies with TAPVR have a small hole (Atrial Septal Defect - ASD) between the two chambers of their heart, the right atrium and the left atrium. This hole allows some oxygenated blood to flow to the left side of the heart and then to the rest of the body. Otherwise, the condition could have been much worse.
The most important thing is that babies with TAPVR definitely need surgery. If surgery is done early, most children can live healthy lives into adulthood. However, sometimes, things like narrowing of the veins later in life may require further surgery or other treatments. Therefore, even after TAPVR surgery, you will need to be monitored by a cardiologist for the rest of your life.
Sometimes doctors also call this condition TAPVC (Total Anomalous Pulmonary Venous Connection). Both are the same condition.
Are there different types of TAPVR?
Yes, there are several main types of TAPVR. These are classified according to how the "good blood" from the lungs is improperly connected to the right atrium.
- Supracardiac TAPVR: In this, the pulmonary veins join and connect to a large vein located above the heart (called the superior vena cava , which is a major vein that carries blood from the upper parts of the body to the right side of the heart).
- Cardiac TAPVR: Here, the pulmonary vein connects directly to the right side of the heart, into the coronary sinus , which is a space that normally collects oxygen-poor blood from the heart muscle.
- Infracardiac TAPVR: In this type, the pulmonary vein passes below the heart, through the liver, and connects to a large vein ( inferior vena cava ) that carries blood from the lower parts of the body to the right side of the heart.
- Mixed TAPVR: This is the rarest and most difficult type to repair. Here, more than one vein from the lungs may be connected, in a combination of one or more of the types mentioned above.
This classification is important for doctors to plan surgery and determine treatment methods.
How common is TAPVR?
TAPVR is a very rare heart condition. Even in a country like America, this condition affects only one in 7,500 newborns. It is also rare in Sri Lanka.
What are the symptoms of TAPVR?
Symptoms of TAPVR are usually visible at birth , especially if there is a narrowing of the pulmonary veins where they connect to the heart. However, some babies may not show symptoms for several weeks.
The main symptoms are these:
- Cyanosis: A bluish or grayish discoloration of the skin, nails, and lips (in babies with dark skin). This is caused by a lack of oxygen in the blood.
- Difficulty sucking: The baby has difficulty sucking and gets tired after drinking a little.
- Fatigue and lethargy: The baby is constantly tired and seems lethargic.
- Heart murmur: When the doctor listens to the baby's heart (with a stethoscope), an abnormal sound is heard.
- Shortness of breath (dyspnea): The baby is breathing rapidly and seems to be having difficulty breathing.
- Poor growth and weight gain: The baby is not gaining weight properly and is stunted.
If your baby has one or more of these symptoms, you should seek medical advice immediately .
What causes TAPVR?
In fact, doctors still don't know the exact cause of TAPVR. It's caused by a defect in the heart and blood vessels during fetal development .
It is not usually seen as an inherited condition. However, there have been reports of cases occurring in siblings within the same family. Some studies have suggested that there may be a link between children with certain syndromes and exposure to paint removers, pesticides, and lead during pregnancy. However, this is not yet proven.
What are the possible complications of TAPVR?
A baby with TAPVR can develop heart failure and high blood pressure in the lungs (pulmonary hypertension) . Even after surgery, sometimes abnormal heart rhythms or blockages in the pulmonary veins can occur. That's why continued medical checkups are important.
How is TAPVR diagnosed?
Sometimes, the condition can be detected before the baby is born, with an echocardiogram (a scan of the heart) done during pregnancy. However, most often the diagnosis is made a few weeks or months after the baby is born.
When the baby is born, the doctor will do a physical exam and listen to the baby's heart. A small device called a pulse oximeter (a clip that fits around the baby's big toe) can be used to measure the oxygen level in the blood.
In addition, tests such as the following may be done to check the condition of the heart and blood vessels:
- Chest X-ray
- CT scan (CT scan - computed tomography)
- Heart MRI (magnetic resonance imaging)
- Echocardiogram
- EKG (EKG or ECG - Electrocardiogram)
Some of these tests (such as MRI) may require the baby to be sedated (put to sleep lightly). But all of these tests are noninvasive. These tests allow doctors to get clear images of the baby's heart and lungs, see how blood is flowing, and look for abnormalities.
Sometimes a test called cardiac catheterization is also used to diagnose the disease. However, since the necessary information can usually be obtained from the scans mentioned above, the baby is rarely subjected to invasive testing.
How is TAPVR treated?
Almost all babies with TAPVR will need surgery to survive. Doctors treat it with open-heart surgery . Often, this surgery is performed as soon as possible after the condition is diagnosed.
Although very rare, some people are diagnosed with TAPVR as adults without having surgery as children. However, by then they may have conditions like pulmonary hypertension that make surgery very challenging.
Preoperative treatment
While waiting for surgery, the baby may need to be given extra oxygen or connected to a ventilator to help him breathe. You may also be given a type of medicine called an inotrope , which helps the heart beat more strongly.
How the surgery is performed
After the baby is completely asleep under general anesthesia , the surgeon will do the following:
1. Incisions are made on the baby's chest and heart.
2. The pulmonary veins are reconnected to the correct place, that is, the left atrium of the heart.
3. Abnormally connected veins are closed.
4. If there is any blockage in the pulmonary veins, it is removed.
5. Often, the hole between the left and right atria ( Atrial Septal Defect - ASD ) is closed.
Although this is a very complex surgery, experienced medical teams perform it successfully.
Complications of treatment
These are some of the complications that may occur after surgery:
- Heartbeat irregularities (Arrhythmias)
- Pulmonary hypertension
- Pulmonary venous obstruction
- Sometimes, after using a heart-lung machine (bypass machine) during surgery, the heart has difficulty beating on its own.
Doctors are always on the lookout for things like this.
Can TAPVR be prevented?
Unfortunately, there is no way to prevent this type of congenital heart disease. If someone in your family has congenital heart disease, or if you have TAPVR, it is a good idea to talk to your doctor before starting a family.
If my child has TAPVR, what should I expect?
Without surgery, some types of TAPVR can be fatal within weeks of birth. However, if the disease is diagnosed early and treated surgically, the outlook for babies with TAPVR is very good. The survival rate after surgery is about 97%. That is a very high success rate.
As your child grows and reaches adulthood, he or she should see a cardiologist regularly . These routine checkups throughout life can help detect and treat problems like heart rhythm irregularities and blood vessel blockages. Sometimes, if a blockage develops, it may require further surgery and may be difficult to treat. That's why it's important to continue following your doctor's instructions.
Your child may need to continue taking medication or undergo treatments such as cardiac catheterization .
How do I take care of my child?
Children with TAPVR may sometimes have difficulty with normal developmental milestones, such as fine motor function. You can monitor your child's developmental milestones and refer them to someone who can help them develop those skills if needed.
Talk to your child's doctor about whether your child can participate in sports and physical activities. You may need to limit strenuous exercise .
When should my child see the doctor?
After surgery, your child will have regular checkups every 6 to 12 months . This will continue into adulthood. Your child's doctor may want to do tests such as an EKG, exercise stress test, or echocardiogram .
When do you need to go to the Emergency Room (ETU) ?
Seek immediate medical attention (go to the ER) if your baby has any of these symptoms:
- If the skin, nails, or lips turn blue/gray.
- If you have difficulty breathing or eating/drinking milk.
- If you appear very sleepy or lifeless.
What questions should I ask my doctor?
Here are some important questions to ask your child's doctor:
- What type of TAPVR does my child have?
- Do you think my child will need a second surgery?
- How many surgeries have you had to repair TAPVR?
An important take-home message
You may have followed your doctor's advice to stay healthy during pregnancy. But sometimes, a newborn baby can develop a heart condition. It's no one's fault. With today's advanced medical science, your baby can expect a much better outcome than children of previous generations. Surgical treatment is very effective.
Remember, although TAPVR is a serious condition, with early diagnosis and proper surgical treatment, children have a great chance of living a good life. After surgery, it is essential to take your child for regular medical check-ups. This is so that your child's health can be monitored and any problems that may arise can be treated quickly. You are not alone, doctors and healthcare staff are there to help you and your baby.
` TAPVR, heart disease, baby, surgery, pulmonary veins, cyanosis, congenital diseases
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