Sometimes you may have noticed that your newborn baby has difficulty breathing, struggles to feed, or even turns blue. These can sometimes be signs of a small problem with your baby's heart. That's right, a heart condition that is present at birth is called Atrioventricular Canal Defect , which we're talking about today.
What is this (Atrioventricular Canal Defect)?
Simply put, Atrioventricular Canal Defect is a heart condition that is present at birth. Doctors sometimes call it AVSD - Atrioventricular Septal Defect . To be precise, it is a combination of several heart problems. The main one is that there is a hole or holes in the wall in the middle of the heart (we call this the septum) . It also involves the valves in the heart not working properly.
Think of it like a hole in a water pipe. In a healthy heart, the oxygen-rich blood pumped from the left side of our heart does not normally mix with the oxygen-poor blood from the right side. But because of this hole, that oxygen-rich blood mixes with the oxygen-poor blood. Most of the time, this hole causes too much blood to go to the lungs. Then the amount of blood going to the rest of the body decreases. If this goes on for a long time, it can sometimes go the other way. That is, more oxygen-poor blood goes to the body, and less goes to the lungs. When that happens, the oxygen level in the body decreases and the skin can turn blue. We call it cyanosis .
This causes the heart to work harder, which can lead to other health problems such as congestive heart failure .
If this (Atrioventricular Canal Defect) is not treated properly, it can sometimes affect the life of the child. Without surgery, children with this condition may only live for a short time, about two to three years. But don't worry! The best thing is that surgery to correct this is very successful. Doctors can detect this condition before it becomes serious, even before the baby is born.
On average, in countries like the United States, about one in 1,700 babies are born with this defect each year. This accounts for between 3% and 5% of all congenital heart defects.
This condition is called by several other names. Your doctor may use one of these names:
- Atrioventricular septal defect (AVSD)
- Endocardial cushion defect
Are there different types of Atrioventricular Canal Defect?
Yes, this heart defect can come in different forms. That is, it depends on things like the size of the hole in the heart and the number of valves.
Complete (Atrioventricular Canal Defect)
What happens in this is that a large hole forms between the two upper chambers of the heart (we call them atria) and the two lower chambers (we call them ventricles ). This means that all four chambers of the heart are connected together. Normally, there should be two valves between the upper and lower chambers. But in this case, there is only one large valve. Its flap may not open or close properly.
Partial (Partial Atrioventricular Canal Defect)
In this case, the hole is between the two upper chambers of the heart (this is the most common) or between the two lower chambers. All four chambers are not connected. There are two valves between the upper and lower chambers. But one valve (most often the mitral valve, located between the upper chamber (left atrium) and the lower chamber (left ventricle) on the left side) does not work properly.
Transitional Atrioventricular Canal Defect
In this, there is a hole between the two upper chambers and a small hole between the two lower chambers. The two valves between the upper and lower chambers (AV valves) are separate.
Unbalanced Complete Atrioventricular Canal Defect
Remember how in a complete heart defect there is one large valve? That valve is located closer to one lower chamber (ventricle) than the other. Then the chamber that receives more blood becomes larger than the other. Doctors call that small chamber 'hypoplastic' .
What are the symptoms of this?
Symptoms of this heart defect can appear even before your baby is born. Check to see if your baby has any of these:
- Blue skin color (Cyanosis): Especially in areas like the lips, tongue, and fingertips.
- Difficulty drinking milk: Feeling tired and lethargic after drinking a little milk.
- Sweating after drinking milk.
- Getting tired quickly: Tired even after doing a little thing.
- Heart palpitations.
- Difficulty breathing (Dyspnea) or rapid breathing.
- Weight gain occurs slowly.
- Swelling (edema) in the abdomen or legs.
- Weak pulse.
However, babies with a partial or transitional type of problem may not show these symptoms at first. These symptoms may appear later in childhood, or in adolescence, or even in adulthood.
Why does this (Atrioventricular Canal Defect) occur?
Researchers still don't know exactly what causes Atrioventricular Septal Defect (AVSD) . It's thought to be a combination of genetic and environmental factors . But one thing is clear: there is a strong link between this heart condition and Down syndrome . About 40% of babies with Down syndrome have AVSD .
What are the risk factors that affect this?
Experts haven't yet identified the exact risk factors that cause this. There may be a genetic influence. Perhaps an abnormal gene passed down from either the mother or the father may increase the baby's chance of developing heart disease while still in the womb.
Apart from that, the following things during pregnancy can also increase the risk of your baby developing a congenital heart disease:
- Certain medications.
- Illegal drugs (Recreational drugs).
- Alcohol-containing beverages.
- Exposure to environmental toxins or chemicals.
- Nutrient or vitamin deficiencies.
- Obesity.
- Tobacco products.
- Not controlling chronic diseases like Diabetes Mellitus or Hypertension.
- Viral diseases such as influenza (flu) or flu.
What are the possible complications of Atrioventricular Canal Defect?
If this condition is severe, meaning it is not treated properly, problems such as these can occur:
- Abnormal heart rate patterns (Arrhythmia).
- Congestive heart failure.
- Heart infections (Endocarditis).
- High blood pressure in the blood vessels leading to the lungs (Pulmonary hypertension).
How is this disease diagnosed?
The good news is that most of the time, doctors can detect this (Atrioventricular Septal Defect) before the baby is born with a few tests:
- Prenatal ultrasound: This can see images of the baby's movements in the womb, including the heart. It can also detect a large septal defect .
- Fetal echocardiogram: This shows images of the heart more clearly than an ultrasound. The doctor can get a better idea of how the heart is structured and how it pumps blood.
After the baby is born, a doctor can listen to the baby's heartbeat with a stethoscope . If an unusual "loud" sound (we call it a heart murmur ) is heard, it could be the sound of blood flowing through the hole.
Other tests performed after birth include:
- Chest X-rays: Check the size and shape of the baby's heart.
- Electrocardiogram (EKG/ECG): View the electrical activity of the baby's heart.
- Echocardiogram: A very detailed look at the structure of the baby's heart.
- Cardiac catheterization: Learn more about the severity of the child's condition.
- Cardiac Magnetic Resonance Imaging (Heart MRI): See the parts of the baby's heart and how they work.
Sometimes, if a baby has a small septal defect , it may not show any symptoms at first. In that case, it may be several years before a doctor discovers the condition.
What are the treatments for this?
The most common treatment for Atrioventricular Canal Defect is open-heart surgery . In this surgery, the surgeon patches the hole in the baby's heart and closes it. If the defect is complete , the single heart valve is divided into two, creating two separate valves, one on the right side and one on the left.
A baby with an unbalanced AV canal defect may need to undergo several surgeries, culminating in a surgery called a Fontan procedure .
It is best to have this surgery as soon as possible, before the condition causes permanent damage to the heart. Most babies have this surgery within the first six months of life. Some babies who have a partial defect but are asymptomatic can have this surgery within the first three years of life.
Sometimes, if the baby is not healthy enough for surgery, or is underweight, medication may be given to control symptoms until he gains weight and strength.
- Diuretics are medications that remove excess water from the body.
- Digoxin helps the baby's heart beat more strongly.
- ACE inhibitors widen blood vessels and facilitate blood flow.
Another short-term solution is a procedure called pulmonary artery banding . This reduces the amount of blood flowing through the pulmonary artery to the baby's lungs. Babies who have had pulmonary artery banding can have a permanent repair done later.
Can complications occur after treatment?
Yes, sometimes things like this can happen after surgery:
- Heart block: This means that the heart's pulse signal does not reach the lower part of the heart properly.
- A leaky left or right AV valve.
- A hole in the wall of the lower chamber (Ventricular septal defect).
- Narrowing (Stenosis) of the left or right AV valve.
- An obstruction to blood flow beneath the aortic valve (Subaortic stenosis).
Can't this (Atrioventricular Canal Defect) be prevented?
There is really no way to prevent this (Atrioventricular Septal Defect) from occurring, because it is often related to genetic causes.
However, if you are pregnant, you can do these things to reduce the risk of your baby having a congenital heart disease:
- Completely avoid illegal drugs, alcohol, and tobacco products.
- Get all the vaccinations you need to protect yourself from diseases.
- Maintain a healthy weight.
- If you have chronic health conditions, manage them well.
- Take prenatal vitamins, especially folic acid, as recommended by your doctor.
If my child has this condition, what will the future be like?
Without surgery, children with AV canal defect may only live for two to three years. Some live into young adulthood.
But the good news is that about 90% of children who have had that repair surgery live 10 years after that treatment. That means they usually live at least another 10 years after the surgery. About 65% live 20 years after the surgery.
But remember this: Even after surgery, a person with an Atrioventricular Canal Defect (AVCD) will not have a completely normal heart. They will need to continue to have echocardiograms at regular intervals to monitor their heart function. This will help catch any complications early.
The patch that is placed over the hole can usually last a lifetime. But over time, one of the repaired heart valves can start to leak. If that happens, between 10% and 20% of people will need a second surgery .
Most people do not need medication or further surgery after surgery. However, cardiac arrhythmias can occur later. If this happens, a doctor may recommend minimally invasive procedures such as ablation .
How do I take care of my child?
You may need to limit your child's physical activity . Ask your child's pediatric cardiologist - a doctor who specializes in heart disease in children - about this.
Children with an atrioventricular septal defect (AVSD) may be advised to take antibiotics before visiting the dentist. This can help prevent a type of heart infection called endocarditis . Talk to your child's cardiologist about this as well.
When should my child see a doctor?
You should definitely take your child to their pediatric cardiologist at regular intervals. They can monitor your child's heart for any problems. Some children with AV canal defects may need further treatment after surgery. The patch may leak, or a repaired valve may leak or become narrow.
A child with an AV canal defect may also have neurological or developmental disorders . They may also need help with that.
When your child is older, they should move to an adult congenital heart specialist and go for check-ups at least once a year.
What questions should I ask my child's doctor?
You can ask your child's doctor questions like:
- What type of AV canal defect does my child have?
- What are the symptoms that indicate the condition is getting worse?
- When will he need surgery?
- Will my child need medication after the surgery?
- Will I have to limit my child's activities?
It's normal to have a range of emotions when you hear that your newborn has a heart condition like Atrioventricular Canal Defect . But your baby's doctors are there to help you, and they want to do what's best for your baby. Find out exactly what's specific to your baby's condition. If there's anything you're not sure about, ask your doctor about it. Now is the time to advocate for your baby's health.
So, what are the things we need to remember from all of this?
- Atrioventricular Canal Defect is a heart condition that is present at birth. It is mainly caused by a hole in the heart and a problem with the valves.
- Early detection and timely surgery are very important. These surgeries are highly successful.
- Babies with Down syndrome are more likely to develop this condition.
- Long-term medical supervision is required even after surgery. You need to be in contact with a cardiologist for the rest of your life.
- If you are pregnant, following a healthy lifestyle and following medical advice can help reduce your baby's risk of congenital heart disease.
- Don't be afraid, the doctors are there to help you and your baby. Ask questions, be informed.
` Heart disease, congenital heart defects, baby's heart, hole in the heart, Atrioventricular Canal Defect, AVSD, pediatric heart disease
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