Is your little one's heart's aorta located in a different place? Let's learn about this (Overriding Aorta)!

Sometimes our little ones come into this world with small changes in their hearts. It is very normal for you, as a mother or father, to feel very scared and worried when you hear something like that. But don't worry, because today we are going to talk about a condition called ``Overriding Aorta'', which is a congenital heart disease. We will talk about many things in simple terms, such as what exactly this is, why it happens, what are the symptoms, and what are the treatments. If you understand these things correctly, it will give you a lot of strength to provide the best medical care for your baby.

What is this `(Overriding Aorta)`? Let's understand it simply, shall we?

Okay, let's first see what this `(Overriding Aorta)` is. Simply put, this is a major blood vessel in our baby's heart, the aorta, that is located in a slightly different place than where it should be.

Now look, in a normally healthy heart, this great artery `(Aorta)` is located on the left side of the heart, that is, connected to the left ventricle. Do you know what? That is where the oxygen-rich, clean blood coming from the lungs can be distributed throughout the body. That is its main function.

But in the case of `(Overriding Aorta)`, this aorta is located above the wall between the left and right ventricles of the heart (this is called the `Ventricular Septum`), or above a hole in that wall (`Ventricular Septal Defect` or VSD). Imagine, what happens here is like setting a water pump hose to the wrong tank.

What happens because of this? The right side of the heart has less oxygen, which means it's used up, slightly "dirty" blood. Because of that hole (`VSD`) and the aorta being in the wrong place, some of this less oxygenated blood enters the aorta and mixes with the blood circulating throughout the body. This means that the amount of oxygen in the blood reaching various parts of the baby's body is reduced. This is the main problem.

What is the relationship between this and the condition called `(Tetralogy of Fallot)`?

Overriding Aorta is not a condition that usually occurs alone. It is one of the four main features of a complex congenital heart disease called Tetralogy of Fallot . "Tetralogy" means four. So, in this condition, four major heart problems are seen together:

1. `(Overriding Aorta)`: The aorta we just talked about is in the wrong place.

2. Ventricular Septal Defect (VSD): A hole in the wall between the left and right chambers of the heart. This causes oxygen-rich blood to mix with oxygen-poor blood.

3. Pulmonary Stenosis: The pulmonary artery, which carries oxygenated blood from the right side of the heart to the lungs, becomes blocked or its valve, which does not open properly, reduces the amount of blood going to the lungs.

4. Right Ventricular Hypertrophy: Because the pulmonary artery is blocked, the right ventricle has to work harder to pump blood. Just like muscle mass increases during weight training, the walls of this ventricle thicken over time.

Imagine, it's like there's no wall between the smoking area and the non-smoking area in a restaurant. Then the smoke easily moves from one place to the other, and no one feels well. That's what happens in this `(Tetralogy of Fallot)` condition. Blood with low oxygen content and blood with high oxygen content get mixed up.

Who is most affected by this condition (Overriding Aorta)?

This condition, called ``Tetralogy of Fallot'', which includes ``Overriding Aorta,'' is statistically seen slightly more often in male babies than in female babies.

Also, babies with certain chromosomal abnormalities, such as Down syndrome, are more likely to develop Tetralogy of Fallot. This heart problem can also be seen with other very rare conditions, such as Apert syndrome and Williams syndrome.

How common is this situation?

It is said that about one in every 100 babies born in the world may have some form of congenital heart defect. When you look at it that way, about 10% of those babies with heart defects, that is, about one in 3,000 newborns, may have a condition called `(Tetralogy of Fallot)`, which includes `(Overriding Aorta)`. So, this is not very common, but it is a condition that doctors see often.

What are the symptoms of this condition?

Not all babies with Tetralogy of Fallot have the same symptoms. Some may have very mild symptoms, while others may have severe symptoms. The nature of these symptoms depends mainly on how much of the pulmonary artery is blocked. That is, how much blood is getting to the lungs. If left untreated, these symptoms can gradually worsen over time.

Minor symptoms

Some babies may show mild symptoms like these:

• Blue skin color (`Cyanosis`): This blue color is especially noticeable in areas like the lips, tongue, and fingernails. This is due to a decrease in the amount of oxygen in the blood.
• Feeling tired quickly: Your baby may seem to get tired quickly after playing or nursing.
• Shortness of breath: You may have difficulty breathing, especially when you are active.
• Appetite and weight gain problems: The baby may not eat well and may not gain weight.

Severe Symptoms and `(Tet Spells)`

Sometimes, a baby's oxygen levels suddenly drop, causing a dangerous condition called ``Tet spells.'' This can happen suddenly and can last from a few minutes to a few hours. Imagine a baby playing and then suddenly turning blue and having trouble breathing.

Severe symptoms such as these can be seen during Tet spells:

• The skin suddenly turns dark blue.
• Feeling very tired, like your body is going numb.
• Fainting may occur.
• Convulsions.
• Difficulty breathing, rapid breathing.
• The baby may suddenly start crying and then pass out.

After a ``Tet spell'' like this, the baby may sleep for a long time. These ``Tet spells'' are a condition that requires urgent medical attention.

Why do conditions like ``Overriding Aorta'' occur?

In fact, most of the time, it is difficult to find a specific reason for such defects when a baby's heart develops in the womb. That is, the cause is unclear (`unknown reasons`). However, it has been found that about 25% of babies with this condition `(Tetralogy of Fallot)` (which includes `(Overriding Aorta)`) have some birth defects or problems in other parts of their body .

How do doctors diagnose this condition (Overriding Aorta)?

As soon as your baby is born, doctors and nurses at the hospital will check the baby's health. At the same time, they will pay special attention to any abnormalities in the heart.

• Pulse Oximetry: A small sensor is placed on the baby's arm or leg to measure the amount of oxygen in the blood. Low oxygen levels can be a sign of heart disease. This is a simple, painless test.
• Heart Murmur: When the doctor listens to the baby's heart with a stethoscope, if there is a condition like ``Overriding Aorta'', a different sound, called a ``heart murmur'' , may be heard in addition to the normal heartbeat. However, not all murmurs are dangerous, but in this case, it is an important sign.

Tests to confirm

If the above symptoms are present, doctors will order tests such as these to confirm the condition and determine the exact nature of the heart problem:

• Echocardiogram (Echo): This is like an ultrasound scan of the heart. It can clearly see everything from the structure of the heart, the function of the valves, the way blood flows, the size of the VSD, and the location of the aorta. This is the main test to diagnose Tetralogy of Fallot.
• Electrocardiogram (ECG/EKG): This records the electrical activity of the heart. It can detect things like enlarged heart chambers and rhythm problems.
• Chest X-ray: This can give an idea of ​​things like the size and shape of the heart and whether the amount of blood flowing to the lungs is too low or too high.
• Computed Tomography (CT) scan or MRI scan of the heart: Sometimes, these tests are done if more detailed information about the heart and large blood vessels is needed.
• Cardiac Catheterization: In this, a very thin tube (catheter) is passed through a blood vessel in the baby's groin or arm to the heart, where the pressure inside the heart is measured, oxygen levels are checked, and sometimes a dye is injected and X-ray images are taken. This is very helpful in accurately assessing the condition of the heart before surgery.
• Blood tests: These help check things like the oxygen level in the blood and the amount of red blood cells (Hemoglobin).

How is this `(Overriding Aorta)` condition treated?

The best and only permanent treatment for this condition (Tetralogy of Fallot) is surgery to correct all four defects in the heart. This is usually done as open-heart surgery .

• Timing of surgery: In most cases, doctors recommend performing this surgery before the baby is 6 months old, or within the first year . There are times when it may be done even earlier for some babies, depending on the baby's condition.
• What happens during surgery?: The surgeon closes the hole in the wall between the ventricles (VSD) with a special patch. He also removes the blockage in the pulmonary artery and valve, allowing blood to flow properly. When these things are corrected, the problem of the overriding aorta and the thickening of the right ventricle are resolved.
• Temporary Shunt: Some babies are very small, or their pulmonary arteries are very small, so it may be difficult to do the full surgery right away. In such cases, a surgeon may temporarily place a shunt . This involves taking a branch from the baby's aorta or other large artery and connecting it to the pulmonary artery. This increases the amount of blood going to the lungs, providing relief until the baby is a little older and the full surgery can be performed.

Doctors have been performing this open-heart surgery to treat Tetralogy of Fallot since the 1950s, so this is a well-established procedure in the medical field.

Types of drugs used

In addition to surgery, some medications are also used to control symptoms and prevent complications:

• Beta-blocker medications (e.g. Propranolol): These help prevent those dangerous Tet spells from occurring and reduce their severity if they do occur.
• Diuretics: These help reduce unnecessary fluid in the body and reduce the pressure on the heart (in conditions such as heart failure).
• Antibiotics: Babies with this type of heart disease are at risk of developing an infection of the inner lining of the heart (infective endocarditis), especially during certain medical procedures like tooth extractions. Therefore, these medications are given to prevent such infections.

Are there any complications with the treatment?

Even if the surgery is successful, some complications may arise over time.

• When repairing Tetralogy of Fallot, surgeons make the pulmonary valve slightly more flexible, so many people may experience a small amount of blood leaking backward through that valve (pulmonary regurgitation).
• This increases the risk of developing heart rhythm problems (arrhythmias).
• Over time, this valve leakage increases, putting more strain on the right side of the heart, causing that part to enlarge, and can even lead to heart failure.
• Years after surgery, things like enlarged aorta (aortic root dilatation) or a leaky tricuspid valve can occur. If this happens, you may need more surgery or other treatment. That's why it's important to continue to have regular checkups.

How to control the baby's symptoms until surgery?

Until the surgery is complete, doctors will do the following to control the baby's symptoms and provide comfort to the baby:

• If necessary, administer heart medication (e.g., beta-blockers).
• Making sure the baby is getting enough fluids and nutrition.
• Check the blood's hemoglobin level regularly and, if necessary, give iron-containing syrups (to prevent anemia).
• Avoid activities that cause excessive strain on the baby, such as crying for long periods of time.
• If a ``tet spell'' occurs, holding the baby's knees close to his chest (the ``knee-chest position'') may provide relief. Your doctor will teach you how to do this.

Can the risk of this happening be reduced?

Although it is often not known exactly what causes heart disease in newborns, researchers believe that the mother's health and lifestyle during pregnancy can affect it. The following factors can increase the risk of a baby developing heart disease:

• Having a congenital heart disease in the mother, father, or another family member.
• Mother smoking during pregnancy.
• The mother develops an infection such as ``Rubella`` (German measles) early in pregnancy.
• The mother has uncontrolled diabetes.
• Taking certain medications (e.g., some seizure medications) during the first three months of pregnancy.
• Mother's alcohol use.
• Nutritional deficiencies during pregnancy.
• Mother's age exceeding 40 years at the time of pregnancy.

So, to reduce this risk in future pregnancies, it is very important to take care of your health before getting pregnant, control existing medical conditions (such as diabetes), completely avoid smoking and alcohol, and do not take any medication without your doctor's approval. If anyone in your family has heart disease, it is also wise to seek advice from a genetic counselor.

What kind of future can a baby with `(Overriding Aorta)` expect?

This may sound scary to you, but the truth is, most babies go on to live normal, healthy lives after surgery.

• More than 90% of babies who have successfully undergone surgery for Tetralogy of Fallot (which includes Overriding Aorta) grow up well and live normal adult lives. They go to school, play, work, and have families. Some live healthy lives into their 70s and 80s.
• However, if surgery is not performed, the condition is dangerous. If that happens, the symptoms will worsen over time, limiting your life expectancy. You may not even live to be 20 years old.

Therefore, the most important thing is to provide the right treatment at the right time.

How to care for the baby after surgery?

When you bring your baby home after surgery, you have a big responsibility.

• Regular follow-up visits: Your baby will need regular follow-up visits with a pediatric cardiologist. These may be more frequent at first, and then more frequent. Don't skip these.
• Protecting against respiratory infections: Children who have had heart surgery like this can develop respiratory infections, which can quickly lead to complications. So keep your baby away from people with colds and fevers. If such an infection develops, seek medical attention immediately.
• Antibiotics: Doctors may recommend antibiotics before some dental procedures or other minor surgeries to prevent heart infections (endocarditis). Ask your doctor about this.
• Nutrition and Growth: Take care of your baby's nutrition and growth.
• Activities: As your baby gets older, talk to your doctor about what activities and games he can do. Most of the time, he can do everything normally.

As we age, new heart problems may arise, so it is very important to continue these heart specialist examinations even into adulthood.

When should I see the doctor?

If your baby shows any of these signs, see a doctor or take him to the hospital immediately:

• If new symptoms appear.
• If your skin, lips, and tongue appear to be turning blue.
• If you have difficulty breathing.
• If you are reluctant to drink milk or eat food.
• If you seem to get tired quickly.

When should I go to the emergency room (ER)?

If your baby is having a severe ``Tet spell'', meaning they suddenly turn blue, have difficulty breathing, or are about to pass out, call 911 immediately or take them to the emergency room of the nearest hospital. There, they can be given oxygen and/or medications such as ``Morphine'' to control the condition.

What questions should I ask my doctor?

At a time like this, you may have many questions on your mind. Don't be afraid to ask your doctor about these things:

• Should I limit my child's sports or other activities?
• How often does my baby need to come for checkups?
• Will my baby need more surgeries? If so, when?
• What are the long-term complications to expect? What can be done to prevent them?
• What medications should be given to the baby? How long should they be given? Do they have side effects?

The most important things we want to take home from this story are

Okay, we've talked a lot about Overriding Aorta and Tetralogy of Fallot. You're probably feeling a lot of pressure right now. But, remember these things:

• Overriding Aorta is a congenital heart defect called Tetralogy of Fallot. This is when the baby's aorta is misaligned, causing oxygen-poor blood to flow throughout the body.
• Symptoms may include blue skin, fatigue, difficulty breathing, and dangerous 'tet spells'.
• This condition can be successfully treated with surgery. Surgeons have been performing this operation very successfully for decades.
• After surgery, many babies live healthy, normal lives.
• Regular medical checkups and following medical advice are the most important things. You are not alone in this journey. There are skilled doctors, nurses, and healthcare staff to help and guide you and your baby. So take heart.

If your newborn baby is waiting for surgery, be sure to ask the doctor and nursing staff about things you can do to help your baby during the procedure, and what to do if a ``tet spell'' occurs. That knowledge will be a great help to you.

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