Are you aware of this rare condition in your baby's heart? (Double Outlet Right Ventricle)

Does your newborn baby's skin and lips look a little blue? Does he seem to be having a little trouble breathing? Or does he sweat a lot when he drinks some milk? Don't just dismiss these as coincidences. Sometimes these can be signs of a heart condition that your baby is born with. Today we're talking about a rare heart condition that you, as a parent, should definitely be aware of. That's Double Outlet Right Ventricle, or DORV for short.

Simply put, what is Double Outlet Right Ventricle (DORV)?

To understand this, let's first think about how a healthy heart works. Our heart is like a small house with four rooms. Two upper rooms and two lower rooms. The two large, strong rooms below are called ventricles. One is on the right side (Right Ventricle), and the other is on the left side (Left Ventricle).

This is what usually happens:

• The main blood vessel that carries oxygen-poor, "dirty" blood to the lungs, the pulmonary artery, begins in the right ventricle .
• The largest blood vessel, the aorta, begins from the left ventricle, carrying "clean" blood that has been oxygenated from the lungs throughout the body.

It's very neat, isn't it? One big tube in each room.

Now, in the case of DORV, this order is disrupted. In a baby with DORV, both of the main blood vessels we mentioned, the Pulmonary Artery and the Aorta, are either completely or partially connected to the Right Ventricle. Then, the Left Ventricle is either not connected to any main blood vessel, or only a very small part is.

This raises two questions:

1. The oxygen-rich, clean blood that needs to travel to other parts of the body is mixed with the oxygen-poor, impure blood.

2. More blood is being pumped through the lungs than is needed. This puts a lot of strain on both the heart and lungs. Over time, these organs can become damaged.

What other problems can be seen with DORV?

Babies with DORV sometimes experience other birth complications. Some of these include:

• Ciliary dysfunction: Problems with the functioning of the very delicate, hair-like parts of the baby's respiratory tract.
• Heterotaxy: The position of the baby's chest and abdominal organs in places other than where they are normally located.
• Intestinal malrotation: An abnormal twisting of the baby's intestines.
• Pulmonary stenosis: Narrowing or narrowing of the valve between the right ventricle and the pulmonary artery.
• Ventricular hypoplasia: One or both chambers of the heart are smaller than normal.

Are there different types of DORV? How can these be distinguished?

Yes, there are several types of DORV. This classification is determined by one very important thing. That is, almost every baby with DORV also has a hole in the wall between the two chambers of the heart (Ventricular Septal Defect - VSD). This hole prevents blood from flowing back and forth between the two chambers.

Doctors classify DORV based on where the VSD, or hole, is located.

Knowing exactly what type this is is very important for planning treatment.

Mom and Dad, be aware of these symptoms!

Symptoms of DORV usually appear within the first few days or weeks after birth. You should be very careful about these.

What causes this condition? What complications can occur?

In fact, researchers have not yet found a definitive cause for DORV. However, it has been found that about 50% of babies with the condition have some kind of change in their chromosomes, the parts of our genetic information (DNA).

If DORV is not treated properly, complications can develop over time.

• Heartbeat irregularities (Arrhythmias)
• Heart valve issues
• Heart failure
• Pulmonary hypertension
• Infection of the inner lining of the heart (Endocarditis)

How do doctors diagnose DORV status?

Sometimes, this type of heart disease can be detected even before the baby is born, during scans during your pregnancy. The special scan used for this is called a ``Fetal Echocardiogram''.

If that's not possible, doctors will suspect this only when the symptoms we talked about earlier appear after the baby is born. Then they will examine the baby.

• Check to see if the baby's skin is turning blue.
• The heart is listened to with a stethoscope to check for any abnormal sounds (murmurs).
• Pulse Oximetry is a test that measures the amount of oxygen in the baby's blood by placing a small clip-like device on the baby's finger or ear. This is a simple test that does not hurt the baby.

After these initial tests, several more tests may be done to confirm the diagnosis.

• Noninvasive imaging tests: CT scans, MRI scans, and X-rays performed outside the baby's body.
• Electrocardiogram (ECG or EKG): A painless test that measures the electrical activity of the heart.
• Echocardiogram (echo): This is like a scan of the heart. It uses sound waves to clearly see everything about the heart, including its shape, function, and how its blood vessels are connected.
• Cardiac catheterization: In this, a very thin tube (catheter) is inserted into the heart through a blood vessel in the baby's groin or arm to obtain very accurate information about the heart's pressure, oxygen levels, and structure.

Don't worry, there is a treatment for this! - Let's learn about surgery

Almost every baby with DORV will need open-heart surgery within the first year of life. This may sound scary to you. That's normal. But remember, with today's medical technology, these surgeries can be performed very successfully.

Your doctor and cardiac surgeon will consider several factors when making decisions about surgery.

• What is the baby's DORV type?
• Whether there are any other problems with the baby's heart or other organs.
• The baby's overall health.

There are several types of surgeries that are commonly performed.

• Intraventricular repair: In this procedure, the surgeon creates a tunnel through the VSD, or hole in the heart, using a special patch. This tunnel creates a path for blood to flow from the left ventricle to the aorta.
• Biventricular repair or arterial switch: If both of the baby's ventricles are in good condition and the DORV is a subpulmonary VSD (Taussig-Bing) type, the surgeon may perform a surgery to remove the aorta and connect it to the left ventricle.
• Univentricular repair (Fontan operation): If the DORV is very complex, the surgeon may recommend this surgery. This involves directing oxygen-poor blood from the lower body directly to the lungs. This is a complex surgery that involves several stages.

There are risks with any heart surgery. These include bleeding, blood clots, infection, and problems with anesthesia. But doctors do their best to minimize these risks.

What will life be like after the surgery?

After the surgery, the baby will need to stay in the hospital for a week or more. The length of time will vary depending on the type of surgery performed and the baby's condition.

But one thing to remember is that a person who has undergone DORV surgery must remain under the supervision of a cardiologist for the rest of their life. It is imperative to go for check-ups at the scheduled time and undergo the necessary tests.

Some people may experience irregular heartbeats or heart problems even years after surgery, so it's important to always take care of your health.

When to see the doctor and when to go to the ETU

Even after surgery, keep a close eye on your child. If the following symptoms appear, notify your doctor immediately.

• If you feel an abnormal, rapid, or pounding heartbeat.
• If you experience chest pain or difficulty breathing (these may be signs of a heart attack).

In the following cases, take the child to the nearest hospital's Emergency Treatment Unit (ETU) without delay.

Important questions to ask your doctor

It's normal to have a lot of questions when you find out your baby has DORV. Don't keep anything to yourself. Ask your doctor everything and get it explained. Here are some questions you can ask.

1. What type of DORV does my child have?

2. What kind of surgery do you want to have?

3. When should the surgery be performed?

4. What are the risks of surgery?

5. What are the risks of not having surgery?

6. Will my child need more surgeries after this surgery?

7. Will my child have to take medication for the rest of his life?

When you know everything properly by asking questions like these, you will be able to make informed decisions without fear.