When the doctor tells you that your baby has a heart problem, it's normal for you, as a parent, to feel a lot of fear and anxiety. Especially if it's a condition with a complicated name like 'Double Inlet Left Ventricle', and it's rare, that fear can be even greater. But don't worry. Let's talk about this simply, in a way that you can understand. Knowing this information will give you a lot of strength to face this journey.
What exactly is this Double Inlet Left Ventricle?
Simply put, this is a congenital heart defect . That is, a change that occurs when the heart is formed while the baby is still in the mother's womb. Our heart has two main lower chambers (chambers). The right ventricle and the left ventricle. But in the heart of a baby with this condition, only one of these two lower chambers functions properly . That is the left ventricle.
Normally, the two upper chambers of the heart (atria) are connected to the two lower chambers separately. But in this case, both upper chambers are connected to the same working chamber, the left ventricle . This is a rare condition called a `single ventricle defect`.
How does this condition affect the baby's body?
To understand this, let's first take a look at how a healthy heart works. Imagine that our heart is like a small house with four rooms.
1. Healthy heart: The body's oxygen-poor, "dirty" blood enters the right atrium. From there, it goes to the right ventricle, which pumps it to the lungs to pick up oxygen. The "clean" blood, which has been oxygenated by the lungs, returns to the left atrium of the heart. From there, it goes to the left ventricle, which pumps clean blood to the entire body. This way, clean and dirty blood never mix together.
2. DILV condition: In a baby with this condition, both oxygen-poor blood and oxygen-rich blood collect in the same working chamber (the left ventricle) . What happens is that these two bloods get mixed together. Then this mixed blood goes to the baby's lungs and throughout the body. As a result, the baby's body tissues do not get enough oxygen.
What are the symptoms of this condition?
These symptoms usually appear within a few days or weeks after the baby is born. Sometimes these symptoms can become severe suddenly.
| Symptom | Simply explained |
|---|---|
| Blue color of the skin and lips (Cyanosis) | The skin, lips, and nails appear blue/purple due to insufficient oxygen in the blood circulating through the body. This is also known as "blue baby." |
| Difficulty breastfeeding and weight loss | When breastfeeding, the baby becomes tired and has difficulty breathing, so they stop breastfeeding. This prevents the baby from gaining weight properly. |
| Difficulty breathing | The breathing rate is high, and you can see the chest going in when you breathe. |
| Sweating | Especially when drinking milk, your forehead and head become wet with sweat. This is because the heart has to work harder. |
| Abnormal heartbeat | The heart rate is too fast, or there is a change in rhythm. |
| Abnormal heart sounds (Heart Murmur) | When the doctor looks through the stethoscope, he hears an extra heart sound. |
What causes this? Can other heart diseases also occur?
The exact cause of this is not yet known . It often occurs during the first 8 weeks of pregnancy when the baby's heart is developing. It is believed to be a combination of genetic influences and environmental factors.
The most important thing is that this is not your fault. Don't feel bad about it because of something you did or didn't do during your pregnancy.
Often, babies with this `Double Inlet Left Ventricle` condition also have other heart defects. These can interfere with the flow of blood to the lungs.
- Aortic Coarctation:Narrowing of the main artery (aorta) that carries blood to the body.
- Pulmonary Atresia: The pulmonary valve that carries blood to the lungs is not properly formed.
- Pulmonary Valve Stenosis: Narrowing of the valve that carries blood to the lungs.
How to diagnose this disease?
Diagnosis during pregnancy
Sometimes your baby's condition can be detected before birth . This is done with a special scan called a fetal echocardiogram. This is usually done between 18 and 24 weeks of pregnancy. Your doctor may recommend this test:
- If something like an abnormal rhythm is seen in the baby's heart during a routine scan.
- If anyone in the family has congenital heart disease.
- If the mother has a medical condition that can affect heart disease, such as diabetes.
Identification after the baby is born
Often this condition is diagnosed after the baby is born.
- Pulse Oximetry (Pulse Ox) test: This test is done within 24 hours of the baby's birth. It measures the oxygen level in the baby's blood. If the oxygen level is low, it could be a heart problem.
- Medical examination: When the doctor examines the baby, he may suspect this by hearing an abnormal heart sound (murmur) or seeing the baby turn blue.
Tests to confirm the condition
Once a suspicion arises, several tests are performed to confirm it and better understand the condition of the heart.
| Test | What does this do? |
|---|---|
| Echocardiogram (Echo) | This is the most important test. It's like a scan. It allows you to clearly see the heart's chambers, valves, and how blood flows. |
| Chest X-ray | You can get an idea of the size and shape of the heart, as well as the amount of blood flowing to the lungs. |
| Electrocardiogram (ECG/EKG) | The electrical activity of the heart is measured to check for any problems with the heart's rhythm. |
| Cardiac Catheterization | This test is performed before surgery to accurately measure the pressure and oxygen levels inside the heart. This is done by passing a very thin tube through a vein in the groin into the heart. |
What are the treatments for this?
This condition is treated with a series of surgeries that are performed in stages . These surgeries do not make the heart "normal", but they do reorganize the blood flow in the heart so that the baby can live well in the long term.
First surgery: Blalock-Taussig Shunt
This is done within the first few days or weeks after the baby is born. This involves inserting a small tube (shunt) to help maintain the amount of blood going to the lungs if there is low blood flow. This allows the baby to develop properly until the second surgery.
Second surgery: Glenn Shunt Procedure
This surgery is performed when the baby is about 4-6 months old . What is done here is to connect the oxygen-poor blood coming from the upper body to the lungs, bypassing the heart. This greatly reduces the strain on the heart.
Third and final surgery: Fontan Procedure
This is done when the child is about 2-3 years old . Here, the oxygen-poor blood coming from the lower part of the body is connected directly to the lungs. After this surgery, the mixing of oxygen-poor and oxygen-rich blood almost completely stops. The oxygen level in the child's body becomes much closer to that of a healthy child.
Medications given in addition to surgery
The child will have to be given several types of medication before and after surgery.
- Digoxin: Strengthen the contraction of the heart.
- ACE inhibitors: Lower blood pressure.
- Anticoagulants: Prevent blood clotting (e.g. Aspirin).
- Diuretics: Remove excess water from the body.
Important: Before undergoing dental treatment (such as tooth extraction), it is imperative that these children be given antibiotics to prevent heart infections (endocarditis). You should discuss this with your doctor as well as your dentist.
What should you think about the baby's future?
This is a difficult question to ask, but it's important to know. A few decades ago, babies with this condition didn't live long. ButWith the advanced surgeries available today, the situation is very different.
Today, more than 70% - 80% of children who complete this series of surgeries survive beyond the age of 10. Many even successfully reach adulthood.
However, these children require lifelong follow-up with a cardiologist . Some complications may also occur, such as heart rhythm problems and frequent pneumonia. They may also be restricted from strenuous sports. However, most children can have a normal school life.
Take-Home Message
- Double Inlet Left Ventricle is a rare congenital heart disease in which only one of the main lower chambers of the heart functions.
- This causes oxygenated and deoxygenated blood to mix and travel through the body, causing symptoms such as the baby turning blue and having difficulty breathing.
- This is treated with a three-stage series of surgeries that improve the baby's blood circulation.
- Although the child will require lifelong medical supervision after these surgeries, many people go on to live successful, active lives.
- Talk openly with your doctor about all your questions and fears. You are not alone in this journey.
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