When the doctor tells you that your little one has a heart condition called "Shone's Complex," you may feel shocked, scared, and confused . It's very common. You may have never heard of this name before. "What is this disease? What will happen to my baby?" You may have many questions in your mind. Don't worry. Let's talk about this simply and clearly. What is Shone's Complex? Let's understand exactly what it does to the baby.
Simply put, what is this Shone's Complex?
Shone's Complex is a congenital heart disease . What happens in this is that there is an obstruction in more than one place in the blood flow path to the left side of our baby's heart. Imagine, the left side of our heart is the one that takes the oxygen-rich, clean blood from the lungs and pumps it to the whole body. Just like a water motor. Now what happens if there are blockages or lesions in this blood flow path? It becomes difficult for the heart to take in blood, and it becomes difficult to pump blood to the body. That's what happens in Shone's Complex.
This condition is called Shone's Complex, after the doctor who first described it. Although he identified four heart defects associated with it, doctors have now discovered that several more defects are involved.
What heart defects can be associated with Shone's Complex?
To be diagnosed with Shone's Complex, a baby must have at least two of the eight heart defects we'll talk about below. Let's see what they are. Some of the words may seem a little complicated, but I'll explain them simply.
| Heart Defect | Simply put, what happens with this? |
|---|---|
| Cor triatriatum | An extra membrane forms inside the upper left chamber of the heart (left atrium). This divides the chamber into two parts, preventing blood from flowing into the lower chamber (left ventricle). |
| Supravalvular mitral ring | A ring of fibrous tissue develops just above the mitral valve in the heart. This narrows the opening through which the valve opens, restricting blood flow. |
| Parachute mitral valve | Normally, there are two muscles (papillary muscles) that help the mitral valve open and close. In this defect, there is only one. It's like all the strings of a parachute are gathered in one place. This can cause the valve to narrow or blood to leak backward. |
| Hypoplastic left ventricle | "Hypoplastic" means not developed properly. Here, the left ventricle, the main pumping chamber of the heart, is not developed properly. It's small. So it can't pump blood properly. |
| Subaortic stenosis | Extra tissue builds up under the aortic valve, which pumps blood into the aorta, narrowing the area. This narrowing makes it harder for the heart to pump blood. |
| Bicuspid aortic valve | Normally, the aortic valve has three flaps. But babies with this defect have only two. This can cause the valve to narrow or leak blood. |
| Small aortic valve annulus | The strong ring (annulus) around the base of the aortic valve becomes smaller than normal, so the amount of blood that can pass through the valve into the aorta is reduced. |
| Coarctation of the aorta | A narrowing in the aorta, the main blood vessel that carries blood to the body, causes the heart to work harder to pump blood through the narrowed area. |
The important thing is that if two or more of these eight defects are present, it is what doctors call Shone's Complex. Most of the time, there is one obstruction on the way in and one obstruction on the way out.
What are the symptoms if my baby has this condition?
Some babies with this condition, if severe, start showing symptoms almost immediately after birth . This is because the baby's heart is unable to pump enough blood to the body. However, sometimes, symptoms can appear during childhood or even adolescence.
Look for these signs in your child:
- Difficulty gaining weight: Not gaining weight properly.
- Feeling tired while drinking milk: After drinking some milk, you start sweating and have difficulty breathing.
- Decreased urine output: Passing less urine than normal.
- Weak pulse: The pulse is very weak, especially in the hands and feet.
- Difference in blood pressure between arms and legs: Blood pressure in the arms is higher than in the legs.
- Accumulation of lactic acid in the blood.
If your child has one or more of these symptoms, it is very important to talk to your doctor as soon as possible.
Why did this happen to my baby? What is the cause of this?
Now you may be wondering, "Why did this happen to my baby? Is it my fault?" Actually, doctors have not yet found a specific cause for this. Most of the time, it occurs sporadically. We still don't have enough information to say for sure whether it is a genetic or environmental factor.
Although some research suggests that it may be hereditary, the way it is passed down within families is very complex. So more research is needed to fully understand this.
So please remember, this is not your fault. We have no control over these things.
What are the complications that can occur due to this condition?
Shone's Complex can cause some complications, which is why prompt treatment and long-term monitoring are important.
- Pulmonary hypertension: An abnormal increase in blood pressure in the blood vessels that carry blood to the lungs.
- Abnormal heart rhythms: Irregular heart rhythms.
- Heart failure: A condition in which the heart is unable to pump enough blood to the body.
How do doctors find this?
Doctors use several tests to diagnose this disease.
- Fetal echocardiogram: This is a special scan done while the baby is still in the womb. It can detect heart problems before the baby is born.
- Transthoracic echocardiogram (TTE): An ultrasound scan of the heart performed after the baby is born. This test is mainly used to diagnose Shone's Complex.
- Cardiac CT scan or MRI: These are not usually done for everyone, but in some cases, these tests can help provide more in-depth information about the structure of the heart.
Most often, the disease is diagnosed before or after birth. However, sometimes the disease is first diagnosed in childhood or adolescence, when symptoms are mild and not so severe. In such cases, a doctor may refer for further testing because of an abnormal sound (heart murmur) heard when listening to the heart.
What are the treatments for this?
The only way to treat Shone's Complex is surgery . There are currently no medications that can cure this condition. However, the doctor may prescribe medication to control the baby's symptoms and support the heart's function until surgery.
There is no specific surgery for this. Instead, doctors will decide what surgery is needed based on what heart defects your child has and how well the heart is working.
Your child may need one or more of the following surgeries:
- Removal of the extra tissue ring around the mitral valve.
- Mitral valve repair.
- Mitral valve replacement (this is a little less common).
- Removal of excess tissue beneath the aortic valve.
- Widening the opening of the aortic valve.
- Repairing a narrowed part of the aorta.
Surgical procedure
Sometimes doctors do this in a staged approach . That is, they don’t do the surgery all at once. They do it in two parts, separated by a period of time. For example, the first surgery removes the blockage in the way blood flows out of the heart. Then, the second surgery removes the blockage in the way blood flows into the heart. Your doctor will explain to you exactly how many surgeries your child will need and when they are best done.
What is the child's future life and prognosis?
The child's future life and well-being depend on several factors:
- Severity of the disease: How severe the heart defect is.
- Speed of treatment: How quickly treatment is started.
- Complications: Whether or not other complications will occur.
- Recurrence of problems: Will previously treated heart defects recur over time?
For example, things like coarctation of the aorta can recur over time. So, as your child gets older, more surgery may be needed. Your doctor will give you more information about this depending on your child's condition.
Many studies have shown that the long-term survival rate after surgery is more than 75%, up to 20 years . That means that three out of four babies live well and healthy lives after treatment.
You may have a thousand questions in your mind after hearing about your child's diagnosis. That's very normal. Write down the questions that come to mind in a notebook. When you go to see the doctor, ask all of them. Your child's medical team will explain all of this to you and guide you on what to do next.
Take-Home Message
- Shone's Complex is a rare congenital heart disease that causes blood flow to the left side of the heart to be obstructed.
- To be diagnosed with this disease, a child must have at least 2 of 8 specific heart defects.
- The only treatment for this is surgery. Surgery is often successful.
- After surgery, the child will need lifelong follow-up with a cardiologist. Do not miss any of these appointments.
- The most important thing is that this situation is not your fault or your partner's fault. Don't worry about it.
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