Today we are going to talk about a slightly complicated, but potentially very important condition of the heart. You may not have heard of this before. It is called Left Ventricular Non-Compaction (LVNC) as doctors call it. The name may sound like a big deal, but don't worry, let's keep it simple.
So what is left ventricular noncongestive heart failure (LVNC)?
Simply put, this is a condition that occurs when the lower chamber on the left side of our heart, the Left Ventricle, does not develop properly. Think about it, normally this left ventricle of our heart is a very strong, smooth piece of meat. It is the main part that pumps blood to our entire body. But in someone with this `(LVNC)`, the left ventricle is like a sponge, a little thicker, and arranged like a net. It has a hilly nature, like the inside of a loaf of bread.
This is usually considered a congenital heart condition. This means that you may have this condition in your heart from the time you were born. If not treated properly, this ``(LVNC)`` can lead to serious heart problems, that is, heart disease.
How does this LVNC condition affect the functioning of the heart?
Now look, the left ventricle of our heart has a very important function. It is this one that pumps the oxygen-rich blood from the lungs to the whole body. Just like a water motor. However, when this `(LVNC)` condition occurs, the left ventricle, which is like a sponge, cannot contract or relax properly. That is, it has difficulty in tightening and pushing the blood forward, or relaxing and filling it with blood again. So, the result is that the function of pumping blood to the whole body does not happen properly . This is why various problems start to arise.
How rare is this condition called LVNC?
This is actually a rare condition. According to medical experts, only about twelve people in a million are diagnosed with this condition each year. However, the real number may be much higher . The reason is that some people do not show any symptoms. Then they do not go to a doctor, and therefore do not receive a diagnosis.
What are the symptoms of LVNC?
This is the most important thing. Some people may not show any symptoms . However, others can develop very serious heart problems. For example, sudden cardiac death can occur. About two-thirds of people with LVNC are at risk of developing heart failure .
Okay, so let's see what kind of symptoms a person with `(LVNC)` might exhibit:
Commonly seen symptoms:
- Blood clots can form. These are very dangerous, because if these clots travel to the brain or lungs, it can be a big problem.
- You may have difficulty breathing while lying in bed .
- Fainting .
- Heart palpitations - This can feel like a drum beating inside the chest, or like the heart is racing.
- Inability to exercise, shortness of breath even with slight exertion (Dyspnea) .
- Irregular heart rhythm (Arrhythmia) .
- Swelling of the legs (Lymphedema) - especially in the ankles.
- Shortness of breath / Dyspnea - Feeling like you are out of breath even with a small amount of effort.
Why does this LVNC condition occur? What are the causes?
Most of the time, this `(LVNC)` condition is caused by a genetic change (Gene mutation) that occurs before you are born. This is a very complex thing. This genetic change can happen randomly, meaning for no reason, or it can be inherited from your parents. That means it can be something that runs in families. Just think, some families have a high incidence of this type of heart disease.
Also, a very small percentage of women may develop LVNC for the first time during pregnancy for no apparent reason. Doctors think this may be because of the extra strain on the heart muscle during pregnancy. For some women, these symptoms go away after giving birth. But for others, LVNC can have long-term effects.
How do doctors diagnose this LVNC condition?
A doctor will perform several tests to determine exactly whether or not you have this ``(LVNC)`` condition. Let's take a look at what they are:
- Echocardiogram: This looks at how well your heart is pumping blood, how well the valves are working, and whether there are any problems with the valves. It's like an ultrasound scan of the heart. This is where you first see the spongy nature of the left ventricle.
- Electrocardiogram (ECG): This measures the electrical activity of your heart. It can detect any problems with your heart rhythm.
- Special imaging tests: These include cardiac CT scans and magnetic resonance imaging (MRI). These can show the structure of the heart, such as how the heart is made and the thickness of its walls. In LVNC, the sponge-like appearance is clearly visible on these scans.
- Coronary angiogram: This tests how blood flows through the coronary arteries that supply blood to the heart, and whether there are any blockages. Sometimes this test may also be needed to rule out other heart conditions.
What are the treatments for this LVNC condition?
Treatment for LVNC varies depending on your symptoms. Not everyone is treated the same way. For example, if you have heart failure, your doctor may prescribe medications such as:
- Angiotensin-converting enzyme inhibitors (ACE inhibitors): For example, benazepril (Lotensin®) or lisinopril (Prinivil®, Zestril®) or sacubitril-valsartan (Entresto®) (angiotensin-neprilysin inhibitors). These work by relaxing and opening up your blood vessels (veins and arteries) to make it easier for blood to flow through them. This reduces the workload on the heart.
- Beta-blockers: For example, metoprolol (Lopressor®, Toprol XL®) or atenolol (Tenormin®). These block certain hormones (e.g. adrenaline), lowering blood pressure and controlling heart rate.
- Digoxin (Digoxin®, Lanoxin®): This controls your heart rate, slightly increases the pumping strength of your heart, and improves blood circulation.
- Diuretics or "water pills": These work by causing the body to excrete excess salt and water through urine. This reduces swelling and reduces the workload on the heart.
Sometimes, if there is a risk of sudden cardiac arrest, the doctor may recommend implanting a device called an Implantable Cardioverter Defibrillator (ICD) under the skin of the heart. This is a small battery-operated device. It detects a fast, life-threatening rhythm in the heart and delivers an electrical shock to the heart to restore normal rhythm. In very severe cases, if other treatments are not successful, a heart transplant may be necessary.
Can this LVNC condition be completely cured? Can it be reversed?
Oh no. There is currently no cure for LVNC . That is, it cannot be reversed. But don't worry. Treatment can improve your quality of life and reduce the complications that can occur due to heart disease . And, as I mentioned before, in the most severe cases, a heart transplant may be necessary.
How do we avoid this LVNC situation?
There is no way to prevent LVNC. This is because it is often caused by genetic factors. However, if you have the gene mutation that causes the condition, or if you think you may have it (for example, if someone in your family has it), genetic testing can be very helpful . A genetic counselor will explain your test results to you and your family. They can help you understand what it means to have LVNC and what your risk is for your children.
Are there other medical conditions associated with this LVNC condition?
Yes. Sometimes this condition called `(LVNC)` can also be seen as part of other genetic conditions (syndromes). These are a bit more complicated, but doctors also consider these conditions when diagnosing `(LVNC)`. Here are a few examples:
- Barth syndrome
- Noonan syndrome
- Roifman syndrome
- Toriello Carey syndrome
What is the future of someone with LVNC? What is the life expectancy?
The outlook for someone with LVNC can vary. It varies from person to person. With good treatment, symptoms can be controlled , and some people live a typical life expectancy.
LVNC is a potentially fatal condition, but it is not always the case . Most people with LVNC do not die from the disease. The most important thing is to see a cardiologist regularly and get tested . He or she can help you manage your heart health and reduce your risk of complications.
If I or my child has LVNC, what questions should I ask the doctor?
If you or your child has (LVNC), or if you have any concerns, it's a good idea to ask your doctor questions like these:
- What is the most likely cause of my/my child's LVNC condition? (i.e. is it genetic or something else)
- Do you recommend genetic testing for LVNC? If so, would it be important for other family members?
- What tests do I/my child need to have to diagnose LVNC? What can I expect from them?
- What are the treatment options for LVNC? Which treatment is best for me/my child? Are there any side effects to these treatments?
- If my child or I have a sudden cardiac arrest, what are the symptoms? What should I do immediately?
These questions will help you discuss them with your doctor and gain a better understanding of your condition.
So, what are the most important things we need to remember from this story?
Left ventricular non-functioning heart (LVNC) is a rare, often congenital heart disease. This is when the left ventricle of the heart does not develop properly. Instead of being a smooth, strong piece of muscle, it becomes thick and spongy. This causes the heart to not pump blood properly.
If you have LVNC, the most important thing is to see a cardiologist regularly and follow their advice . Although there is no cure for this condition, treatment can reduce your risk of complications and improve your quality of life. It is important to stay calm and get proper treatment . Remember, you are not alone, and there are doctors who can help you.
` Heart disease, left ventricle, LVNC, heart disease symptoms, heart failure, genetic diseases, heart disease treatment
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