LVNC is a structural heart problem that develops before birth. Later in life, it can cause symptoms like shortness of breath, irregular heart rhythms, and chest pain. Treatments include medications and cardiac assistive devices.
Left ventricular noncompaction cardiomyopathy (LVNC) is a rare congenital heart problem. It develops from faulty development of your left ventricle. The left ventricle is the main heart chamber responsible for pumping oxygenated blood to your body.
During development before birth, your heart muscle undergoes compaction, meaning it condenses into a firm, smooth muscle that expands and contracts forcefully as it pumps blood.
In LVNC, part of the muscle remains noncompacted. Part or all of the ventricle wall resembles a thick, honeycombed sponge. This can cause problems with heart function.
People with LVNC are usually born with the condition. Because the severity of noncompaction can vary, the symptoms are quite variable.
Depending on the degree and extent of LVNC, you may have no symptoms at all. You might develop symptoms as you get older. In some cases, LVNC can cause problems as early as infancy or young childhood.
Symptoms of LVNC will occur when the noncompacted area:
- reduces your heart’s ability to pump blood effectively, causing heart failure or blood clotting
- affects the heart muscle’s electrical conduction, leading to abnormal heart rhythms (arrhythmias)
People with LVNC may experience:
First, a doctor will talk with you about your symptoms, discuss your personal and family medical history, and perform a physical exam.
If they suspect LVNC or other heart disease, they will refer you to a cardiology specialist.
Tests that can help a doctor diagnose LVNC include:
- electrocardiogram (EKG), which shows your heart’s electrical rhythms and helps identify arrhythmias
- echocardiogram (echo), a dynamic ultrasound that shows your heart’s pumping action
- cardiac CT or MRI imaging, for a detailed view of your heart anatomy
If you or a family member has received a diagnosis of LVNC, the doctor may also refer you to a geneticist.
Doctors sometimes diagnose asymptomatic LVNC as part of family screening or routine athlete screening programs. Improvements in heart imaging techniques also contribute to incidental diagnoses.
There’s no cure for LVNC, but you can treat your symptoms. A doctor can also help you reduce your risk of complications.
Some people with LVNC never develop serious illness, but they still require ongoing monitoring with a cardiologist.
Depending on your circumstances, a doctor might recommend medications to:
- manage blood pressure
- regulate heart rhythm
- strengthen your heart’s pumping action
- prevent blood clots
- reduce fluid buildup
Other treatment options include:
- rhythm control devices, such as an implantable pacemaker or defibrillator (ICD), to prevent or treat dangerous arrhythmias
- cardiac assistive devices, such as a mechanical ventricular assist device (VAD), which can help your heart pump effectively when its function is very low
- a heart transplant in very severe cases
LVNC is a highly variable condition. Your specific outlook depends on:
- how severe and widespread the noncompaction is
- how much it affects your heart function
- other specifics of your heart condition
In general, people with LVNC have an increased risk of developing heart failure, arrhythmias, and blood clots. This can affect life expectancy.
In particular, when LVNC diagnosis occurs in infancy or young childhood, research shows an approximately
But many people with LVNC, including children, can go on to lead healthy lives.
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LVNC can also occur as part of a specific genetic syndrome or with other congenital heart disease.
Ventricular noncompaction usually occurs before birth. As a developing baby’s heart forms, the heart muscle originates as a mesh-like tissue. Over the course of typical development, the muscle mesh compacts and organizes to form a healthy cardiac ventricle.
But gene mutations associated with LVNC interrupt and halt this process, leaving part or all of the left ventricle thick and spongy.
Experts think LVNC is primarily a genetic condition linked to multiple different gene mutations and genetic syndromes.
Having one of the rare genetic syndromes associated with LVNC increases your risk. These syndromes include:
- Noonan syndrome
- Barth syndrome
- Roifman syndrome
- Toriello-Carey syndrome
Likewise, a family history of LVNC is present in up to
Noncompaction cardiomyopathy is diagnosed more frequently in males. However, pregnancy is a risk factor for the acquired form of LVNC.
What is the prevalence of noncompaction cardiomyopathy?
Experts estimate that
According to
Is there a cure for noncompaction cardiomyopathy?
There’s no known cure for noncompaction cardiomyopathy. However, doctors can help treat symptoms and reduce the risk of complications. In the most severe LVNC cases, doctors may recommend a heart transplant.
Can you prevent noncompaction cardiomyopathy?
LVNC typically arises due to a genetic mutation. There’s no known way to prevent LVNC.
If you have a family history of cardiomyopathy, sudden cardiac death, or sudden unexplained death, talk with a doctor. You might benefit from genetic testing to help you understand any risks to you or your future children.
Noncompaction is a genetic, developmental problem of the heart muscle. It causes LVNC, a rare but increasingly identifiable type of cardiomyopathy.
LVNC is a highly variable condition. It can present in infancy with severe early heart failure requiring a transplant. Sometimes, symptoms might not appear at all until later in life. In some cases, LVNC causes no symptoms at all.
People who have received a diagnosis of LVNC have an increased risk of heart failure and heart arrhythmias, among other complications.
If you have a family history of cardiomyopathy or sudden death, or think you are otherwise at risk of LVNC, talk with a doctor to determine your next steps.
If you have received a diagnosis of LVNC, your healthcare team can work with you to reduce symptoms and lower your risk of complications.