Left Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is an heart disorder characterized by a heart that has not fully packed together its individual tissue layers, leading to deep recesses within the muscle wall. It affects approximately 1 in 700 people. LVNC can sometimes be caused by mutations in genes that encode for structural proteins of heart muscle. There is approximately a 1 in 5 chance that someone will develop LVNC if a parent, sibling or child has it.

People with LVNC may experience blood clots in the heart, heart failure, and unstable heart rhythms. They also carry a higher risk of stroke and sudden death than the general population. Nonetheless, many people with LVNC can be entirely without symptoms for their whole life.

 

LVNC is diagnosed by echocardiogram or MRI (magnetic resonance imaging). A careful family history can reveal if the LVNC seems to follow an inherited pattern.

 

Treatment for LVNC is recommended to ameliorate symptoms and prevent complications. Physicians may suggest beta-blockers for unstable heart rhythms and may recommend anti-coagulants to prevent the risk of blood clots. Other medications may be used for heart failure symptoms. If a patient is thought to be at high risk of sudden death, a defibrillator is sometimes implanted by an electrophysiologist.

 

Genetics

A small number of different genes have been associated with LVNC and these genes are responsible for a minor percentage of individuals with the condition. As the scientific understanding of LVNC progresses, we expect to identify more genes responsible for LVNC.

Genetic testing can be used to try to identify the genetic mutation causing LVNC so other family members can have genetic testing to determine their risk for abnormal heart rhythms associated with the condition.

 

Family members

Since LVNC syndrome can be a genetic condition, the family members of someone who has the condition may be at risk of developing the abnormal heart rhythms, heart failure or sudden death. Close relatives (parents, siblings, and children) of someone with LVNC should be evaluated for the condition by consulting a cardiologist familiar with LVNC. In some cases, genetic testing can be performed if the gene mutation associated with the condition in the family has been found.

Precautions

Individualized recommendations about exercise and diet should be made with the help of a cardiologist.

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