Cardiomyopathy is a disease of the heart muscle that impairs contraction, ultimately reducing the heart’s ability to pump blood.
Left ventricular non-compaction is one of the several types of cardiomyopathy that exist.
The heart is an organ consisting of four chambers. It has two atria and two ventricles, on the left and on the right.
Each ventricle serves a specific function. The right ventricle contracts and pushes blood into the pulmonary arteries.
The left ventricle contracts and pushes blood into the aorta. The oxygen-rich blood is then delivered to the organs, muscles and tissues in the body.
Left ventricular non-compaction is a rare hereditary disease characterized by the presence of trabeculations in the heart muscle, which is an abnormal tissue formation. This abnormality may be linked with dilatation of the left ventricle. It affects both the structure and function of the heart muscle and can lead to heart failure. Trabeculations can also increase the likelihood of clot formation, thus increasing the risk of an embolism.
Useful information: An embolism is the sudden blockage of a blood vessel by a blood clot.
Symptoms
People with left ventricular non-compaction are sometimes completely asymptomatic.
When symptoms do occur, they can vary greatly. They can include shortness of breath, especially during physical activity.
They can also include excessive fatigue that is not necessarily linked to physical activity. Some people experience palpitations, the sensation of a rapid or irregular heartbeat. Finally, edema may also develop, causing swelling in the hands, ankles or legs.
Diagnosis
The patient should consult a primary care physician, who will make an initial diagnosis before recommending further examinations.
An electrocardiogram is used to detect any rhythm and conduction disorders.
Echocardiography can help identify and diagnose ventricular non-compaction.
Magnetic Resonance Imaging (MRI) confirms the diagnosis and provides more information about the heart condition.
Since left ventricular non-compaction is hereditary, first-degree relatives of individuals affected by the disease are advised to undergo screening with a cardiologist.
Treatment
Treatment of left ventricular non-compaction can first of all include prescribing medication such as beta-blockers to treat heart failure if necessary or antiarrhythmics to correct certain rhythm disorders. Anticoagulants can also be prescribed to prevent the formation of blood clots.
Medical devices, such as an implantable cardioverter-defibrillator can be implanted to prevent cardiac arrest related to ventricular arrhythmia.
In the most acute cases, where neither medication or medical devices work, a heart transplant may be considered.
Those affected by this condition must also limit their physical activity initially to give the heart time to heal.
1 person out of 7,000
Left ventricular non-compaction affects approximately one in 7,000 people.
What is an implantable cardioverter-defibrillator?
The implantable cardioverter-defibrillator referred to in this article is a small device connected to leads that are attached to the heart. It is designed to treat ventricular arrhythmias that can result in sudden cardiac death. It should not be confused with an automated external defibrillator, which is used to revive victims of cardiac arrest.
A disease that appears early in life
Ventricular non-compaction begins during the development of the heart, which is the first functional organ to form in the embryo. This occurs between the fifth and eighth weeks of intrauterine life during an arrest in embryogenesis—the process of embryo development, including heart formation. This causes the persistence of trabeculations, leading to abnormal tissue formation.
What are trabeculations?
Trabeculations, which lead to dysfunction resulting from ventricular non-compaction, are abnormal tissue formations. To better visualize them, imagine the left ventricular chamber as a cave. Inside the cave, thin columns develop in a disorganized manner alongside other, more well-structured columns.
Reviewed and approved by Benjamin Bouyer, MD, Cardiologist at Bordeaux University Hospital (CHU)
