What Is Left Ventricular Noncompaction?
Left ventricular noncompaction (LVNC) is a rare heart disease characterized by anatomical structure by prominent left ventricular trabeculae and deep intratrabecular indentations. This condition causes progressive systolic and diastolic dysfunction, conduction abnormalities, and occasionally thromboembolic events. In addition, anomalies in the heart's muscles can occur during the process by which the heart develops in the embryo.
What Are the Symptoms?
The person who has LVNC might not have any symptoms, but they would still be at risk for consequences linked with the condition. An individual with heart failure, such as sudden cardiac arrest, experiences a sudden loss of heart function or an irregular heart rhythm. The symptoms of heart failure might include several different conditions.
Symptoms in people and children who are older would include:
-
Symptoms of dyspnea and weariness.
-
Feelings of faintness or vertigo.
-
A fainting spell or a syncope.
-
Palpitation.
-
Increase in weight.
-
Lymphedema - It is also known as lymphatic blockage and is a chronic illness that causes tissue edema due to the buildup of extra fluid in the affected area.
-
Problems sleeping down in a horizontal position.
-
Clots form in the blood.
Signs and symptoms in infants and younger children:
-
An abnormally high rate of perspiration in response to meals or activity.
-
Feeding issues or poor growth may be present.
It is vitally necessary for those with cardiomyopathy to monitor their risk factors.
What Causes Left Ventricular Noncompaction?
-
A gene mutation causes the LVNC at the embryological stage and has a hereditary component associated with other genetic syndromes, metabolic disorders, and mitochondrial diseases.
-
Congenital heart disease is a condition that is caused by abnormalities in the structure of the heart.
What Are the Diagnostic Methods?
Several diagnostic tests can be performed to determine the conditions, including:
Echocardiogram: The examination's sensitivity was considerably increased by using contrast, which was extensively utilized for diagnosing left ventricular noncompaction. The diagnostic approach was commonly used. The characteristic would consist of the following:
-
An appearance of two layers on the mucosal surface of the left ventricle.
-
Regional thickening of the trabeculation of the left ventricle.
-
Intertrabecular crypts and recesses are pronounced.
-
Dysfunction of the left ventricle in both the systolic and diastolic positions.
Electrocardiogram: An electrocardiogram, often known as an ECG, is a diagnostic tool that measures the heart's electrical activity to identify irregular cardiac rhythms. The modifications, which occur when the condition is present, are not specific and include left ventricular hypertrophy and repolarization problems. However, the left bundle branch block is prevalent, particularly in patients suffering from LV dysfunction.
Imaging Test: The imaging test should show the ratio of non-compacted to compacted end-diastolic myocardium, the section of the afflicted left ventricle, and the function of the left ventricle.
Plain Radiograph: The plain X-ray is used to diagnose complications of cardiomyopathy, such as pulmonary edema and cardiomegaly.
CT Scan: Due to the great spatial resolution offered by the technology, CT (computed tomography) scans are recommended as an alternative for patients with acoustic limitations that prevent them from undergoing echocardiography and who have contraindications for doing CMR (cardiac magnetic resonance imaging). The diagnostic method helps evaluate the quantitative and qualitative assessment of global and regional ventricular function and coronary artery to exclude anomalies or coronary artery disease and elicits the abnormal architecture of the left ventricular wall in noncompaction. Additionally, it helps determine whether or not a patient has coronary artery disease.
MRI: Cardiac MRI (magnetic resonance imaging) is the most accurate method for diagnosing spongiform cardiomyopathy because of its contrast resolution and the fact that it helps to estimate the percentage of left ventricular myocardial mass, the total left ventricular myocardial mass index, the proportion of non-compacted to compacted myocardium, and the trabeculation in segments.
Coronary Angiogram: Angiography is a process that utilizes X-ray imaging to visualize blood arteries after they have been filled with contrast material. This assists in determining whether or not there is a limitation in blood flow to the heart.
What Are the Complications?
There are many different issues that can arise from left ventricular noncompaction, some of which include the following:
-
Heart Failure: It is a syndrome characterized by symptoms like feeling short of breath, extreme weariness, and swelling of the legs that is brought on by a dysfunction in the heart's ability to pump blood. As the heart muscle becomes weaker, it becomes increasingly difficult for the heart to pump blood with the same efficiency level.
-
Thromboembolism: It is a disorder caused by blood clot formation in a blood vessel that breaks loose, moves through the circulatory system, and stops another blood vessel, causing significant harm to several organs and death.
-
Arrhythmias: An abnormality in the heart's rate or rhythm characterizes this condition. There would be tachycardia, also known as a rapid heartbeat, or bradycardia, sometimes known as a slow heartbeat, as well as irregular heartbeats.
What Are the Management Techniques?
-
Monitoring: Susceptible but asymptomatic individuals with LVNC are advised to undergo periodic testing and monitoring for the condition. The tests are customized to each individual's health situation. It consists of echocardiograms, cardiac MRI, stress testing, ECG, and rhythm monitoring. These examinations aid in the diagnosis of LVNC and reveal any potential problems.
-
Exercise: Patients without symptoms with LVNC and normal systolic function, no significant ventricular tachyarrhythmias on ambulatory monitoring or exercise testing, and no history of unspecified syncope may be considered for participation in competitive sports, except for those in which syncope may cause fatalities or severe injuries.
Those diagnosed with LVNC and have reduced systolic function or severe atrial or ventricular tachyarrhythmias on ambulatory ECG monitoring or exercise testing, in addition to those with a history of syncope, are advised to refrain from engaging in high-intensity activity and competitive sports.
-
Anticoagulation: If the left ventricle is dilated and dysfunctional, as well as if there is a history of embolic occurrences in the patient's past, it is recommended to take this medication to assist in avoiding thromboembolism.
-
Genetic Testing and Family Screening: Genetic testing is a medical test that identifies alterations in genes, chromosomes, or proteins. This can be done to help diagnose diseases. The outcome of a genetic test can validate the suspected genetic issue, elicit further information about it, and identify the genetic ailment.
-
Pregnancy: Preconception counseling regarding maternal and fetal risks, including the likelihood of illness transfer, is important in managing women with LVNC who are considering becoming pregnant.
-
ICD Implantation: A cardioverter-defibrillator (ICD) is indicated for individuals with LVNC due to their increased risk of sudden cardiac arrest. In addition, in certain patients with an LVNC pacemaker, resynchronization therapy is combined with ICD. The mechanical pacing from various parts of the heart would enhance the heart's capacity to provide blood to the body.
-
CRT Therapy: CRT (cardiac resynchronization therapy) aims to coordinate the contraction of the weakened heart using a special pacemaker or implantable cardioverter defibrillator (ICD) that performs this function using an additional pacing lead that is not present in a standard pacemaker. This lead is necessary for CRT therapy to be successful. CRT has been shown to improve heart function, decrease feelings of breathlessness, and increase patient survival rates. The apparatus that is able to control the rate of the heartbeat.
-
Heart Surgery and Comprehensive Support: The advanced stage of left ventricular noncompaction (LVNC) may result in serious heart failure that calls for more significant medical intervention, such as a left ventricular assist device (LVAD) or a heart transplant. Modifications to individuals' lifestyles in addition to behavioral therapy to accommodate a given diagnosis.
Conclusion
Left ventricular noncompaction (LVNC) is a structural anomaly of the heart characterized by large left ventricular trabeculae and wide intertrabecular recesses. It is an inherited feature commonly associated with many phenotypes and congenital heart abnormalities. In order to influence the long-term management and prognosis of LVNC, it is important to improve diagnostic accuracy and promote knowledge of the disease. Owing to the possibility of association with other cardiac abnormalities, neuromuscular abnormalities, and systemic anomalies, accurate identification of a non-compaction is vital. The care of LVNC includes treatment options for heart failure, arrhythmia, and thromboembolic events; however, there is presently no cure for the disease's underlying cause.
