What is left ventricular noncompaction?

What is left ventricular noncompaction?

Left ventricular noncompaction is a heart (cardiac) muscle disorder that occurs when the lower left chamber of the heart (left ventricle), which helps the heart pump blood, does not develop correctly. Instead of the muscle being smooth and firm, the cardiac muscle in the left ventricle is thick and appears spongy.

What is LV Hypertrabeculation?

Left ventricular (LV) hypertrabeculation is defined by the presence of three or more trabeculations apically and up to the level of papillary muscles, seen in one echocardiographic view. 1 It can be distinguished from left ventricular non-compaction (LVNC) by the absence of thin compacted myocardial layer.

How is LVNC diagnosed?

The diagnosis of LVNC is based on cardiac testing, family history, medical history, and physical exam. Cardiac testing: An echocardiogram is the most common test used to diagnose LVNC, as the trabeculations within the left ventricle and overall squeeze of the heart can be measured with this test.

Is LVNC fatal?

Left ventricular noncompaction has a high mortality rate and is strongly associated with arrhythmias in children. Preceding cardiac dysfunction or ventricular arrhythmias are associated with increased mortality. Children with normal cardiac dimensions and normal function are at low risk for sudden death.

What is myocardial Noncompaction?

Noncompaction of the ventricular myocardium is a cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. This abnormality is often associated with other congenital cardiac defects, but it is also seen in the absence of other cardiac anomalies.

What is Hypertrabeculation Noncompaction?

Abstract. Ventricular hypertrabeculation (noncompaction) is a poorly characterized condition associated with heart failure. The condition is widely assumed to be the retention of the trabeculated ventricular design of the embryo and ectothermic (cold-blooded) vertebrates.

What is non compacted cardiomyopathy?

Non-compaction cardiomyopathy (NCM) is a myocardial disorder, which is thought to occur due to the failure of left ventricle (LV) compaction during embryogenesis, leading to distinct morphological characteristics in the ventricular chamber.

Is LVNC a congenital heart disease?

Background: Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy that is associated with high morbidity and mortality rates. Recently, LVNC was classified into several phenotypes including congenital heart disease (CHD).

Left ventricular noncompaction. An article from the e-journal of the ESC Council for Cardiology Practice. Left ventricular noncompaction is a rare cardiomyopathy, that should be considered as a possible diagnosis because of its potential complications which are heart failure, ventricular arrhythmias, and embolic events.

Is MRI better than Echo for left ventricular noncompaction?

Left Ventricular Noncompaction: Imaging Findings and Diagnostic Criteria. Cardiac MRI generally performs better than echocardiography in the identification of myocardial trabeculations and affords more accurate and reliable evaluation of the extent of non-compacted myocardium than does 2D echo-cardiography [ 58 ].

What is isolated noncompaction of the LV myocardium?

In 1990 Chin et al. [ 4] introduced the term “isolated noncompaction of the LV myocardium” and recognized the underlying arrest of the normal compaction process during embryo-genesis. Most commonly, the apical and midventricular segments of both the inferior and lateral walls are affected [ 5 ].

Can CMR detect left ventricular noncompaction?

Left ventricular noncompaction is a rare cardiomyopathy that should always be considered as a possible diagnosis because of its potential complications. Echocardiography is the standard tool for diagnosis, and CMR is very useful to confirm or rule out this disease, especially when the apex is difficult to visualise.