General description of the pathology

Non-compacted left ventricle (NSV) It is a rare form of cardiomyopathy characterized by large portions of the LV wall with a more superficial part trabecular, with deep recesses between the trabeculae, that is, “not compact”, and a deeper layer “compact”, thinner than normal (6 mm in adults). These areas have a spongy appearance and are easily recognizable with echocardiography and magnetic resonance imaging.
Areas of "non-compaction" are usually present at the tip and lateral wall of the LV, but in some patients they may also be extensive and associated with LV dilation with reduced contraction force, a finding of dilated cardiomyopathy (DCM), or in other patients with hypertrophic areas, a finding of hypertrophic cardiomyopathy (HCM). They can also be observed in the LV of patients with various types of congenital heart disease.

The areas of non-compaction are believed to derive from an "incomplete maturation” of the heart walls, in the final stage of heart development.

 

It often derives from genetic mutations, more likely to be identified, as in other cardiomyopathies, if other cases are present in family members or if associated with CMI or CMD.

Small "non-compaction areas" can be identified even in asymptomatic individuals and in trained athletes. In these cases, it is necessary to carefully distinguish whether cardiomyopathy is also present. In some cases, arrhythmias, both atrial and ventricular, can occur, even life-threatening and even leading to cardiac arrest. If associated with dilation or reduced contractile force of the LV, it can lead to heart failure or embolic phenomena.


Healthy heart

Healthy heart

Left ventricular non-compaction

Left ventricular non-compaction


Symptoms

Initially, CMI is often asymptomatic, meaning that the symptoms may be absent or not perceived by the patient and the diagnosis in these cases is made through random checks such as during a sports medical examination. The most common symptoms, when present, are:


Diagnosis

To make the diagnosis, it is necessary to perform non-invasive clinical and instrumental tests:

These tests allow us to evaluate the severity of the heart disease and the risk of developing any complications.

From the Video Library of the Heart

Video insights on cardiomyopathies

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