What happened to the left ventricular non-compaction cardiomyopathy? to be or not to be: This is the question

Abstract

For several years, left ventricular non-compaction (LVNC) was considered as a true cardiomyopathy and several definitions have followed one another.

Particularly, LVNC was characterized by prominent left ventricular trabeculae separated from deep intertrabecular recesses. Several echocardiographic criteria and cardiac magnetic resonance imaging (CMR) criteria have been used to diagnose LVNC, leading to overestimate the diagnosis of LVNC in patients with other diseases and/or physiological conditions.

Left ventricular hypertrabeculation (LVH) can be present in several cardiac diseases and physiological conditions: heart failure with reduced ejection fraction, thalassemia and other hematological diseases, pregnancy, athlete's heart. Thus, the presence of LVH does not necessarily indicate the presence of an LVNC.

In addition, the great heterogeneity of clinical manifestations has raised concerns regarding the existence of a true LVNC as a cardiomyopathy. In fact, LVNC ranges from genetic to acquired and even transient conditions, isolated forms or forms associated with other cardiomyopathies, congenital heart diseases or syndromes with a very different prognosis.

Thus, considering LVH as a manifestation of various diseases and physiological conditions, the recent 2023 ESC guidelines on cardiomyopathies did not include LVNC among cardiomyopathies, but they suggested using the term “LVH” rather than LVNC, to describe this phenotype especially when it is transient or of adult-onset.

In this review, we aimed to make an excursion on LVNC, from its initial description to the present day, to understand why current guidelines decided to consider LVH as a phenotypic trait rather than a distinct cardiomyopathy.