Left ventricular noncompaction cardiomyopathy: Recent advances

Abstract

From its initial description to the present day, left ventricular noncompaction cardiomyopathy has been the subject of numerous studies and publications. In question as a real cardiomyopathy, left ventricular noncompaction can appear in isolation or in association with other cardiac malformations, genetic syndromes, and neuromuscular disorders. As a genetically heterogeneous disorder, it can be sporadic or familial, with an autosomal dominant pattern with variable penetrance most frequently observed. Different diagnostic criteria have been described through the years, first by using echocardiogram and later on by cardiac magnetic resonance. The lack of universally accepted diagnostic criteria has led to the condition being over-diagnosed in the general population. Differential diagnosis between real cardiomyopathy, epiphenomenon (phenocopy in the setting of loading conditions or even other cardiomyopathies), and physiological hypertrabeculation, like in the athlete's heart must be considered. Clinically it can present as heart failure, ventricular arrhythmias, and even sudden death, but it can also be asymptomatic during familial screening. The main prognosis factors are left ventricular dilatation, dysfunction, and fibrosis. There is no specific treatment. Familial screening is recommended and special recommendations in the case of athletes must be taken into account. In the present article, we review the myth and reality concerning main and more recent aspects of left ventricular noncompaction.

Biventricular non-compaction cardiomyopathy: Rare disease and far rarer case

“…Reported complications in IVNC are heterogeneous and poorly understood [5]. The present case study shows that IVNC may include both ventricles and present as thromboembolic events deriving from both sides of the heart.…”

Impact of Cardiac Magnetic Resonance on the Diagnosis of Left Ventricular Noncompaction—A 15-Year Experience

“…Although the first LVNC case was described almost a century ago, there is ongoing discussion about its classification and diagnostic criteria [1]. The WHO described LVNC as unclassified cardiomyopathy [14], while the American Heart Association classified LVNC as a primary genetic disorder [15], and according to the European Society of Cardiology (ESC) working group, LVNC belongs to the unclassified cardiomyopathies [16].…”

“…Cardiomyopathies encompass a heterogeneous group of diseases affecting the cardiac muscle. One of them is left ventricular noncompaction (LVNC), wherein the myocardium has a bilayered structure with a compacted thin epicardial layer and a much thicker, trabeculated noncompacted endocardial layer with deep recesses [1]. LVNC can be isolated or can coexist with congenital heart disease [2].…”

Can left ventricular entropy by cardiac magnetic resonance late gadolinium enhancement be a prognostic predictor in patients with left ventricular non-compaction?

“…15,16 In this study, the incidence of MACE in patients with LVNC was 34.9%, with HF occurring most frequently, which was consistent with previous reports. [17][18][19] Therefore, investigating the prognosis of patients with LVNC has significant clinical implications. Many studies have been conducted to predict the risk of MACE in patients with LVNC using the thickness of non-compacted myocardium and LV trabeculated mass, atrial size, LVEF, LGE, brain natriuretic peptide, and genes 3,7,[19][20][21][22][23] with LVEF and LGE being the most commonly used predictors.…”

“… 15 , 16 In this study, the incidence of MACE in patients with LVNC was 34.9%, with HF occurring most frequently, which was consistent with previous reports. 17 , 18 , 19 Therefore, investigating the prognosis of patients with LVNC has significant clinical implications.…”