Abstract

Left ventricular noncompaction (LVNC) is a type of cardiomyopathy characterized anatomically by prominent ventricular trabeculation and deep intertrabecular recesses. The mortality associated with LVNC ranges from 5% to 47%. The etiology of LVNC is yet to be fully understood, although decades have passed since its recognition as a clinical entity globally. Furthermore, critical questions, i.e., whether LVNC represents an acquired pathology or has a congenital origin and whether the reduced contractile function in LVNC patients is a cause or consequence of noncompaction, remain to be addressed. In this study, to answer some of these questions, we analyzed the clinical features of LVNC patients. Out of 9582 subjects screened for abnormal cardiac functions, 45 exhibit the characteristics of LVNC, and 1 presents right ventricular noncompaction (RVNC). We found that 40 patients show valvular regurgitation, 39 manifest reduced systolic contractions, and 46 out of the 46 present different forms of arrhythmias that are not restricted to be caused by the noncompact myocardium. This retrospective examination of LVNC patients reveals some novel findings: LVNC is associated with regurgitation in most patients and arrhythmias in all patients. The thickness ratio of the trabecular layer to compact layer negatively correlates with fractional shortening, and reduced contractility might result from LVNC. This study adds evidence to support a congenital origin of LVNC that might benefit the diagnosis and subsequent characterization of LVNC patients.

Highlights

  • Left ventricular noncompaction (LVNC: OMIM No 604169) is a type of cardiomyopathy anatomically characterized by prominent ventricular trabeculation and deep intertrabecular recesses [1–3]

  • One of them is whether LVNC patients associate with novel clinical ffeeaattuurreess iinn aaddddiittiioonn ttoo tthhee rreeppoorrtteedd ssyymmppttoommss

  • We analyzed the clinical symptoms of 45 LVNC and 1 right ventricular noncompaction (RVNC) patient from 9582 echocardiograms performed on subjects with potential heart diseases

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Summary

Introduction

Left ventricular noncompaction (LVNC: OMIM No 604169) is a type of cardiomyopathy anatomically characterized by prominent ventricular trabeculation and deep intertrabecular recesses [1–3]. It was reported for the first time in 1969, being addressed as a spongy myocardial condition back [4]. Its symptoms are progressive, considered the 3rd most common cardiomyopathy in the pediatric population, and the mortality of patients with LVNC ranges from 5% to 47% [6–8]. The focus of most clinical investigations on isolated adult LVNC patients failed to trace the anomalies in embryonic developmental stages, and the knowledge gap regarding the molecular level regulation of trabeculation could be the possible reason [10,11]

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