Left Ventricular Noncompaction Is Associated with Valvular Regurgitation and a Variety of Arrhythmias

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.

Clinical Features of Noncompaction Cardiomyopathy Across All Age Strata

“…This suggests that the prevalence of valvular heart disease in NCM patients within these age groups may not exhibit a strong age-related correlation. In our investigation, MR emerged as the most prevalent among valvular diseases, aligning with ndings from prior research 4,11,20 . Zou et al underscored the robust association between MR and diminished overall left ventricular function, along with geometric remodeling characterized by left ventricular diastolic and end-systolic enlargement in NCM patients.…”

“…The anatomical feature of deep recessions in NCM patients has been proposed to facilitate the in ltration of Purkinje bers into the myocardium, potentially causing irregular depolarization and delayed repolarization, ultimately leading to arrhythmias in LVNC 18 . Arrhythmia stands out as a prominent clinical manifestation in LVNC patients, with previous research indicating that more than 50% of LVNC patients experience arrhythmias [18][19][20] . In the present study, the incidence of arrhythmias in the population aged over 1 year approached or exceeded 50%, while the proportion of arrhythmias in the 0-1 year old group remained notably low (11.8%).…”

Myocardial Mechanics and Associated Valvular and Vascular Abnormalities in Left Ventricular Noncompaction Cardiomyopathy

“…In the MAGYAR-Path Study, 33% − 33% of patients showed regurgitation of grades 1 and 2 MV [ 18 ]. In other studies, 48.2% and 78% of the subjects showed MV regurgitation [ 69 , 88 ].…”

Advances in symptomatic therapy for left ventricular non-compaction in children

“…Sotalol has been proved to be effective in the treatment of ventricular arrhythmia and atrial fibrillation in both adults and pediatrics ( 67 , 68 ). It is worth mentioning that antiarrhythmic drugs should be used with caution because of their side effects and unknown risks ( 35 , 69 , 70 ) ( Figure 2 ).…”

“…In conclusion, patients with LVNC of any type should be closely followed up and analyzed because of the conditions’ unknown nature and poor prognosis ( 132 – 134 ). Especially when LVEF function decreases, the prognosis of patients will be worse ( 28 , 70 , 135 ). We also recommend perfect genetic testing for every LVNC patient and their immediate family members, which will be of great importance to advance our research for patients with LVNC.…”