Isolated left ventricular non-compaction cardiomyopathy in adults

Abstract

Left ventricular non-compaction (LVNC) is a heart-muscle disorder characterized by prominent myocardial trabeculations and deep intertrabecular recesses in the LV cavity. LVNC is often diagnosed by echocardiography and cardiac magnetic resonance imaging, but a universally accepted definition of LVNC is lacking. Although the prevalence of LVNC in adults remains unclear, improvements in diagnostic techniques account for the relatively high incidence of LVNC in recent years. The clinical presentation is highly variable from asymptomatic to symptomatic. Meanwhile, the classical triad of heart failure, ventricular arrhythmias, and systemic embolism constitute typical complications of this disease. Unfortunately, there is no specific therapy for LVNC, and management depends on the clinical manifestations. In this review, we discuss what is currently known about LVNC and conclude that multicenter registries are required for a better understanding of this rare disorder.

Non Compaction Cardiomyopathy Masquerading as Asthma: A Case Report

“…Because of its variable phenotypical and clinical presentation, it is often unrecognized and misdiagnosed as another cardiomyopathies or diseases [6,7]. The diagnosis typically requires genetic testing combined with various cardiac imaging modalities [3,6].…”

Non-compaction cardiomyopathy in an asymptomatic athlete

“…The most commonly employed definition was proposed by Jenni et al : (1) the absence of coexisting cardiac anomalies; (2) the presence of multiple, prominent trabeculations; (3) multiple deep intertrabecular recesses filled with blood from ventricular cavity, as demonstrated by colour Doppler imaging; and (4) the maximal end-systolic ratio of non-compacted endocardial layer (NC) to the compacted myocardium (C) of NC to C ratio >2 4. Our patient met all four criteria.…”

Non-Compaction Left Ventricle Myocardium: A Symptom, Syndrome or Development Variation?

“…В результате предло-женные критерии могут оказаться неспецифичными. Действительно, согласно Ikeda F, et al [6], при обсле-довании здоровой популяции у 8,3% лиц выполня-ется как минимум один ЭхоКГ диагностический критерий. Возможно, принятые в настоящее время ЭхоКГ-критерии излишне чувствительны и недоста-точно специфичны.…”

“…По мне-нию некоторых авторов, клиническая триада симпто-мов сердечной недостаточности, желудочковых арит-мий и системной эмболии -классическая клиническая картина СНМЛЖ [6]. По определению, синдром является устойчивым сочетанием симпто-мов с едиными этиологией и патогенезом.…”