Introduction: Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disease first described nine decades ago in 1926, by Grant et al (Medicine et al. 2014). At that time and still today, LVNC has been characterized by three primary criteria: prominent ventricular trabeculations, deep intertrabecular recesses, and the development of two distinct layers of myocardium, compacted and noncompacted (Chin et al. 1990). Similar to Grant et al’s original case, the majority of early descriptions of LVNC were in association with varying forms of congenital heart disease (Stähli et al. 2013; Towbin 2010). However, evidence has emerged that there are patients who reach adulthood without any manifestations of their underlying myocardial
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At approximately four weeks gestation, the ventricles begin to develop luminal projections known as trabeculations. These spongy collections of trabeculations serve to increase the surface contact of the ventricular lumen and myocytes as myocardial mass increases prior to the development of the coronary arteries. As the coronary circulation develops from the epicardium to the endocardium, the ventricular myocardium undergoes compaction into organized myocyte bundles from the base to the apex of the heart. Compaction is typically greater in the left ventricle than the right, and is the reason trabeculations are considered a normal aspect of the right ventricular morphology and not that of the left ventricle (Sedmera & McQuinn …show more content…
In addition to congestive heart failure, 17% of patients presented with documented or presumed arrhythmias. This is particular concerning given that about 12% of those presenting with arrhythmias presented following aborted sudden death. The most common arrhythmias included ventricular tachycardia, atrial tachycardia, and reentrant supraventricular tachycardia (Brescia et al. 2013). The etiology of the arrhythmogenic myocardium in LVNC is unclear at this time though may be related to the increased dispersion of repolarization seen in more immature, non-compacted myocardium. In addition to congestive heart failure and arrhythmias, adults often present following a thromboembolic event. In these instances, the lack of laminar flow through intertrabecular recesses combined with stasis caused by a hypokinetic left ventricle are the presumed
At approximately four weeks gestation, the ventricles begin to develop luminal projections known as trabeculations. These spongy collections of trabeculations serve to increase the surface contact of the ventricular lumen and myocytes as myocardial mass increases prior to the development of the coronary arteries. As the coronary circulation develops from the epicardium to the endocardium, the ventricular myocardium undergoes compaction into organized myocyte bundles from the base to the apex of the heart. Compaction is typically greater in the left ventricle than the right, and is the reason trabeculations are considered a normal aspect of the right ventricular morphology and not that of the left ventricle (Sedmera & McQuinn …show more content…
In addition to congestive heart failure, 17% of patients presented with documented or presumed arrhythmias. This is particular concerning given that about 12% of those presenting with arrhythmias presented following aborted sudden death. The most common arrhythmias included ventricular tachycardia, atrial tachycardia, and reentrant supraventricular tachycardia (Brescia et al. 2013). The etiology of the arrhythmogenic myocardium in LVNC is unclear at this time though may be related to the increased dispersion of repolarization seen in more immature, non-compacted myocardium. In addition to congestive heart failure and arrhythmias, adults often present following a thromboembolic event. In these instances, the lack of laminar flow through intertrabecular recesses combined with stasis caused by a hypokinetic left ventricle are the presumed