LVNC, otherwise known as spongy myocardium or spongiform cardiomyopathy is a rare congenital cardiomyopathy that is believed to be the result of an arrest during normal embryogenesis [3]. Ordinarily, the ventricular walls of the left and right ventricles consist of a compacted layer of myocardial fibers. In LVNC, the left ventricular apex consists of trabeculations and deep intertrabecular recesses [1,3]. These intertrabecular recesses are not compacted into capillaries and lead to a lack of development of the microcirculation [1,4,5].

Overall, LVNC has a prevalence of just under 0.25% in the general population, and has been classified as a primary genetic cardiomyopathy by the American Heart Association in 2006 [3,4]. Cases can be familial or sporadic, and symptoms can arise at any age. Heart failure is the most common complaint at admission [3]. When associated with other congenital heart diseases, studies have shown it to be associated with a mutation in the α-dystrobrevin gene and transcription factor NKX2.5 [4].

Echocardiography has been used as the initial non-invasive diagnostic method of LVNC [6,3]. Chin et al., proposed a diagnostic criteria for LVNC in which the ratio of X/Y <0.5; where X is equal to the distance from the epicardial surface to the trabecular recess trough and Y is equal to the distance from the epicardial surface to the trabeculation peak. This method evaluated the trabeculation size relative to the compacted wall thickness in different echocardiographic views [3]. Contrast echocardiography can improve the sensitivity. Other diagnostic modalities used are cardiac magnetic resonance imaging or computed tomography. Cardiac MRI usage has increased due to its ability to capture images of  the compacted and non-compacted layers with clarity; therefore, providing a better ratio of non-compacted to compacted layers [3]. On the other hand, cardiac CT is not used as often due to its limited ability to evaluate the left ventricular function [3]. It is important to distinguish LVNC from dilated cardiomyopathy, hypertrophic cardiomyopathy, acquired changes seen in pulmonary atresia, layered mural thrombus in LV, intramyocardial hematoma, mycotic invasion of the heart, or Fabry’s disease. Symptomatic patients with congestive heart failure should be treated with angiotensin-converting enzyme inhibitors, beta-blockers, diuretics afterload reducing agents or digoxin. Some patients may eventually require cardiac transplantation [3].

By itself, LVNC can predispose patients to arrhythmias, thromboembolic disease, left ventricular dysfunction, congestive heart failure, sepsis, and sudden cardiac death [6,7]. Hypertrabeculation in patients with reduced LV function are at risk of forming clots which might lead to cerebrovascular events; therefore, it is recommended to place these patients on oral anticoagulants once the diagnosis of LVNC is made [7]. Likewise, congenital left ventricular aneurysms can lead to the same disease processes [2]. Congenital LV aneurysms itself has a prevalence of approximately 0.34% [2]. In the setting of LVNC, congenital LV aneurysms are postulated to occur due to the aforementioned lack of capillary formation in LVNC, resulting in ventricular thinning and aneurysmal formation [2]. Literature review reveals only a handful of case reports with both conditions. As a result, there is limited data regarding the treatment. In one study, a patient with LVNC and left ventricular aneurysm suffered from recurring embolisms, with the authors suggesting anticoagulation be considered as an option in patients with LVNC and LV aneurysms [7]. In comparison, our patient did not suffer from thrombi, and was not on anticoagulation.