Abstract
Ventricular non-compaction (VNC) is a rare myocardium disorder, which can be both genetic and sporadic. A poor wall compaction process or an excessive trabeculae formation may be at the genesis of myocardial hypertrabeculation with multiple recesses. It is often complicated by ventricular dysfunction, arrhythmias and cardiac embolism. Herein we report a case of a 20-year-old male patient with no particular past medical history who was followed up at the cardiology department for dyspnea. Echocardiography showed reduced ejection fraction of the left ventricle with potential hypertrabeculation in the right ventricle, confirmed by cardiac MRI. The patient was not put under medication and was later lost to follow-up. He died few months later without a clear cause explaining death. A forensic autopsy was performed that attributed death to acute ventricle arrhythmia secondary to VNC, emphasizing the major role of an early and specific treatment to avoid such a fatal outcome.
Highlights
Ventricular non-compaction (VNC) is a complex and heterogeneous cardiomyopathy first described in 1926
The etiology and embryogenetic mechanisms leading to VNC are still unknown and several hypothesis are suggested
The most frequent on is that hypertrabeculation may result from excessive trabeculae formation and/or a defect in the later compaction processes[1,5]
Summary
Ventricular non-compaction (VNC) is a complex and heterogeneous cardiomyopathy first described in 1926 It is a rare disease with a reported prevalence of 0.014–0.17%1. Several terms are used to describe this disease namely: “cardiac hyper and excessive trabecularization”, “spongy myocardium”, “honeycomb myocardium”, or “persisting myocardial sinusoids”, and even “isolated ventricular abnormal trabeculation” It is characterized by hypertrabeculation with an excessive lace-like network of trabeculae and deep trabecular pockets in the ventricle, creating a perfect environment for thrombi formation. VNC can be detected in all age groups, ranging from the fetal period to adulthood[2] It can occur sporadically or is hereditary secondary to chromosomal abnormalities. Based on clinical history and the necropsy findings, death was attributed to acute ventricle arrhythmia secondary to a myocardium non-compaction
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