Case Report: Sudden cardiac death due to ventricular myocardial non-compaction

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.

Isolated Left Ventricular Noncompaction Presenting With Heart Failure With Reduced Ejection Fraction and Intrahospital Cardiac Arrest: A Case Report and Literature Review

“…LVNC is characterized by the excessive formation of trabeculae and intertrabecular recesses in the uncompressed inner myocardial wall associated with the mesocardium, mostly affecting the apical region of the left ventricle, but in rare cases, it can affect the right ventricle [ 3 ]. It was first described in 1926, and has a reported prevalence of 0.014-0.17%, with a higher prevalence in male patients (52-86% of cases) [ 3 , 5 ]. In patients with HF, the prevalence of ventricular noncompaction was reported to be 3% [ 3 ].…”

“…The main cause of LVNC is the mutation of genes encoding sarcomeric proteins, but also mutations in genes encoding cytoskeletal, Z-line, and mitochondrial proteins may be identified [ 3 ]. The most commonly mutated genes are MYH7, MYBPC3, and TTN [ 5 ]. LVNC is an uncommon primary genetic cardiomyopathy, so first-degree relatives should undergo a screening process [ 3 , 4 ].…”

“…Initial ECG’s most common findings are intraventricular conduction delay, including left branch block, auriculoventricular block, and repolarization abnormalities such as QTc prolongation; also, LV hypertrophy is often present, although it can be normal in 13% of cases [ 5 , 9 , 10 ]. ECG abnormalities are strongly associated with LNVC, with repolarization disturbances predisposing patients to malignant ventricular arrhythmias that can lead to sudden cardiac death [ 10 ].…”

“…LVNC is managed like cardiomyopathy from other etiologies [ 4 ]. The prognosis of patients can vary according to structural and hemodynamic complications [ 5 ].…”