Left ventricular non-compaction cardiomyopathy and sudden death: A case report

Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiac disease. This study presents a rare case of an 18-year-old male who died following sudden ventricular fibrillation, with LVNC identified at autopsy. The patient had a known clinical diagnosis of LVNC and a primary-prevention implantable cardioverter-defibrillator (ICD), with a left ventricular ejection fraction of 35-40%. Gross autopsy findings revealed increased trabeculation in the left ventricle, dark color changes in the trabecular muscles, and evidence of ischaemia in the anterior papillary muscle. Histopathological examination of the anterior papillary muscle showed myocardial infarction consistent with the first 24 hours post-infarction, hypertrophy in the trabecular muscles, and extensive perivascular, interstitial, and subendocardial fibrosis with positive Masson's trichrome staining. Widespread pulmonary edema, fibrin thrombi in the brachiocephalic vein, and fibrin thrombi within small blood vessel lumens were detected. This case emphasizes that LVNC is a potentially lethal disease due to electrical heterogeneity creating a re-entry arrhythmia substrate and a predisposition to myocardial infarction, which may lead to sudden cardiac death. When investigating sudden cardiac death cases in young patients, LVNC should be included in the differential diagnosis. Early diagnosis, appropriate cardiac monitoring, and preventive treatments such as the use of implantable cardioverter-defibrillators play a critical role in reducing the risk of sudden death. Postmortem genetic testing of the deceased and genetic counseling of first-degree relatives should be an integral part of management.