Sudden Death in a Case of Isolated Left Ventricular Noncompaction An Autopsy Case Report

摘要

Abstract: We report a case of isolated left ventricular noncompaction in a 15-year-old girl, who presented with sudden death. There were no associated anomalies or history of cardiac disease. Isolated left ventricular noncompaction (LVNC) is a rare genetic cardiomyopathy. The common clinical presentation can be heart failure, thromboembolic events, and sudden death. We present a young female patient who was apparently healthy with no medical or surgical history. She presented with sudden cardiac death. The diagnosis of isolated LVNC was made at autopsy. CASE REPORT A 15-year-old female patient who was apparently alright had sudden onset chest pain and breathlessness. She fell unconscious on her way to the hospital. She was declared dead on admission. She had no history of any medical or surgical illness. She was not on any medication. At autopsy, she was of average built and nourishment. Heart was enlarged and weighed 400 g. Apex was central and formed by the left ventricle. There was biventricular hypertrophy. There was marked hypertrophy of the left ventricular myocardium with marked increase in the trabeculated part (Fig. 1). The ratio of the trabeculated portion with the compact part was 1.5:1. Apical region of the left ventricle showed more pronounced changes. Small areas of fibrosis were noted in the compact region of the left ventricle. Also seen was subendocar-dial fibrosis. There was no thrombus seen in the left ventricle. The right ventricle showed mild ventricular hypertrophy. All the other chambers and the cardiac valves were normal on gross examination. All the other organs were normal on gross examination. The provisional cause of death given was "after histopathological examination." Histopathology sections studied from the left ventricle showed fibrosis in the subendocardial region with hypertrophy of myofibers with mild myofiber disarray. There was focal myocytolysis with focal areas of fibrosis. The gross and the microscopic features were diagnostic of noncompaction of the left ventricle. The section from the lung showed oedema fluid and pigment laden macrophages in the alveolar spaces. Other organs showed normal histology.