Li Ling, Burke Allen, Zhang Xiang, Fowler David
Key Laboratory of Evidence Science, China University of Political Science and Law, Beijing, China.
Am J Forensic Med Pathol. 2010 Jun;31(2):122-4. doi: 10.1097/PAF.0b013e3181ca4b06.
Left ventricular noncompaction of the myocardium is a rare genetic cardiac disease characterized by a prominent meshwork and deep intertrabecular recesses of the left ventricle. The condition has frequently been misdiagnosed as other cardiomyopathies. Sudden deaths due to the disease are rarely reported in the literature of forensic sciences. We report 2 cases of sudden death due to undiagnosed left ventricular noncompaction. One was a 30-year-old man who suddenly collapsed while eating breakfast. He was pronounced dead on arrival at the hospital. The other 24-year-old man had a witness collapse at home. Despite immediate resuscitation, he expired 11 hours after hospitalization. Postmortem examination revealed that both patients died of left ventricular noncompaction. The key pathologic findings are described. Additionally, the literature is reviewed, and the incidence, clinical and pathophysiologic presentation, pathogenesis, and diagnostic criteria for the left ventricular noncompaction are discussed.
左心室心肌致密化不全是一种罕见的遗传性心脏疾病，其特征为左心室存在显著的肌小梁网状结构和深陷的小梁间隐窝。该病症常被误诊为其他心肌病。法医学文献中鲜有关于该疾病导致猝死的报道。我们报告2例因未确诊的左心室心肌致密化不全导致的猝死病例。1例为一名30岁男性，他在吃早餐时突然晕倒。到达医院时被宣告死亡。另1例24岁男性在家中被目睹晕倒。尽管立即进行了复苏，但他在住院11小时后死亡。尸检显示两名患者均死于左心室心肌致密化不全。描述了关键的病理发现。此外，回顾了相关文献，并讨论了左心室心肌致密化不全的发病率、临床和病理生理表现、发病机制及诊断标准。
