Faculty of Medicine, Department of Cardiology, Marmara University, Istanbul, Turkey.
Cardiol J. 2011;18(6):691-4. doi: 10.5603/cj.2011.0035.
Left ventricular noncompaction (LVNC) is an uncommon cardiomyopathy characterized by the persistence of fetal myocardium with a pattern of prominent trabecular meshwork and deep intertrabecular recesses, systolic dysfunction and left ventricular dilatation. It is thought to be caused by the arrest of normal endomyocardial morphogenesis. There is no consensus on the definition, diagnostic criteria, pathogenesis or treatment of LVNC. We report the case of a 43 year-old patient with LVNC, nonsustained ventricular tachycardia and family history of sudden cardiac death (SCD). An implantable cardioverter-defibrillator (ICD) was prophylactically implanted because of the individual's high SCD risk. Although ICD is an effective option for preventing SCD, data on the long-term follow-up of patients with LVNC is limited.
左心室心肌致密化不全（LVNC）是一种罕见的心肌病，其特征是胎儿心肌持续存在，呈现出明显的小梁网状结构和深的小梁间凹陷，伴有收缩功能障碍和左心室扩张。它被认为是由正常的心内膜心肌形态发生的停滞引起的。对于 LVNC 的定义、诊断标准、发病机制或治疗方法尚无共识。我们报告了一例 43 岁的 LVNC 患者，伴有非持续性室性心动过速和家族性心源性猝死（SCD）病史。由于个体的 SCD 风险较高，预防性植入了植入式心脏复律除颤器（ICD）。尽管 ICD 是预防 SCD 的有效选择，但关于 LVNC 患者长期随访的数据有限。
