Baquero Giselle A, Colegrove Dustin J, Banchs Javier E
Penn State Hershey Heart and Vascular Institute, Hershey, Pennsylvania 17033-0850, USA.
Tex Heart Inst J. 2013;40(3):331-8.
Isolated left ventricular noncompaction is a rare form of cardiomyopathy characterized by prominent left ventricular trabeculations and intertrabecular recesses. The typical clinical manifestations are severe systolic and diastolic dysfunction, conduction abnormalities, and cardiac embolic events theorized to result from thrombus formation within the intertrabecular recesses. Evidence-based recommendations for preventing thromboembolic events in isolated left ventricular noncompaction have not been established. We report the case of a woman who, at 10 years of age, had been diagnosed with hypertrophic cardiomyopathy without systolic dysfunction. At age 30, she presented with left hemiparesis consequent to a large right-hemispheric ischemic stroke, and she was diagnosed with isolated left ventricular noncompaction. In addition to discussing the patient's case, we review the medical literature that pertains to isolated left ventricular noncompaction.
孤立性左心室心肌致密化不全是一种罕见的心肌病形式，其特征为显著的左心室小梁和小梁间隐窝。典型的临床表现为严重的收缩和舒张功能障碍、传导异常以及理论上由小梁间隐窝内血栓形成导致的心脏栓塞事件。目前尚未建立关于预防孤立性左心室心肌致密化不全患者血栓栓塞事件的循证推荐。我们报告了一名女性患者的病例，该患者10岁时被诊断为无收缩功能障碍的肥厚型心肌病。30岁时，她因右侧大脑半球大面积缺血性卒中出现左侧偏瘫，并被诊断为孤立性左心室心肌致密化不全。除了讨论该患者的病例外，我们还回顾了与孤立性左心室心肌致密化不全相关的医学文献。
