Cavusoglu Yuksel, Ata Necmi, Timuralp Bilgin, Gorenek Bulent, Goktekin Omer, Kudaiberdieva Gulmira, Unalir Ahmet
Department of Cardiology, Faculty of Medicine, Osmangazi University, Eskisehir, Turkey.
Echocardiography. 2003 May;20(4):379-83. doi: 10.1046/j.1540-8175.2003.03045.x.
Noncompaction of the ventricular myocardium is a rare, unclassified cardiomyopathy due to an arrest of myocardial morphogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular spaces communicating with the left ventricular (LV) cavity. The disease typically involves the LV myocardium, but right ventricular (RV) involvement is not uncommon. The clinical manifestations include heart failure (HF) signs, ventricular arrhythmias and cardioembolic events. Noncompacted myocardium may occur as an isolated cardiac lesion, as well as it can be in association with congenital anomalies. We describe two illustrative cases of noncompaction of the ventricular myocardium, a 19-year-old male with bicuspid aortic valve and progressive worsening of HF, and a 61-year-old male with marked RV involvement in addition to LV apical involvement, both with the typical clinical and echocardiographic features of the disease.
心室肌致密化不全是一种罕见的、未分类的心肌病，由心肌形态发生停滞所致。其特征性超声心动图表现包括多个突出的心肌小梁以及与左心室（LV）腔相通的深陷小梁间隙。该病通常累及左心室心肌，但右心室（RV）受累也并不少见。临床表现包括心力衰竭（HF）体征、室性心律失常和心源性栓塞事件。致密化不全心肌可作为孤立的心脏病变出现，也可与先天性异常相关。我们描述了两例心室肌致密化不全的典型病例，一例是一名19岁男性，患有二叶式主动脉瓣且心力衰竭进行性加重；另一例是一名61岁男性，除左心室心尖部受累外，右心室也有明显受累，两例均具有该病典型的临床和超声心动图特征。
