ISOLATED NONCOMPACTION OF LEFT-VENTRICULAR MYOCARDIUM - A STUDY OF 8 CASES
ISOLATED NONCOMPACTION OF LEFT-VENTRICULAR MYOCARDIUM - A STUDY OF 8 CASES
复制标题
DOI：
10.1161/01.cir.82.2.507
复制
发表时间：
1990-08-01
期刊：
影响因子：
37.8
通讯作者：
MOHRMANN, R
中科院分区：
文献类型：
--
作者：
CHIN, TK;PERLOFF, JK;MOHRMANN, R
孤立性左心室心肌致密化不全是一种罕见的肌内膜形态发生障碍，其特征为大量、过度突出的心室小梁和深的小梁间隐窝。这项研究包括八个班级，其中三个在necrosspy。年龄11个月~ 22.5岁，随访5年。大体形态学严重程度范围从中度异常心室小梁到严重异常、松散致密的小梁。超声心动图图像具有诊断性，与尸检时的形态学表现一致。通过定量超声心动图X-Y比评估小梁间隐窝的深度，其显著大于正常对照组（p < 0.001）。该疾病的临床表现包括左心室收缩功能抑制5例，室性心律失常5例，全身栓塞3例，独特的面部畸形3例，家族复发4例。我们的结论是，孤立的左心室心肌致密化不全是一种罕见的，如果不是唯一的疾病，具有特征性的形态特征，可以确定的二维超声心动图。心血管并发症的发生率很高。这种疾病可能与面部畸形和家族复发有关。
Isolated noncompaction of left ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations and deep intertrabecular recesses. This study comprised eight class, including three at necrospy. Ages ranged from 11 months to 22.5 years, with follow-up as long as 5 years. Gross morphological severity ranged from moderately abnormal ventricular trabeculations to profoundly abnormal, loosely compacted trabeculations. Echocardiographic images were diagnostic and corresponded to the morphological appearances at necropsy. The depths of the intertrabecular recesses were assessed by a quantitative echocardiographic X-to-Y ratio and were significantly greater than in normal control subjects (p < 0.001). Clinical manifestations of the disorder included depressed left ventricular systolic function in five patients, ventricular arrhythmias in five, systemic embolization in three, distinctive facial dysmorphism in three, and familial recurrence in four patients. We conclude that isolated noncompaction of left ventricular myocardium is a rare if not unique disorder with characteristic morphological features that can be identified by two-dimensional echocardiography. The incidence of cardiovascular complications is high. The disorder may be associated with facial dysmorphism and familial recurrence.