Rigopoulos Angelos, Rizos Ioannis K, Aggeli Constadina, Kloufetos Periklis, Papacharalampous Xenophon, Stefanadis Christodoulos, Toutouzas Pavlos
Department of Cardiology, University of Athens, Greece.
Cardiology. 2002;98(1-2):25-32. doi: 10.1159/000064677.
Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy, which appears to represent an arrest in intrauterine endomyocardial morphogenesis. It is diagnosed both in children and adults. Its common presentation involves heart failure symptoms, ventricular tachyarrhythmias and thromboembolic events, but the age of onset varies widely. The diagnosis is made by the combined appearance of numerous, excessively prominent trabeculations and multiple deep intertrabecular recesses perfused from the ventricular cavity, commonly involving the apical and midventricular segments of the left ventricle. Although the peculiar echocardiographic picture may possibly lead to the correct diagnosis, this condition may be often misdiagnosed or unrecognized since it is not widely known.
心室心肌致密化不全是一种罕见的先天性心肌病，似乎代表着子宫内心内膜形态发生过程中的停滞。儿童和成人均可诊断出该病。其常见表现包括心力衰竭症状、室性快速心律失常和血栓栓塞事件，但发病年龄差异很大。诊断依据是心室腔内灌注的大量、过度突出的小梁和多个深陷的小梁间隐窝同时出现，通常累及左心室的心尖和心室中段。尽管这种独特的超声心动图表现可能有助于正确诊断，但由于该病并不广为人知，所以常常会被误诊或漏诊。
