Thuny F, Philip E, Caucino K, Ambrosi P, Jacquier A, Avierinos J F, Vailloud J M, Aguirre L, Lambert M, Bartoli J M, Habib G
Service de cardiologie, hôpital de la Timone, bd Jean Moulin 13005 Marseille.
Arch Mal Coeur Vaiss. 2003 Apr;96(4):339-43.
Isolated non compaction of the left ventricle is a rare congenital cardiomyopathy linked to an arrest of normal myocardial embryogenesis. We report two cases of isolated non compaction of the left ventricle discovered by echocardiography in 2 males of 30 and 55 years. The first had progressively worsening cardiac insufficiency, the second was being followed for an unexplained cardiomyopathy. In both cases, the diagnosis was able to be confirmed by transthoracic echocardiography, supported by MRI data. Although present from birth, this condition can become apparent at various ages and is complicated by sudden death (principal cause of mortality), severe cardiac insufficiency, or thrombo-embolic accidents. The diagnosis of left ventricular non compaction should be considered when faced with unexplained cardiac insufficiency in the adult.
孤立性左心室心肌致密化不全是一种罕见的先天性心肌病,与正常心肌胚胎发育停滞有关。我们报告两例经超声心动图发现的孤立性左心室心肌致密化不全病例,患者为两名30岁和55岁男性。第一例患者心脏功能不全逐渐加重,第二例因不明原因心肌病接受随访。在这两例中,经胸超声心动图诊断均得以证实,并得到了MRI数据的支持。尽管该病自出生就存在,但在不同年龄段都可能显现出来,并会并发猝死(主要死亡原因)、严重心脏功能不全或血栓栓塞事件。成人出现不明原因的心脏功能不全时,应考虑孤立性左心室心肌致密化不全的诊断。