Correia Emanuel, Santos Luís Ferreira, Rodrigues Bruno, Gama Pedro, Ferreira Pedro, Nunes Luis, Pipa João, Cabral Costa, Dionísio Odete, Santos Oliveira
Serviço de Cardiologia, Hospital de S. Teotónio EPE, Viseu, Portugal.
Rev Port Cardiol. 2009 Nov;28(11):1277-83.
Left ventricular noncompaction (LVNC) is a rare congenital disease caused by an arrest in normal myocardial embryogenesis, leading to persistence of numerous prominent trabeculations that communicate with the left ventricle. It was first described as a congenital condition affecting children, but several cases have been reported of late presentation. The main clinical manifestations are congestive heart failure, arrhythmias (supraventricular or ventricular) and systemic embolism. We present the case of a 51-year-old patient brought to our emergency department after an episode of symptomatic ventricular flutter requiring electrical cardioversion. Two-dimensional echocardiography with color Doppler suggested the diagnosis and the three-dimensional echocardiogram revealed the deep trabeculations typical of LVNC.
左心室心肌致密化不全(LVNC)是一种罕见的先天性疾病,由正常心肌胚胎发育停滞引起,导致众多与左心室相通的粗大肌小梁持续存在。它最初被描述为一种影响儿童的先天性疾病,但已有多例迟发性病例的报道。主要临床表现为充血性心力衰竭、心律失常(室上性或室性)和全身性栓塞。我们报告一例51岁患者,在发生有症状的室性扑动并需要进行电复律后被送至我院急诊科。二维超声心动图结合彩色多普勒提示了诊断,三维超声心动图显示了LVNC典型的深部肌小梁。