Dulac Y, Heitz F, Baunin C, Roux D
Service de médecine infantile C, CHU Purpan.
Arch Mal Coeur Vaiss. 1995 May;88(5):761-4.
A two and a half year old child was admitted to hospital with peripheral cyanosis. Echocardiography revealed severe dilated cardiomyopathy affecting both ventricles with abnormally hypertrophied and irregular myocardial walls. The pulmonary hypertension was suprasystemic with raised pulmonary resistances. The magnetic resonance imaging and angiography confirmed the diagnosis of persistence of spongy myocardium. This is a rare abnormality due to the persistence of embryonic myocardium with a network of trabeculations and intertrabecular spaces as usually observed during echocardiography. This case is noteworthy because of the biventricular involvement and the absence of associated congenital cardiac malformation. The prognosis is characterised by a high risk of severe cardiovascular complications.
一名两岁半的儿童因外周性发绀入院。超声心动图显示严重扩张型心肌病，累及双心室，心肌壁异常肥厚且不规则。肺动脉高压为体循环以上水平，肺血管阻力升高。磁共振成像和血管造影证实了海绵状心肌持续存在的诊断。这是一种罕见的异常情况，是由于胚胎心肌持续存在，伴有通常在超声心动图中观察到的小梁网络和小梁间隙。该病例值得注意，因为双心室受累且无相关先天性心脏畸形。预后的特点是严重心血管并发症的风险很高。
