Esteban I, Cabrera A, Pastor E, Irurita M, Aguirre J M, Cea J M, Eizaguirre J
An Esp Pediatr. 1983 Jun;18(6):457-66.
We describe 13 cases of Ebstein's anomaly of the tricuspid valve. Seventy percent of there cases were detected at one month of age. Ten patients died and 9 presented associated cardiac anomalies. Signs of right heart failure were the clinical date that made possible the diagnosis of the disease in the most severe cases in the neonatal period. Only two patients had alteration of the cardiac rhythm: One had tachycardia that ended with ventricular fibrillation and the other had a 2:1 heart block. Echocardiography was very useful to establish the diagnosis: one hundred percent of the patients showed a delay in closing of the tricuspid in relation to the mitral valve, with a range of 0,03 to 0,06 seconds. Angiocardiographic studies revealed a tricuspid valve displacement in 8 cases, and double contour image in 6. The septal and posterior leaflets were hypoplasic in the 10 anatomic cases and in 80 percent of them it was possible to differentiate the cords from the papillary muscles.
我们描述了13例三尖瓣埃布斯坦畸形病例。其中70%的病例在1月龄时被发现。10例患者死亡，9例伴有心脏相关异常。右心衰竭体征是新生儿期最严重病例中得以诊断该病的临床依据。仅有2例患者出现心律改变：1例为心动过速，最终发展为心室颤动，另1例为2:1房室传导阻滞。超声心动图对确诊非常有用：100%的患者显示三尖瓣相对于二尖瓣关闭延迟，延迟范围为0.03至0.06秒。心血管造影研究显示8例存在三尖瓣移位，6例出现双重轮廓影像。在10例解剖病例中，隔叶和后叶发育不全，其中80%的病例能够区分腱索和乳头肌。
