Drude L, Klein H H, Maisch B
Abteilung Innere Medizin-Kardiologie, Philipps-Universität Marburg.
Herz. 1994 Jun;19(3):171-5.
We describe a 63-year old female patient presenting with increasing dyspnea on exertion. The chest X-ray was suggestive but not compelling for Ebstein's anomaly. Although Ebstein's anomaly is most often discovered first in childhood, rare cases, such as ours, are described in late adulthood, however. Obesity may be one reason why it was not possible in our patient to detect the characteristic features in transthoracic echocardiography. The diagnosis of Ebstein's anomaly could be established only by transesophageal echocardiography: There the right atrium was grossly enlarged due to the distal insertion of the septal tricuspid valve leaflet. Colour flow echocardiography clearly demonstrated severe tricuspid valve incompetence. No additional congenital or acquired cardiac disorder could be detected. Cardiac catheterization confirmed the echocardiographical findings, whereas magnetic resonance tomography did not show the abnormal insertion of the tricuspid valve leaflet. If Ebstein's anomaly is suspected and cannot be ascertained by transthoracic echocardiography, transesophageal echocardiography can be employed to definitely diagnose this fairly rare form of congenital heart disease in an adult population.
我们描述了一位63岁的女性患者,她表现为运动时呼吸困难逐渐加重。胸部X线检查提示存在埃布斯坦畸形,但证据并不确凿。虽然埃布斯坦畸形最常在儿童期首次被发现,但像我们这样在成年晚期出现的罕见病例也有报道。肥胖可能是我们的患者经胸超声心动图无法检测到特征性表现的原因之一。埃布斯坦畸形的诊断只能通过经食管超声心动图来确立:在经食管超声心动图检查中,由于三尖瓣隔叶远端附着,右心房明显增大。彩色血流超声心动图清晰显示严重的三尖瓣反流。未检测到其他先天性或后天性心脏疾病。心导管检查证实了超声心动图的结果,而磁共振成像未显示三尖瓣叶的异常附着。如果怀疑存在埃布斯坦畸形且经胸超声心动图无法确诊,可采用经食管超声心动图来明确诊断这种在成人中相当罕见的先天性心脏病。