Maruo T, Kano S, Ichino K, Imai Y, Asakawa M, Itoh N, Tamiya E, Ikenouchi H, Hada Y
Department of Cardiology, JR Tokyo General Hospital.
J Cardiol. 1998;31 Suppl 1:131-6; discussion 137.
A 66-year-old man presented with Ebstein's anomaly associated with left ventricular dysfunction. He had been followed since 40 years of age for cardiomegaly and arrhythmia, and experienced episodes of orthopnea at the age of 64. He was referred to our hospital in April 1997 because of lower extremity edema. Physical examination revealed dilated external jugular vein, tenderness of the right hypocondorium, and lower extremity edema. Electrocardiography confirmed atrial fibrillation. Transthoracic echocardiography revealed bilateral atrial and ventricular dilation, and paradoxical septal movement. The apical four-chamber view demonstrated 15 mm apical displacement of the septal leaflet. Color Doppler echocardiography revealed moderate tricuspid regurgitation. Transesophageal echocardiography revealed low echoic and hypoplastic tricuspid valve. Left ventriculography showed diffuse hypokinesis, and the ejection fraction was 49%. The coronary artery was normal. Atrial septal defect was not detected. Diffuse fibrosis, which may be found in the hearts of patients with Ebstein's anomaly at autopsy may have been responsible for the left ventricular depressed systolic function in this patient.
一名66岁男性因埃布斯坦畸形合并左心室功能障碍前来就诊。他从40岁起就因心脏肥大和心律失常接受随访，64岁时出现端坐呼吸发作。1997年4月，他因下肢水肿被转诊至我院。体格检查发现颈外静脉扩张、右季肋部压痛和下肢水肿。心电图证实为心房颤动。经胸超声心动图显示双侧心房和心室扩张，以及室间隔矛盾运动。心尖四腔心切面显示隔叶心尖移位15毫米。彩色多普勒超声心动图显示中度三尖瓣反流。经食管超声心动图显示三尖瓣低回声且发育不全。左心室造影显示弥漫性运动减弱，射血分数为49%。冠状动脉正常。未检测到房间隔缺损。尸检时在埃布斯坦畸形患者心脏中可能发现的弥漫性纤维化可能是该患者左心室收缩功能降低的原因。
