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一例Ebstein畸形患者,于体检时发现心脏杂音。心电图示完全性右束传导阻滞。右房肥大;心音图见心尖部全收缩期杂音,心尖部及剑突处低频第三心音;x线胸片心影呈梨形,右房室扩大;MME示二尖瓣与三尖瓣同时显示,三尖瓣前叶呈“帆状”增大,振幅46mm,且舒张期斜率减慢,三尖辨比二尖瓣关闭延迟0.083秒;二维扇形超声心动图示三尖瓣隔瓣在室间隔上的附着点与二尖瓣前叶的附着点相距2.4Cm,右心房明显扩大,主动脉根部水平横切面和二尖瓣口水平切面,均可见三尖瓣隔叶位于11点至12点处。复习文献,对本病的临床表现及诊断进行了讨论。
A case of Ebstein malformations found heart murmur at physical examination. ECG complete right bundle block. Right atrium hypertrophy; phonocardiogram to see apex systolic murmur, apical and xiphoid at the third heart sound; x-ray chest shadow was pear-shaped, right atrioventricular enlargement; MME showed mitral and tricuspid valve at the same time The anterior tibial plateau showed a “sail-like” shape with an amplitude of 46 mm and a slowed diastolic slope. The trisomic resolution was delayed by 0.083 second compared with mitral valve closure. A two-dimensional fan-shaped echocardiogram of the tricuspid valve Ventricular septal attachment point and the anterior mitral valve attachment point 2.4Cm away from the right atrium was significantly enlarged, the aortic root horizontal cross-section and the mitral level horizontal section, can be seen in the tricuspid valve at 11 to 12 Point. Review the literature, the clinical manifestations and diagnosis of the disease were discussed.