Ebstein's Anomaly

V. Delyagin, N. Doctorova, I.Ye. Belokrinitskaya
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引用次数: 1

Abstract

Ebstein’s anomaly is a rare congenital malformation characterized by apical displacement of the right atrioventricular ring with an anomaly of the development of the tricuspid valve and the frequent presence of other heart defects. According to our data, this defect was diagnosed in 2 cases in more than 6 000 sectional studies: in an adult and a child. According to the echocardiography rooms of non-specialized multidisciplinary children's hospitals and children's polyclinics, the defect is detected with a frequency of 1:24.000–1:32.000 studies. Genetic and environmental factors (viral infections, benzodiazepines, lithium) are indicated as probable causes. Clinical signs are cyanosis, shortness of breath, heart failure, cardiac arrhythmias. Morphologically there are abnormalities of the tricuspid valve, papillary muscles and chordal apparatus, right ventricular myocardial hypertrophy, thinning of the walls of the right atrium and atrialized part of the right ventricle. Decisive for the diagnosis is ultrasound. Displacement of the right atrioventricular ring is revealed. The degree of bias determines the severity of the defect. The amplitude of the tricuspid valve movement is increased. Its valve closure occurs later than the mitral. A 40 ms difference between the tricuspid and mitral valve closure points is a diagnostically important sign of Ebstein’s anomaly. The tactics of a doctor are determined by the clinical picture
原发异常
Ebstein畸形是一种罕见的先天性畸形,其特征是右房室环顶端移位,伴有三尖瓣发育异常,并经常出现其他心脏缺陷。根据我们的资料,在超过6 000个断面研究中诊断出2例这种缺陷:一名成人和一名儿童。根据非专业多学科儿童医院和儿童综合诊所的超声心动图室,该缺陷的检测频率为1:24 000 - 1:32 000。遗传和环境因素(病毒感染、苯二氮卓类药物、锂)被认为是可能的原因。临床表现为发绀、呼吸短促、心力衰竭、心律失常。形态学上表现为三尖瓣、乳头肌和索索器官异常,右心室心肌肥厚,右心房壁变薄,右心室部分心房化。超声是诊断的决定性因素。显示右房室环移位。偏差的程度决定了缺陷的严重程度。三尖瓣运动幅度增大。它的瓣膜关闭晚于二尖瓣。三尖瓣和二尖瓣关闭点之间40毫秒的差异是Ebstein异常的重要诊断标志。医生的策略是由临床情况决定的
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