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1 病例介绍 患者 女,年龄4 8/(12)岁。生后心脏有杂音,后紫绀逐渐加重。喜抱位,喜蹲踞,活动后紫绀加重。查体发现:心率102次/分,血压10/6 kPa(75/45 mmHg),口唇发绀。胸骨左缘3、4肋间可闻Ⅲ/6收缩期杂音,可及震颤,肺动脉第二音未闻及。肝左肋下0.5cm,重度杵状指趾。胸部平片示:心胸比例:0.54。靴型心、肺血少。心电图示:窦性心律,电轴左偏,右室肥厚伴劳损。多普勒超声心动图示:两大动脉发自右室,主动脉与肺动脉平行。两条大动脉与房室瓣之间为肌性圆锥组织连接。室缺近于肺动脉瓣下,房缺1.5cm。室水平左向右分流,房水平右向左分流为主。多普勒超声诊断:右室双出口,肺动脉狭窄,室缺近肺动脉瓣下,房缺。心血管造影检查,主动脉、肺动脉起自右室,右室为双肌性流出道,主动脉与二尖瓣失纤维连接。肺动脉下流出道严重狭
1 Case Description Female patient, age 4 8 / (12) years old. After living a murmur heart, cyanosis gradually increased. Hi-bit, hi squat, cyanosis worsened after the event. Physical examination found: heart rate 102 beats / min, blood pressure 10/6 kPa (75/45 mmHg), lips cyanosis. Sternal left border 3,4 intercostal can smell Ⅲ / 6 systolic murmur, and tremor, the second pulmonary sound did not smell. Liver left rib 0.5cm, severe clubbing toes. Chest flat film shows: mind chest ratio: 0.54. Boots heart, less lung blood. ECG shows: sinus rhythm, left axis deviation, hypertrophy with strain of right ventricle. Doppler echocardiography showed: two major artery from the right ventricle, aorta and pulmonary artery parallel. Two large arteries and atrioventricular valve for the connection between the muscular cone. Ventricular proximity near the pulmonary valve, atrial septal 1.5cm. Room level left to right shunt, room level right to left shunt. Doppler ultrasound diagnosis: double outlet right ventricle, pulmonary stenosis, near empty pulmonary valve, atrial septal defect. Cardiac angiography, aorta, pulmonary artery from the right ventricle, right ventricle for the dual muscular outflow tract, aortic and mitral valve loss of fiber connection. Pulmonary artery outflow tract serious narrow