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患儿,女,4个月。生后即现青紫。曾多次无明显诱因青紫加重,每次均需吸氧方能缓解。检查:神志不清,全身明显青紫,口唇、指甲尤甚。诊断为先天性心脏病,经抢救无效死亡。尸体解剖所见:心脏大小、形态无特殊。肺动脉缺如,2条肺静脉与心房左上方相通,主动脉开口于心室右前上方,向上移行约2cm 形成主动脉弓,弓上自右向左发出头臂干、左颈总动脉和左锁骨下动脉。自头臂干发出一枝通向肺,肺发育正常。上、下腔静脉分别汇入心房的左上方和右下方。心房壁厚0.2～0.3cm。心室壁厚0.6～1.3cm。在心房后壁上,下腔静脉入口间有一长约2.5cm、宽0.2cm之粘膜皱壁,
Children, women, 4 months. Health is purple after birth. Many times there was no obvious incentive purple bruising, each need oxygen can ease. Check: unconscious, obviously purple body, lips, nails especially. Diagnosis of congenital heart disease, died of rescue invalid. Autopsy seen: heart size, shape no special. The pulmonary artery is absent. Two pulmonary veins communicate with the upper left atrium. The aorta is opened at the upper right front of the ventricle. The aorta is displaced upward by about 2 cm. The brachial trunk, the left common carotid artery and the left subclavian artery emerge from right to left. A branch from the trunk to the lungs, normal lung development. On the inferior vena cava were imported into the upper left and right atrium. Atrial wall thickness 0.2 ~ 0.3cm. Ventricular wall thickness 0.6 ~ 1.3cm. In the atrium of the posterior wall, the entrance of the inferior vena cava has a length of about 2.5cm, 0.2cm wide mucosal folds,