完全性肺静脉异位引流患儿临床及尸检分析

Clinical manifeststion of total anomalous pulmonary venous connection in children

目的 :探讨儿童完全性肺静脉异位引流 (TAPVC)的临床及尸检特征性改变。方法 :回顾性总结我院 1980～ 2 0 0 0年间收治的 2 3例TAPVC患儿的临床表现、心电图、胸片、心脏超声、心导管检查、手术及尸检资料。结果 :2 3例TAPVC患儿中属心上型 17例 (占 74 % ) ,心内型 6例 (占 2 6 % ) ,其中伴有动脉导管未闭 (PDA) 6例 ,房间隔缺损 (ASD) 11例 ,卵圆孔未闭 5例 ,室间隔缺损 (VSD) 2例 ,二尖瓣发育不良 1例。 2 3例患儿中 5例经内科保守治疗后好转出院 ,4例放弃治疗 ;3例施行纠治术 ,2例痊愈出院 ,1例死亡 ;死亡 12例 (5 2 % ) ,年龄均 <6个月 ,其中 10例尸检年龄为 2 4d～ 4个月 ,均发现有肺静脉梗阻性病变。结论 :TAPVC在先心病中并不少见 ,常伴发一些临床综合征及其他心脏畸形 ,大多在婴幼儿即有症状 ,本组病例中 5 2 %死于 6个月以内 ,特别是患肺血管梗阻性病变者 ,多于数日或 3～ 4个月内死亡 ,极易误诊为新生儿肺炎 ,尸检中才得以证实。心脏超声。

Objective:To analyse the clinical characteristics and autopsy findings in patients with total anomalous pulmonary venous connection(TAPVC). Methods and Results:From 1980 to 2000, twenty three patients with TAPVC were admitted, seventeen with supracardiac (74%), six with cardiac type (26%). It was associated with patent ductus arteriosus in six patients, atrial septal defect in eleven, patent foramen ovalie in five, ventricular septal defect in two and hypoplasia of mitral valve in one. Three cases were operated and one died. There were autopsy findings in twelve cases died within six months. They were complicated obstructive pulmonary venous lesion. Conclusion:TAPVC is not rare, symptoms usually emerged in early infancy. Mortality is high when complicated with obstruction in venous return. Respiratory symptoms were the major manifestation, and were misdiagnosed as pneumonia. Echocardiogram, catheterization and angiography can make the diagnosis.