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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、心导管及选择性心血管造影术是常用的有效确诊手段
Objective: To investigate the clinical and autopsy characteristics of complete pulmonary venous drainage (TAPVC) in children. Methods: The clinical manifestations, electrocardiogram, chest radiograph, echocardiography, cardiac catheterization, surgery and autopsy data of 23 TAPVC children admitted to our hospital from 1980 to 2000 were retrospectively reviewed. Results: Twenty - three cases of TAPVC had cardiac type in 17 cases (74%), intracardiac type in 6 cases (26%), associated with patent ductus arteriosus (PDA) in 6 cases, atrial septal defect ASD) in 11 cases, patent foramen ovale in 5 cases, ventricular septal defect (VSD) in 2 cases and mitral dysplasia in 1 case. Among the 3 cases, 5 cases were discharged after conservative treatment and 4 cases were given up their treatment. 3 cases were cured and discharged, 2 cases were discharged and 1 case died; 12 cases died (52%), all of whom were less than 6 Month, of which 10 cases of autopsy age of 24 days to 4 months, were found pulmonary obstruction. Conclusion: TAPVC is not uncommon in congenital heart disease, often accompanied by some clinical syndromes and other cardiac malformations, most of which are symptomatic in infants and young children. In this group of cases, 52% died within 6 months, especially pulmonary vascular Obstructive lesions, more than a few days or 3 to 4 months of death, easily misdiagnosed as neonatal pneumonia, autopsy was confirmed. Echocardiography, cardiac catheterization and selective cardiovascular angiography are commonly used tools for effective diagnosis