Barazer P Y, Losay J, Lacour-Gayet F, Planché C, Binet J P
Centre chirurgical Marie-Lannelongue, Le Plessis-Robinson.
Arch Mal Coeur Vaiss. 1989 May;82(5):707-11.
Between 1970 and 1985, 78 children with complete atrioventricular canal were operated upon before the age of one year at the Marie-Lannelongue Surgical Centre. Forty-seven survivors were followed up for periods of 3 months to 13 years after the initial operation; 2 were lost of following. Among the 13 children who survived banding, 1 died spontaneously, 1 had Blalock-Taussig anastomosis and 11 underwent full repair with 5 deaths; out of 6 survivors, 4 are doing well without any treatment, 1 was reoperated upon twice and died during the second operation, and 1 was lost sight of; the survival rates at 5 and 10 years are 46 +/- 23 p. 100 and 29 +/- 23 p. 100 respectively. Among the 34 children who survived an initial complete repair, 2 were reoperated upon 3 months after the repair, with 1 death, 7 subsequently died of infection (6 had trisomy 21); out of 26 survivors followed up for 3 years and 3 months on average after the complete repair (2 to 13 years), 18 show an excellent result and 3 a mediocre result; the survival rates at 5 and 10 years are 45 +/- 9 p. 100 and 30 +/- 27 p. 100 respectively. Whether the patients initially had banding followed by complete repair or complete repair from the start, the probability of survival at 5 and 10 years was the same. The presence of trisomy 21 is an unfavourable prognostic factor at medium-term.(ABSTRACT TRUNCATED AT 250 WORDS)
1970年至1985年间，78例完全性房室通道患儿在一岁前于玛丽 - 拉讷隆格外科中心接受了手术。47名幸存者在初次手术后接受了3个月至13年的随访；2名失访。在接受束带术的13名存活儿童中，1名自然死亡，1名接受了布劳洛克 - 陶西格吻合术，11名接受了根治性修复，其中5例死亡；6名幸存者中，4名未经任何治疗情况良好，1名接受了两次再次手术并在第二次手术时死亡，1名失访；5年和10年生存率分别为46±23‰和29±23‰。在初次根治性修复后存活的34名儿童中，2名在修复后3个月接受了再次手术，1例死亡，7例随后死于感染（6例患有21三体综合征）；在初次根治性修复后平均随访3年零3个月（2至13年）的26名幸存者中，18名效果极佳，3名效果中等；5年和10年生存率分别为45±9‰和30±27‰。无论患者最初是先进行束带术再行根治性修复还是一开始就进行根治性修复，5年和10年的生存概率相同。21三体综合征的存在是中期预后的不利因素。（摘要截于250字）
