Complete atrioventricular septal defect, Down syndrome, and surgical outcome: Risk factors

Complete atrioventricular septal defect, Down syndrome, and surgical outcome: Risk factors
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DOI:
10.1016/s0003-4975(02)04026-2
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发表时间:
2003-02-01
影响因子:
4.6
通讯作者:
Shinebourne, EA
Shinebourne, EA
中科院分区:
医学2区
文献类型:
--
作者:
Al-Hay, AA;MacNeill, SJ;Shinebourne, EA

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背景本研究旨在评估手术结果,并确定住院死亡率和完全性房室间隔缺损修补术后再次手术的危险因素。在1986年1月至1998年12月期间,共有147名儿童接受了修复。其中,106人患有唐氏综合征,37%的人染色体正常,4人患有其他综合征; 108人接受了一期修复术,19人以前有肺动脉束带,20人患有法洛四联症。初次修复时的中位体重为4.5 kg。88%的患者使用了双贴片技术。30天死亡率为15%(70%置信区间[CI]为12%-19%)。发现双孔房室瓣是一个显著的风险因素(p = 0.002),11例患者中有6例死亡。如果排除双孔房室瓣患者,死亡率福尔斯降至12%(70% CI 9%-15%)。唐氏综合征和染色体正常儿童的死亡率没有差异,但后者更常需要再次手术。染色体正常的儿童常伴有发育不良的共同房室瓣(24%比Down儿童的3%,p < 0.001)。在包括两个变量的多变量考克斯模型中,房室瓣发育不良是再次手术的重要风险因素。在控制了发育不良房室瓣的存在后,唐氏综合征仍具有显著的保护作用,但可信区间的上限接近1。双孔房室瓣的存在是不可预见的死亡风险因素。(C)2003年由胸外科医师协会发布。
Background. This study was conducted to evaluate surgical outcome and to identify risk factors for hospital mortality and reoperation after repair of complete atrioventricular septal defect.Methods. A total of 147 consecutive children underwent repair between January 1986 and December 1998. Of those, 106 had Down syndrome, 37% had normal chromosomes, and 4 had other syndromes; 108 underwent primary repair, 19 had prior pulmonary artery banding, and 20 had additional tetralogy of Fallot. The median weight at primary repair was 4.5 kg. A two-patch technique was used in 88%.Results. The 30-day mortality was 15% (70% confidence interval [CI] 12% to 19%). A double orifice atrioventricular valve was found to be a significant risk factor (p = 0.002), with 6 of 11 patients dying. If double orifice atrioventricular valve patients are excluded, the mortality rate falls to 12% (70% CI 9% to 15%). No difference in mortality was found between Down syndrome and chromosomally normal children but the latter more commonly required reoperation. Chromosomally normal children frequently have a dysplastic common atrioventricular valve (24% versus 3% in Down children, p < 0.001). In a multivariate Cox model including both variables, the presence of a dysplastic atrioventricular valve was a significant risk factor for reoperation. After controlling for the presence of a dysplastic atrioventricular valve, Down syndrome retained a significant protective effect but the upper limit of the confidence interval was close to 1.Conclusions. The presence of a double orifice atrioventricular valve emerged as an unforeseen risk factor for death. (C) 2003 by The Society of Thoracic Surgeons.