Intensive cardiac management in patients with trisomy 13 or trisomy 18
Intensive cardiac management in patients with trisomy 13 or trisomy 18
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DOI：
10.1002/ajmg.a.32311
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发表时间：
2008-06-01
影响因子：
2
通讯作者：
Kawakami, Tadashi
中科院分区：
文献类型：
--
作者：
Kaneko, Yukihiro;Kobayashi, Jotaro;Kawakami, Tadashi
强化心脏管理，如导管通畅的药物干预（吲哚美辛和/或甲芬那酸用于闭合，前列腺素E1用于维持）和姑息或矫正手术是先天性心脏病的标准治疗。然而，它是否会成为13三体或18三体综合征儿童的治疗选择是有争议的，因为这些三体患者的生存疗效尚未得到评估。我们回顾性分析了2000年至2005年期间在新生儿病房出生后6小时内收治的31例13三体或18三体新生儿。机构管理政策在三个不同时期有所不同。在第一阶段，药物导管介入和心脏手术均被暂停。在第二种情况下，药物导管干预被作为一种选择，但心脏手术被拒绝。在第三阶段，这两种战略都可用。第一、第二和第三阶段的13、9和9例新生儿的中位生存时间分别为7、24和243天。单因素和多因素分析证实，在第三阶段的患者生存时间明显长于其他。在本系列研究中，由导管通畅性药物干预和心脏手术组成的强化心脏管理被证明可提高13三体或18三体患者的生存率。因此，我们认为这种方法是与这些三体相关的心脏病变的治疗选择。这些数据有助于临床医生和家庭考虑这些三体患者的最佳治疗。(c)2008 Wiley-Liss，Inc.
Intensive cardiac management Such as pharmacological intervention for ductal patency (indomethacin and/or mefenamic acid for closure and prostaglandin E1 for maintenance) and palliative or corrective surgery is a standard treatment for congenital heart defects. However, whether it would be a treatment option for children with trisomy 13 or trisomy 18 syndrome is controversial because the efficacy on survival in patients with these trisomies has not been evaluated. We retrospectively reviewed 31 consecutive neonates with trisomy 13 or trisomy 18 admitted to our neonatal ward within 6 hr of birth between 2000 and 2005. The institutional management policies differed during three distinct periods. In the first period, both pharmacological ductal intervention and cardiac surgery were withheld. in the second, pharmacological ductal intervention was offered as an option, but cardiac surgery was withheld. Both strategies were available during the third period. The median Survival times of 13, 9, and 9 neonates from the first, second, and third periods were 7, 24, and 243 days, respectively. Univariate and multivariate analyses confirmed that the patients in the third period survived significantly longer than the others. Intensive cardiac management consisting of pharmacological intervention for ductal patency and cardiac surgery was demonstrated to improve survival in patients with trisomy 13 or trisomy 18 in this series. Therefore, we suggest that this approach is a treatment option for cardiac lesions associated with these trisomies. These data are helpful for clinicians and families to consider in the optimal treatment of patients with these trisomies. (c) 2008 Wiley-Liss, Inc.