Radical surgery for a ventricular septal defect associated with trisomy 18
Radical surgery for a ventricular septal defect associated with trisomy 18
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18 三体相关室间隔缺损的根治性手术
DOI：
10.1007/s11748-009-0431-3
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发表时间：
2010
影响因子：
1.2
通讯作者：
K. Tsuchiya
中科院分区：
文献类型：
--
作者：
Jotaro Kobayashi;Y. Kaneko;Yuusuke Yamamoto;H. Yoda;K. Tsuchiya
目的充血性心力衰竭是18三体患者早期死亡的主要原因之一。室间隔缺损（VSD）是18三体患者最常见的心脏缺陷，VSD的闭合可以延长患者的寿命。这些患者的形态学特征，如胸廓畸形、显著的右心室肥大和发育不良的三尖瓣可能使VSD闭合复杂化。我们报告我们的初步经验，VSD关闭的患者与三体18和估计的可行性的外科手术procedure.MethodsBetween 2005年6月和2007年9月，5名女性患者与三体18和VSD进行了根治性手术。他们中的4人接受了姑息性手术，需要肺动脉banding.ResultsAll患者存活手术。手术期间平均心脏骤停时间为74 ± 22分钟。手术后30天内无早期死亡。1例脑积水患者因术后颅内高压在医院死亡，之前接受过脑室腹腔分流术。5名患者中有4人出院。在4名出院患者中，1名接受初次根治手术的女孩死于肺炎。另一名患者死于心肺骤停。在这项研究中，患者的平均生存期为815 ± 389天，在数据acquisition.Conclusionclosure VSD的患者与三体18被发现是可行的，并与延长生存期。
PurposeCongestive heart failure is one of the major causes of early death of patients with trisomy 18. Ventricular septal defect (VSD) is the most common heart defect in patients with trisomy 18, and closure of the VSD may elongate the lifespan of the patient. Morphological characteristics of these patients, such as thoracic deformity, prominent right ventricular hypertrophy, and dysplastic tricuspid valve may complicate closure of the VSD. We report our initial experience of VSD closure in patients with trisomy 18 and estimate the feasibility of the surgical procedure.MethodsBetween June 2005 and September 2007, five female patients with trisomy 18 and VSD underwent radical operations. Four of them had undergone previous palliative surgery entailing pulmonary artery banding.ResultsAll patients survived surgery. The average cardiac arrest time during surgery was 74 ± 22 min. No early death occurred within 30 days of surgery. One patient with hydrocephalus treated by a previous ventriculoperitoneal shunt died in hospital owing to postoperative intracranial hypertension. Four of five patients were discharged from the hospital. Of the four discharged patients, a girl who underwent primary radical operation died of pneumonia. Another patient died of sudden cardiopulmonary arrest. The average survival of the patients in this study was 815 ± 389 days at data acquisition, with two patients still alive.ConclusionClosure of VSDs in patients with trisomy 18 was found feasible and was associated with extended survival.