Surgical management of congenital heart defects associated with heterotaxy syndrome
Surgical management of congenital heart defects associated with heterotaxy syndrome
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DOI：
10.1016/j.ejcts.2010.02.044
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发表时间：
2010-12-01
影响因子：
3.4
通讯作者：
Belli, Emre
中科院分区：
文献类型：
--
作者：
Serraf, Alain;Bensari, Nawel;Belli, Emre
目的：异位综合征（HS）通常与复杂的先天性心脏缺陷相关，尽管最近外科手术取得了进展，但发病率和死亡率仍然很高。只有很少的报告涉及整个手术人群。方法：1989 年至 2008 年间，139 名 HS 患者参加了手术修复计划。 51 名患者适合进行单心室心脏修复 (UVR)，88 名患者适合进行双心室修复 (BVR)。在追踪 UVR 的人中，有 2 人转为 BVR，11 人从 BVR 转为 UVR。首次手术的中位年龄为 4.4 个月（范围：3 天至 43 岁），其中 34 例为新生儿。平均每位患者手术次数为 1.99 例。37 例患者进行了初次 BVR。22 例患者需要再次手术，其中 BVR 后 15 例，UVR 后 7 例（p > 0.05）。结果：总死亡率为 20.8%，第一次手术后为 7.2%，第二次后为 6.6%，第三次后为 11.5%，双心室组中单心室生理学患者的总体死亡率为 25.5% 和 18.2%（p < 0.05），在 UVR 组中，BVR 组中的死亡率为 18% 和 15.6%（p = NS）。中位随访时间为 127 个月（范围：1 个月至 19 年）。考虑到心室解剖情况，单心室组的 15 年生存率为 69%，双心室组的 15 年生存率为 74.2%（p > 0.05）。 UVR 为 85.1%，BVR 为 77% (p = NS) 对于姑息治疗组，15 年时仅为 15% (p = NS)。
Objective: Heterotaxy syndrome (HS) is generally associated with complex congenital cardiac defects and has a high morbidity and mortality despite recent surgical progress. Only few reports deal with an overall surgical population. Methods: Between 1989 and 2008, 139 patients with HS entered a programme of surgical repair. Fifty-one patients were suitable for univentricular heart repair (UVR) and 88 for biventricular repair (BVR). Among those tracked for UVR, two were switched to BVR and 11 from BVR to UVR. Median age at first surgery was 4.4 months (range: 3 days to 43 years] of whom 34 were neonates. The mean number of surgical procedure per patient was 1.99. Primary BVR was performed in 37 patients. Re-operation was required in 22 patients, 15 after BVR and seven after UVR (p > 0.05). Results: The overall mortality was 20.8%. It was 7.2% after the first surgery, 6.6% after the second and 11.5% after the third. The overall mortality in patients with univentricular physiology was 25.5% and 18.2% in the biventricular group (p < 0.05). According to the surgical track, in the UVR group, mortality was 18% and 15.6% in the BVR group (p = NS). This rate was 40% in patients with long-lasting palliation (p < 0.05 vs both other groups). Median follow-up was 127 months (range: 1 month to 19 years). The overall survival rate at 15 years was 70.6%. When considering ventricular anatomy, survival rates at 15 years were 69% for the univentricular group and 74.2% for the biventricular group (p > 0.05). According to the type of surgical approach, at 15 years they were 85.1% for UVR and 77% for BVR (p = NS). For the palliation group, it was 15% only at 15 years (p