Outcome of Glenn Anastomosis for Heterotaxy Syndrome with Single Ventricle
Outcome of Glenn Anastomosis for Heterotaxy Syndrome with Single Ventricle
复制标题
格伦吻合术治疗单心室异位综合征的疗效
DOI：
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发表时间：
2006
影响因子：
0.7
通讯作者：
C. Fraser
中科院分区：
文献类型：
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作者：
M. Koudieh;E. McKenzie;C. Fraser
对1996年1月至2001年5月期间接受Glenn吻合术的157例患者进行了回顾性研究。其中，33例患有内脏异位综合征：20例男性和13例女性，平均年龄为1.26 ± 2.8岁。右心房异构25例，左心房异构5例。24例为共同房室瓣，18例为双侧上级腔静脉，18例为肺静脉异位回流。8例肺静脉回流异常患者行修补术，11例行肺动脉扩张术。与124例因单心室而接受Glenn手术且无异位的患者相比，异位组的机械通气、重症监护室停留时间和正性肌力支持时间明显更长，死亡率也更高。单心室异位综合征仍有较高的发病率和死亡率。严重房室瓣反流的患者存在早期死亡的风险。不可能所有患者都有完全的Fontan循环，Glenn吻合术可能是最终的姑息治疗。
A retrospective study was undertaken of 157 patients who underwent a Glenn anastomosis between January 1996 and May 2001. Of these, 33 had heterotaxy syndrome: 20 males and 13 females, with a mean age of 1.26 ± 2.8 years. Twenty-five had right atrial isomerism and 5 had left isomerism. A common atrioventricular valve was found in 24 patients, 18 had bilateral superior venae cavae, and 18 had anomalous pulmonary venous return. Repair was carried out in 8 patients with anomalous pulmonary venous return, and pulmonary artery augmentation was performed in 11. Compared to the 124 patients who had a Glenn operation for single ventricle without heterotaxy, there were significantly longer durations of mechanical ventilation, intensive care unit stay, and inotropic support, as well as higher mortality in the heterotaxy group. Heterotaxy syndrome with single ventricle still has a high rate of morbidity and mortality. Patients with severe atrioventricular valve regurgitation are at risk of early death. Complete Fontan circulation may not be possible in all patients, and Glenn anastomosis may be their final palliation.