Surgical outcome of hypoplastic left heart syndrome with intact atrial septum.
Surgical outcome of hypoplastic left heart syndrome with intact atrial septum.
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DOI：
10.1177/0218492315606581
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发表时间：
2015-11-01
影响因子：
0.7
通讯作者：
Sano, Shunji
中科院分区：
文献类型：
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作者：
Arai, Sadahiko;Fujii, Yasuhiro;Sano, Shunji
背景技术背景：尽管在诺伍德手术前引入了产前诊断，但房间隔完整的左心发育不良综合征的手术结局仍然很差。混合操作包括双侧肺动脉束带和球囊房间隔切除术的治疗策略之一，以潜在地改善这一患者population.METHODS的长期结果是未知的：6例连续的左心发育不良综合征与完整的房间隔，谁接受了混合操作之间2006年10月和2014年7月，进行了回顾性分析。结果：3例患者死于混合手术后，由于败血症，脑出血，心力衰竭。3例行改良诺伍德手术。其中一人在等待双向格伦手术时因严重缺氧死亡。其他人进行了双向Glenn手术和随后的Fontan完成，但死于肺功能障碍和突然咯血.CONCLUSIONS：混合动力手术与完整的房间隔左心发育不良综合征可能改善了新生儿的结局和Fontan完成率在这一极高的风险组的患者，但长期的结果仍然令人沮丧。考虑到与肺部并发症相关的晚期死亡率，积极的胎儿干预以建立非限制性房间隔沟通，促进肺血管的正常发育可能是改善长期结局的最后手段。
BACKGROUND: The surgical outcomes of hypoplastic left heart syndrome with intact atrial septum remains very poor in spite of the introduction of prenatal diagnosis before the Norwood operation. The hybrid operation consisting of bilateral pulmonary artery banding and balloon atrioseptectomy is one of the treatment strategies to potentially improve this patient population, however, the long-term outcomes are unknown.METHODS: Six consecutive patients with hypoplastic left heart syndrome with intact atrial septum, who underwent the hybrid operation between October 2006 and July 2014, were retrospectively reviewed. Hypoplastic left heart syndrome with highly restrictive atrial communication was excluded.RESULTS: Three patients died after the hybrid operation, due to sepsis, cerebral bleeding, and heart failure. Three patients underwent the modified Norwood operation. One of these died due to severe hypoxia while awaiting the bidirectional Glenn operation. The others underwent a bidirectional Glenn operation and subsequent Fontan completion but died due to lung dysfunction and sudden hemoptysis.CONCLUSIONS: The hybrid operation for hypoplastic left heart syndrome with intact atrial septum may have improved the neonatal outcome and Fontan completion rate in this extremely high-risk group of patients, but the long-term outcome remains dismal. Considering the late mortality related to pulmonary complications, aggressive fetal intervention to create a nonrestrictive atrial septal communication to promote normal development of the pulmonary vessels may be the last resort to improve the long-term outcome.