目前治疗低发性左心综合征和相关单心室病变
Richard G Ohye1, Dietmar Schranz2, Yves D'Udekem2
1From University of Michigan C. S. Mott Children's Hospital, Ann Arbor (R.G.O.); Pediatric Heart Center, Justus Liebig University Giessen, Germany (D.S.); and Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia (Y.D'U.). ohye@umich.edu.
Circulation
|October 26, 2016
概括
通过手术缓解左心缩症候群的进展, 显著提高了生存率. 诺伍德手术和混合方法的创新为单一右心室病变提供了更好的结果.
科学领域:
- 儿童心脏病学
- 遗传性心脏手术
- 新生儿重症监护
背景情况:
- 低发性左心综合征 (HLHS) 和相关的单一右心室病变曾经是普遍致命的.
- 在这些复杂的先天性心脏缺陷的三阶段手术缓解方面取得了显著进展.
- 虽然第二阶段和第三阶段的风险已经降低,但诺伍德程序的第一阶段风险仍然很高.
研究的目的:
- 审查HLHS和单一右心室病变的三阶段手术缓解方面的创新.
- 突出诺伍德程序的改进和混合方法的引入.
- 强调人们越来越需要了解Fontan患者的长期结果.
主要方法:
- 对单心室息手术技术的进展进行回顾.
- 对单心室重建试验的讨论,比较诺伍德手术的突变类型.
- 综述混合程序作为传统诺伍德方法的替代方案.
主要成果:
- 经验丰富的中心报告说,尽管存在固有的风险,诺伍德手术的生存率高于90%.
- 单心室重建试验提供了长期随访数据.
- 混合手术和改进的诺伍德技术旨在改善患者的短期和长期结果.
结论:
- 患有HLHS和单一右心室病变的患者的生存率大大提高.
- 传统的分阶段治疗和混合疗法都提高了青春期和成年期的生存率.
- 进一步的研究对于了解Fontan幸存者的长期疾病负担,神经发育,心理社会发展和生活质量至关重要.
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