系统性心室功能的连续变化和心房门功能在患者的失败Fontan的功能
Muneaki Matsubara1,2,3, Vincent Dahmen1,2,3, Paula Gaebert1,2,3
1Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany.
概括
失败的Fontan显示出不同的模式:在蛋白质丧失肠病/塑性支气管炎病例中保持了心室功能,但在心力衰竭病例中出现了渐进式功能障碍. 这澄清了Fontan故障的时间和机制.
科学领域:
- 心脏病学 心脏病学
- 儿童心脏病学 儿童心脏病学
- 遗传性心脏病是一种先天性心脏病.
背景情况:
- 芬坦手术是一种治疗单心室先天性心脏缺陷的缓解手术.
- 丰坦息后的长期结果是可变的,其中一个子集发展为"失败的丰坦"综合征.
- 了解心室和心房功能的演变对于管理Fontan患者至关重要.
研究的目的:
- 在全腔肺连接后的患者中,研究心室和心房膜功能的纵向变化.
- 阐明与不同呈现失败的Fontan.相关的独特机制和时间.
- 为了区分失败Fontan相关的蛋白质损失肠病/塑性支气管炎与渐进性心力衰竭的功能概况.
主要方法:
- 从650名接受了全腔肺连接 (1994-2023) 的患者的回顾性心声图数据分析.
- 长度评估心室功能 (静脉和腹) 和心室回.
- 根据Fontan失败的发展及其相关的临床表现,患者的分层.
主要成果:
- 在平均6.2年的随访期间,Fontan失败发生在12%的患者中.
- 患有蛋白质损失性肠病或塑性支气管炎的患者 (n=37) 显示早期的Fontan衰竭,但保持了心室功能和心房膜能力.
- 患有心力衰竭进展的患者 (n=41) 经历了后期发病的Fontan衰竭,随着渐进的心室功能障碍和增加心房回.
结论:
- 失败的Fontan表现出异质的病理生理路径.
- 保持心室功能的特征是失败的Fontan与失去蛋白质的肠道病变/塑性支气管炎相关.
- 渐进的心室功能障碍和心室膜反是失败的标志,Fontan进展到心力衰竭.
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