芬坦相关肝病:查,治疗和移植方面的考虑
Juliet Emamaullee1,2, Ali N Zaidi3,4, Thomas Schiano5
1Liver Transplant Center, Children's Hospital-Los Angeles, CA (J.E.).
在Fontan手术中幸存的成年人面临肝脏并发症,包括纤维化和肝硬化. 本综述侧重于这些患者与Fontan相关的肝病和移植方面的考虑.
科学领域:
- 心脏病学
- 肝病学
- 移植医学
背景情况:
- 由于手术的进步, 患有无子内生心脏病的儿童如今活到成年.
- 据估计,Fontan手术对7万名患者进行,导致大多数患者患有Fontan相关的肝病 (FALD).
- 患有FALD可能会发展到晚期, 需要心肝移植.
研究的目的:
- 审查有关Fontan相关肝病的文献.
- 专注于FALD的诊断和监测策略.
- 在Fontan患者中研究心脏移植与联合心脏肝移植的考虑因素.
主要方法:
- 对Fontan循环和肝脏疾病的文献综述.
- 对显示FALD进展的监测活检数据的分析.
- 对接受移植的患者的结果的检查.
主要成果:
- 几乎所有的Fontan幸存者在青春期发展出临床无声肝纤维化.
- 包括肝硬化和肝细胞癌在内的晚期FALD病例越来越多.
- 结合心肝移植在末期FALD中变得越来越常见.
结论:
- 缺乏管理FALD和指导移植决策的共识指南.
- 早期识别和管理FALD对于改善长期结果至关重要.
- 多学科合作对于优化治疗肝脏疾病的Fontan幸存者至关重要.
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