发病率,风险因素和Fontan相关肝病的预后:系统性审查和探索性元分析
Jacob Y Cao1, Kathyrn M Wales2, Yves d'Udekem3
1Department of Cardiology, St. Vincent's Hospital, Sydney, Australia; Sydney Medical School, University of Sydney, Sydney, Australia.
JACC. Advances
|April 26, 2025
概括
在Fontan循环后,肝脏疾病很常见,肝硬化影响了21%的患者. 风险因素,如Fontan后的几年和心室功能障碍影响结果,强调需要明确的Fontan相关肝病定义.
科学领域:
- 心脏病学 心脏病学
- 肝病学 肝病学是一种肝病学.
- 儿童心脏病学 儿童心脏病学
背景情况:
- 患有Fontan循环的患者面临着进展性肝病的风险.
- 丰坦相关性肝病 (FALD) 的患病率和危险因素尚未得到充分了解.
研究的目的:
- 审查关于FALD患病率,诊断方法和风险因素的公正数据.
- 在可用的FALD数据上进行探索性元分析.
主要方法:
- 对37项未经选择的FALD查研究 (5,701名患者) 的系统审查.
- 结果包括肝硬化,晚期纤维化,门高血压和肝细胞癌.
- 使用了探索性元分析,子组分析和元回归.
主要成果:
- 估计的FALD患病率高度异质.
- 在21%的患者中诊断出肝硬化 (范围为0-76%),在30%的患者中诊断出晚期纤维化,在17%的患者中诊断出门门高血压,在2%的患者中诊断出肝细胞癌.
- 肝硬化风险因素包括Fontan后的几年,心房肺Fontan,心室功能障碍和肺毛细血管压升高. 图像检查诊断出肝硬化比活检更常见.
结论:
- 肝脏疾病是常见的Fontan后完成,在患病率有显著的变化.
- 鉴定的风险因素可以指导患者对FALD的查.
- 标准化,具有预后意义的FALD定义对于未来的研究和临床实践至关重要.
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