The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium.
The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium.
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DOI：
10.1007/s00246-012-0315-7
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发表时间：
2012-10
影响因子：
1.6
通讯作者：
Wells, Rebecca G.
中科院分区：
文献类型：
--
作者：
Rychik, Jack;Veldtman, Gruschen;Rand, Elizabeth;Russo, Pierre;Rome, Jonathan J.;Krok, Karen;Goldberg, David J.;Cahill, Anne Marie;Wells, Rebecca G.
随着单心室型先天性心脏病幸存者队列的增长，越来越明显的是，Fontan循环中固有的慢性静脉压升高和心输出量减少的状态为功能状态的进行性下降提供了基础。其中一个风险很大的器官是肝脏。夹在两个毛细血管床之间，肺静脉床位于下游，在没有功能性右心室的情况下通常不添加脉动能量，而内脏床位于上游，由于Fontan循环的固有心输出量限制特性，内脏床可能具有受损的流入，肝脏处于不稳定状态。本文综述了2011年6月在费城儿童医院举行的多学科研讨会上达成的共识。讨论包括目前的知识有关的血液动力学基础的肝脏问题，可用的诊断工具，独特的组织病理学的Fontan手术后的肝脏，并提出了肝纤维化在细胞水平上的机制。在研讨会结束时，作者小组提出了一项共识建议，即在Fontan手术后10年，在我们的实践中对所有患者的肝脏进行临床评价。
As the cohort of survivors with the single-ventricle type of congenital heart disease grows, it becomes increasingly evident that the state of chronically elevated venous pressure and decreased cardiac output inherent in the Fontan circulation provides the substrate for a progressive decline in functional status. One organ at great risk is the liver. Wedged between two capillary beds, with the pulmonary venous bed downstream, which typically has no pulsatile energy added in the absence of a functional right ventricle, and the splanchnic bed upstream, which may have compromised inflow due to inherent cardiac output restriction characteristic of the Fontan circulation, the liver exists in a precarious state. This review summarizes a consensus view achieved at a multidisciplinary symposium held at The Children’s Hospital of Philadelphia in June 2011. The discussion includes current knowledge concerning the hemodynamic foundations of liver problems, the diagnostic tools available, the unique histopathology of the liver after the Fontan operation, and proposed mechanisms for hepatic fibrosis at the cellular level. At the completion of the symposium, a consensus recommendation was made by the authors’ group to pursue a new prospective protocol for clinical evaluation of the liver for all patients in our practice 10 years after the Fontan operation.
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