Pediatric Cardiology Department, Prince Sultan Cardiac Center, Riyadh, Saudi Arabia.
Radiology Department, Prince Sultan Medical Military City, Riyadh, Saudi Arabia.
Cardiol Young. 2023 Oct;33(10):1834-1839. doi: 10.1017/S1047951122002992. Epub 2022 Oct 19.
High venous pressures and associated hepatic congestion are important drivers for Fontan-associated liver disease. The prognostic significance of hepatomegaly as a marker of congestion however is not well defined and is further explored in this research study.
Fontan patients who have had liver ultrasound scans were identified from the Prince Sultan Cardiac Centre Fontan Database and had their anatomic, surgical, clinical histories abstracted from the electronic medical records following institutional ethics approval. Liver volumes were determined retrospectively from reviewing individual US images, and these, divided into tertiles, were analysed in the context of the predefined endpoints of (i) Primary - death or heart or liver transplantation, or (ii) Secondary - combined endpoint of death, transplantation, arrhythmia, or protein-losing enteropathy.
Mean indexed liver volumes for the entire cohort (n = 199) were 1065.1 ± 312.1 ml/m, range 387 to 2071 ml/m. Patients with the largest liver volumes (highest tertile) were less likely to have a functioning fenestration compared to those in the lowest tertile 44% versus 56% p = 0.016 and experienced the highest burden of mortality and heart or heart-liver transplantation, p = 0.016, and were more likely to reach the composite endpoint of death, protein-losing enteropathy, arrhythmia, or transplantation, p = 0.010. Liver volumes had an overall predictive accuracy for the combined outcome of 61% (CI 53%, 67%, p = 0.009).
Liver volumetry may serve as a potentially important congestion biomarker for adverse outcomes after the Fontan operation.
高静脉压和相关的肝充血是导致 Fontan 相关肝病的重要因素。然而，作为充血标志物的肝肿大的预后意义尚未明确，本研究对此进行了进一步探讨。
从王子苏丹心脏中心 Fontan 数据库中确定了接受过肝脏超声检查的 Fontan 患者，并在获得机构伦理批准后，从电子病历中提取他们的解剖学、手术和临床病史。通过回顾个人超声图像来确定肝脏体积，将其分为三分位，并根据以下预设终点分析：（i）主要终点 - 死亡或心脏或肝脏移植，或（ii）次要终点 - 死亡、移植、心律失常或蛋白丢失性肠病的联合终点。
整个队列（n = 199）的平均指数化肝体积为 1065.1 ± 312.1 ml/m，范围为 387 至 2071 ml/m。与最低三分位组相比，肝体积最大（最高三分位）的患者更有可能没有功能窗，分别为 44%和 56%，p = 0.016，且死亡率和心脏或心肝移植负担最高，p = 0.016，更有可能达到死亡、蛋白丢失性肠病、心律失常或移植的复合终点，p = 0.010。肝体积对复合终点的总体预测准确性为 61%（CI 53%，67%，p = 0.009）。
肝体积测定可能是 Fontan 手术后不良结局的潜在重要充血生物标志物。
