Elevated non-invasive liver fibrosis markers and risk of liver carcinoma in adult patients after repair of tetralogy of Fallot
Elevated non-invasive liver fibrosis markers and risk of liver carcinoma in adult patients after repair of tetralogy of Fallot
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DOI：
10.1016/j.ijcard.2019.04.032
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发表时间：
2019-07-15
影响因子：
3.5
通讯作者：
Ohga, Shouichi
中科院分区：
文献类型：
--
作者：
Yamamura, Kenichiro;Sakamoto, Ichiro;Ohga, Shouichi
背景：充血性肝病和肝细胞癌是 Fontan 手术后的严重并发症。法洛四联症修复术（rTOF）后的成年患者也可能发生因肝充血而导致的肝纤维化。然而其发病率和严重程度仍不清楚。方法：共纳入2009年至2016年111例成人先天性心脏病患者。通过心脏磁共振成像和导管插入术评估的肝纤维化标志物和血流动力学参数对 50 名有明显肺动脉瓣反流和/或狭窄的 rTOF 患者、50 名 Fontan 患者和 11 名对照进行分析。结果：rTOF 患者的肝纤维化标志物显着高于对照组，并且往往低于 Fontan 患者（中位透明质酸：25.8 vs. 15.9 vs. 40.8，IV 型胶原：分别为 129 vs. 113 vs. 166，ng/mL，p < 0.05）。 rTOF 患者比对照患者更频繁地表现出透明质酸水平异常，而比 Fontan 患者则更少（分别为 22% vs. 0% vs. 38%，p < 0.05）。多变量分析表明右心房压力与 IV 型胶原或透明质酸水平呈正相关（每个，p < 0.001，p = 0.003）。腹部超声检查显示 50% 接受测试的 rTOF 患者有肝充血。两名透明质酸水平最高的 rTOF 患者的肝活检显示中度和重度（F2 和 F3）肝纤维化的病理证据，其中一名合并肝细胞癌和胆管癌。 结论：我们首先证明了成年 rTOF 患者的肝纤维化标志物升高。这些水平可能有助于预测进行性肝病以及考虑肺动脉瓣置换术的时机。 (c) 2019 Elsevier B.V. 保留所有权利。
Background: Congestive hepatopathy and hepatocellular carcinoma is a serious complication after Fontan procedure. Liver fibrosis due to hepatic congestion could occur also in adult patients after repair of tetralogy of Fallot (rTOF). However, the incidence and severity remain unclear.Methods: A total of 111 patients with adult congenital heart disease between 2009 and 2016 were enrolled. Liver fibrosis markers and hemodynamic parameters assessed by cardiac magnetic resonance imaging and catheterization were analyzed in 50 rTOF patients having significant pulmonary regurgitation and/or stenosis, 50 Fontan patients and 11 controls.Results: Liver fibrosis markers in patients with rTOF were significantly higher than controls, and tended to be lower than Fontan patients (median, hyaluronic acid: 25.8 vs. 15.9 vs. 40.8, type IV collagen: 129 vs. 113 vs. 166, ng/mL, p < 0.05, respectively). Patients with rTOF showed abnormal hyaluronic acid levels more frequently than controls, and less frequently than Fontan patients (22% vs. 0% vs. 38%, respectively, p < 0.05). Multivariate analyses indicated a positive association of right atrial pressure with type IV-collagen or hyaluronic acid levels (each, p < 0.001, p = 0.003). Abdominal ultrasonography revealed hepatic congestion in 50% of rTOF patients tested. Liver biopsy of the two rTOF patients with highest hyaluronic acid levels showed pathological evidence of moderate and severe (F2 and F3) liver fibrosis and one had combined hepatocellular and cholangiocarcinoma.Conclusions: We first demonstrated elevated liver fibrosis markers in adult patients with rTOF. These levels may help to predict the progressive liver disease as well as consider the timing of pulmonary valve replacement. (c) 2019 Elsevier B.V. All rights reserved.