Cincinnati Children's Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Cincinnati Children's Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Am J Cardiol. 2022 Feb 15;165:88-94. doi: 10.1016/j.amjcard.2021.10.045. Epub 2021 Dec 7.
Elevated central venous pressure in those with Fontan circulation causes liver congestion and hepatomegaly. We assessed if liver volume by magnetic resonance imaging (MRI) is associated with adverse cardiovascular outcomes. Retrospective study of 122 patients with Fontan circulation who were >10 years old and had a liver MRI with magnetic resonance elastography. Liver volume (ml) was measured by manual segmentation from axial T2-weighted images and was indexed to body surface area. The composite outcome included death, heart transplant, ventricular assist device placement, or nonelective cardiovascular hospitalization. The median age at the time of MRI was 18.9 (interquartile range 15.8 to 25.9) years, and 47% of the patients were women. The mean indexed liver volume was 1,133 ± 180 ml/m. Indexed liver volume was not significantly associated with age, years since Fontan, or with liver stiffness (r = 0.15, p = 0.10), but was positively correlated with Fontan pressure (r = 0.32, p = 0.002). Over a median follow-up of 2.1 (0.8 to 4.2) years, 32 patients (26%) experienced the composite outcome. Higher indexed liver volume was associated with a greater hazard for the composite outcome (hazard ratio per 1 SD increase = 1.74, 95% confidence interval 1.27 to 2.35, p = 0.0004) but increased liver stiffness was not significantly associated with the composite outcome (hazard ratio per 1 SD increase 1.44, 95% confidence interval 0.90 to 2.21, p = 0.11). In conclusion, greater liver volume indexed to body surface area is associated with unfavorable hemodynamics and adverse outcomes in patients with Fontan circulation. Liver volume may be a useful, simple imaging biomarker in adolescents and adults with Fontan circulation.
在 Fontan 循环患者中，中心静脉压升高会导致肝淤血和肝肿大。我们评估了磁共振成像（MRI）测量的肝体积与不良心血管结局的相关性。这是一项回顾性研究，纳入了 122 名年龄大于 10 岁且接受过 MRI 肝脏磁共振弹性成像检查的 Fontan 循环患者。通过对轴位 T2 加权图像进行手动分割测量肝体积（ml），并将其按体表面积指数化。复合结局包括死亡、心脏移植、心室辅助装置植入或非择期心血管住院。MRI 时的中位年龄为 18.9（四分位距 15.8 至 25.9）岁，47%的患者为女性。平均肝体积指数为 1133±180ml/m2。肝体积指数与年龄、Fontan 术后时间或肝硬度无显著相关性（r=0.15，p=0.10），但与 Fontan 压力呈正相关（r=0.32，p=0.002）。中位随访 2.1（0.8 至 4.2）年后，32 名患者（26%）发生了复合结局。肝体积指数越高，复合结局的风险越高（每增加 1 个标准差的危险比为 1.74，95%置信区间 1.27 至 2.35，p=0.0004），但肝硬度增加与复合结局无显著相关性（每增加 1 个标准差的危险比为 1.44，95%置信区间 0.90 至 2.21，p=0.11）。结论：Fontan 循环患者中，按体表面积指数化的肝体积越大，血流动力学越差，结局越差。肝体积可能是 Fontan 循环青少年和成年患者有用的简单影像学生物标志物。
