Div of Radiology and Nuclear Medicine, Section of Paediatric Radiology, Oslo University Hospital, Oslo, Norway.
Dept of Paediatric Cardiology, Oslo University Hospital, Oslo, Norway.
Abdom Radiol (NY). 2021 Jul;46(7):3205-3216. doi: 10.1007/s00261-021-02994-0. Epub 2021 Mar 10.
Patients with Fontan circulation are at risk of developing hepatic fibrosis/cirrhosis. The mechanisms and disease development are unclear and early secondary liver cancer is a concern. This study will describe hepatic imaging findings in a national cohort of adolescents with Fontan circulation.
The patients prospectively underwent abdominal contrast enhanced magnetic resonance imaging (MRI) including diffusion-weighted imaging. Images were assessed for criteria of fibrosis/cirrhosis including characterization of hepatic nodules. These nodules were in addition, assessed by ultrasonography (US). Nodules ≥ 1 cm were investigated and monitored to evaluate malignant transformation. Clinical and hepatic serological data were recorded.
Forty-six patients, median age of 16.5 years (15.4-17.9 years) were enrolled. All patients underwent US examination and MRI was performed in 35/46 patients. On MRI, 60% had hepatomegaly and 37% had signs of fibrosis/cirrhosis. Seven patients had together 13 nodules ≥ 1 cm in diameter. Only 4/13 (17%) where seen on US. Nodules had variable MRI signal characteristics including hepatobiliary contrast enhancement and two nodules revealed portal venous phase 'wash-out' on the first examination. No further imaging signs of malignancy were revealed during the follow-up period of median 24.4 (7-42) months.
The majority of adolescents with Fontan circulation had imaging findings of fibrosis/cirrhosis of varying severity. US had low detection rate of hepatic nodules compared to MRI. The imaging work-up before transition to adult cardiology care did not reveal findings suggestive of malignancy. However, the high prevalence of Fontan-associated liver disease calls for surveillance strategies even in childhood.
Fontan 循环患者有发生肝纤维化/肝硬化的风险。其发病机制和疾病进展尚不清楚，早期发生继发性肝癌是一个关注点。本研究将描述 Fontan 循环青少年患者的全国队列的肝脏影像学表现。
前瞻性纳入的患者行腹部增强磁共振成像（MRI）检查，包括弥散加权成像。评估纤维化/肝硬化的影像学标准，包括肝结节的特征。这些结节另外通过超声（US）进行评估。对 ≥ 1cm 的结节进行研究和监测，以评估恶性转化。记录临床和肝脏血清学数据。
46 例患者，中位年龄为 16.5 岁（15.4-17.9 岁）。所有患者均行 US 检查，35/46 例患者行 MRI 检查。MRI 显示 60%的患者有肝肿大，37%的患者有纤维化/肝硬化的迹象。7 例患者共有 13 个 ≥ 1cm 的结节。只有 4/13（17%）在 US 上可见。结节的 MRI 信号特征各异，包括肝胆对比增强，2 个结节在首次检查时门静脉期呈现“洗脱”表现。在中位随访 24.4（7-42）个月期间，未发现进一步的恶性肿瘤影像学征象。
大多数 Fontan 循环的青少年患者有不同严重程度的纤维化/肝硬化影像学表现。与 MRI 相比，US 对肝结节的检出率较低。在过渡到成人心脏病学治疗之前的影像学检查未发现提示恶性肿瘤的表现。然而，Fontan 相关肝病的高患病率呼吁即使在儿童期也应采取监测策略。
