Bulut Ozlem P, Bailey Smita S, Bhat Deepti P
Division of Gastroenterology, Phoenix Children's Hospital, University of Arizona Phoenix, Phoenix, AZ, USA.
Division of Radiology, Phoenix Children's Hospital, University of Arizona Phoenix, Phoenix, AZ, USA.
Cardiol Young. 2024 May 28:1-7. doi: 10.1017/S1047951124025241.
We performed a single-centre retrospective study comparing the accuracy of non-invasive elastography with liver biopsy in accurate assessment of Fontan-associated liver disease.
Fontan patients who underwent combined assessment with a percutaneous liver biopsy and non-invasive elastography between January 2015 and December 2023 at our Children's hospital were included. Liver biopsies were classified using the Congestive Hepatic Fibrosis Score as early Fontan-associated liver disease (scores 1, 2) and advanced Fontan-associated liver disease (score 3/bridging fibrosis and score 4/cirrhosis). Elastography values were categorised as advanced Fontan-associated liver disease for liver elasticity >2.1 m/s by ultrasound and liver stiffness >5 KPa on magnetic resonance elastography.
We included 130 patients (116 children, 89%, mean age at biopsy: 14.6 years ± 3.6) who underwent liver biopsy at a mean duration of 11.1 years (±0.3) following Fontan surgery. Advanced Fontan-associated liver disease was noted in 41 (31.5%) patients with 13 (10%) showing frank cirrhosis. Pre-biopsy ultrasound showed advanced liver fibrosis in 18/125 (14%), with low sensitivity (23%), high specificity (90%), and low accuracy (68%, = 0.1) in diagnosing advanced Fontan-associated liver disease. Similarly, pre-biopsy magnetic resonance elastography showed advanced fibrosis in 23/86 (27%) of patients, with low sensitivity (30%), fair specificity (75%), and low accuracy (63%, = 0.1). Interestingly, advanced Fontan-associated liver disease was missed by ultrasound in 29% and by magnetic resonance elastography in 25% of patients. Advanced Fontan-associated liver disease was associated with lower platelet count (p = 0.02) and higher Gamma-glutamyl Transferase levels (p = 0.02).
Advanced hepatic fibrosis is common among paediatric Fontan patients. Non-invasive elastography may overestimate and underestimate the degree of liver fibrosis, and therefore, liver biopsy may be required for confirming disease severity.
我们进行了一项单中心回顾性研究，比较非侵入性弹性成像与肝活检在准确评估Fontan相关肝病方面的准确性。
纳入2015年1月至2023年12月期间在我院儿童医院接受经皮肝活检和非侵入性弹性成像联合评估的Fontan患者。肝活检采用充血性肝纤维化评分分类，分为早期Fontan相关肝病（评分1、2）和晚期Fontan相关肝病（评分3/桥接纤维化和评分4/肝硬化）。弹性成像值在超声检查中肝脏弹性>2.1 m/s以及磁共振弹性成像中肝脏硬度>5 KPa时被分类为晚期Fontan相关肝病。
我们纳入了130例患者（116例儿童，占89%，活检时平均年龄：14.6岁±3.6岁），这些患者在Fontan手术后平均11.1年（±0.3）接受了肝活检。41例（31.5%）患者被诊断为晚期Fontan相关肝病，其中13例（10%）表现为明显肝硬化。活检前超声检查显示18/125例（14%）患者存在晚期肝纤维化，诊断晚期Fontan相关肝病的敏感性较低（23%），特异性较高（90%），准确性较低（68%， = 0.1）。同样，活检前磁共振弹性成像显示23/86例（27%）患者存在晚期纤维化，敏感性较低（30%），特异性中等（75%），准确性较低（63%， = 0.1）。有趣的是，29%的患者超声检查漏诊了晚期Fontan相关肝病，25%的患者磁共振弹性成像漏诊了该疾病。晚期Fontan相关肝病与较低的血小板计数（p = 0.02）和较高的γ-谷氨酰转移酶水平（p = 0.02）相关。
晚期肝纤维化在儿科Fontan患者中很常见。非侵入性弹性成像可能高估或低估肝纤维化程度，因此，可能需要进行肝活检来确认疾病严重程度。
