Ginde Salil, Hohenwalter Mark D, Foley W Dennis, Sowinski Jane, Bartz Peter J, Venkatapuram Suneetha, Weinberg Catherine, Tweddell James S, Earing Michael G
Departments of Pediatrics, Division of Pediatric Cardiology Radiology Internal Medicine, Division of Cardiovascular Medicine Cardiothoracic Surgery, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
Congenit Heart Dis. 2012 May-Jun;7(3):235-42. doi: 10.1111/j.1747-0803.2012.00632.x. Epub 2012 Feb 23.
Recent data indicate that patients after the Fontan procedure are at risk for significant liver dysfunction; however, the prevalence and extent of liver disease in the Fontan population remains unknown. Furthermore, limited data exist in regard to screening for liver disease in adult Fontan patients. We sought to determine the prevalence of liver disease in adult patients following the Fontan procedure using computed tomography (CT) and serum biomarkers of liver fibrosis.
Adult Fontan patients underwent screening for liver disease as part of their annual evaluation. Screening consisted of laboratory evaluation and dual-phase liver CT scan. Laboratory evaluation included analysis of liver function, viral hepatitis serologies, and FibroSURE panel (LabCorp), a test that analyzes the results of serum biomarkers to provide a quantitative surrogate marker for liver fibrosis.
Sixteen patients, mean age 30.3 (range 20-41) years, were enrolled in the study. Mean length of follow-up from time of Fontan palliation was 20.5 (range 11-33) years. No patients had serologic evidence of viral hepatitis or synthetic liver dysfunction. Twelve patients (75%) had abnormal FibroSURE scores, seven (44%) had elevated FibroSURE scores predictive of Metavir fibrosis stage F2 or greater on liver biopsy, and one (6%) had a FibroSURE score predictive of cirrhosis on biopsy. All 16 patients had abnormal radiologic liver findings identified on CT, including heterogeneous enhancement in 11 (69%), varices in six (38%), and liver nodules in five patients (31%). Length of time since Fontan surgery correlated significantly with an elevated FibroSURE score (P = .05) and having more CT scan abnormalities (P = .04).
Liver fibrosis detected by serum biomarkers and dual phase CT scan is common in adult patients following the Fontan procedure. Further studies are needed to determine the long-term clinical significance of these findings.
近期数据表明，接受Fontan手术的患者有发生严重肝功能障碍的风险；然而，Fontan人群中肝脏疾病的患病率和严重程度仍不清楚。此外，关于成年Fontan患者肝病筛查的数据有限。我们试图通过计算机断层扫描（CT）和肝纤维化血清生物标志物来确定成年Fontan手术后患者肝病的患病率。
成年Fontan患者作为年度评估的一部分接受肝病筛查。筛查包括实验室评估和肝脏双期CT扫描。实验室评估包括肝功能分析、病毒性肝炎血清学检查以及FibroSURE检测（LabCorp公司），该检测通过分析血清生物标志物的结果来提供肝纤维化的定量替代标志物。
16例患者纳入研究，平均年龄30.3岁（范围20 - 41岁）。从Fontan姑息手术时起的平均随访时间为20.5年（范围11 - 33年）。没有患者有病毒性肝炎的血清学证据或肝脏合成功能障碍。12例患者（75%）FibroSURE评分异常，7例（44%）FibroSURE评分升高，预测肝活检时Metavir纤维化分期为F2或更高，1例（6%）FibroSURE评分预测活检时为肝硬化。所有16例患者CT检查均发现肝脏影像学异常，包括11例（69%）不均匀强化、6例（38%）静脉曲张和5例（31%）肝结节。Fontan手术后的时间长度与FibroSURE评分升高（P = 0.05）以及CT扫描异常增多（P = 0.04）显著相关。
血清生物标志物和肝脏双期CT扫描检测到的肝纤维化在成年Fontan手术后患者中很常见。需要进一步研究来确定这些发现的长期临床意义。
