Evans William N, Acherman Ruben J, Ciccolo Michael L, Carrillo Sergio A, Galindo Alvaro, Rothman Abraham, Mayman Gary A, Adams Elizabeth A, Reardon Leigh C, Winn Brody J, Yumiaco Noel S, Shimuizu Lesley, Inanaga Yoko, Deleon Rowena J, Restrepo Humberto
Department of Pediatric Cardiology, Children's Heart Center Nevada, Las Vegas, Nevada, USA.
Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, Las Vegas, Nevada, USA.
Congenit Heart Dis. 2018 Jan;13(1):38-45. doi: 10.1111/chd.12558. Epub 2017 Dec 1.
We hypothesized that clinic-based, hepatic-ultrasound, elastography measurements, either alone or in combination with other noninvasive variables, might correlate with liver-biopsy fibrosis scores in patients post-Fontan.
Between March 2012 and February 2017, we identified patients post-Fontan that underwent elective cardiac catheterization and simultaneous transvenous hepatic biopsy. From this group, we selected patients that met inclusion criteria for liver-ultrasound, shear-wave elastography. Utilizing the results of elastography, laboratory testing, and time post-Fontan, we constructed a composite Fontan hepatic index as a sum of elastography measurements in kilopascals, model for end-stage liver disease excluding INR scores, and the square root of the number of years post-Fontan. Further, we analyzed correlations between Fontan hepatic index values and fibrosis scores from hepatic biopsy.
We identified a total of 79 post-Fontan patients that underwent cardiac catheterization and liver biopsy. Of the 79 patients, 53 met inclusion criteria, and 32 consented to undergo hepatic-ultrasound elastography. Of the 32 that underwent elastography, data from 30 patients was used for analysis. We found no statistically significant differences in demographics, laboratory values, or cardiac catheterization data between the 30 included patients and the 21 that did not participate. Utilizing data from the 30 included patients, we found a strong, highly statistically significant correlation between the Fontan hepatic index values and total fibrosis scores (R = 0.8, P < .00001). However, the cohort size prevented reliable discriminating cut-off values for the range of total fibrosis scores.
In a small cohort of patients post-Fontan, preliminary findings suggest that the composite Fontan hepatic index might be a clinically useful, noninvasive method of serially monitoring for hepatic fibrosis. Further studies, with large patient cohorts, are necessary to validate our findings and develop clinically useful discriminatory cutoff values.
我们推测，基于临床的肝脏超声弹性成像测量，单独或与其他非侵入性变量相结合，可能与Fontan术后患者的肝活检纤维化评分相关。
在2012年3月至2017年2月期间，我们确定了接受择期心导管检查并同时经静脉肝活检的Fontan术后患者。从该组中，我们选择了符合肝脏超声剪切波弹性成像纳入标准的患者。利用弹性成像结果、实验室检测结果和Fontan术后时间，我们构建了一个复合Fontan肝脏指数，该指数为以千帕为单位的弹性成像测量值、排除国际标准化比值（INR）评分的终末期肝病模型以及Fontan术后年数的平方根之和。此外，我们分析了Fontan肝脏指数值与肝活检纤维化评分之间的相关性。
我们共确定了79例接受心导管检查和肝活检的Fontan术后患者。在这79例患者中，53例符合纳入标准，32例同意接受肝脏超声弹性成像检查。在接受弹性成像检查的32例患者中，30例患者的数据用于分析。我们发现，在纳入分析的30例患者与未参与的21例患者之间，人口统计学、实验室值或心导管检查数据无统计学显著差异。利用纳入分析的30例患者的数据，我们发现Fontan肝脏指数值与总纤维化评分之间存在强的、高度统计学显著的相关性（R = 0.8，P <.00001）。然而，队列规模妨碍了为总纤维化评分范围确定可靠的区分临界值。
在一小群Fontan术后患者中，初步研究结果表明，复合Fontan肝脏指数可能是一种临床上有用的、用于连续监测肝纤维化的非侵入性方法。需要进一步开展大患者队列研究，以验证我们的研究结果并制定临床上有用的区分临界值。
