Evans William N, Acherman Ruben J, Reardon Leigh C, Ciccolo Michael L, Galindo Alvaro, Rothman Abraham, Winn Brody J, Yumiaco Noel S, Restrepo Humberto
Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA.
Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA.
Pediatr Cardiol. 2016 Jan;37(1):30-6. doi: 10.1007/s00246-015-1234-1. Epub 2015 Jul 18.
We previously noted, in a small group of post-Fontan patients, a possible association between hepatic fibrosis scores and the status of pulmonary blood flow at birth. To further explore this observation, we examined data from all Fontan patients seen in our center from July 2010 to March 2015. We identified 200 patients for analysis. Of the 200 patients, 56 underwent transvenous-hepatic biopsy. Of the 200 patients, 13 (6.5%) had protein-losing enteropathy. We divided both the 56 biopsy patients and the entire cohort of 200 patients into 4 groups: (1) unobstructed pulmonary blood flow at birth with functional left ventricles, (2) unobstructed pulmonary blood flow at birth with functional right ventricles, (3) obstructed pulmonary blood flow at birth with functional left ventricles, and (4) obstructed pulmonary blood flow at birth with functional right ventricles. Analysis of the 56 liver-biopsy patient groups showed median hepatic total-fibrosis scores for the 4 groups of 2 (0-6), 2 (0-8), 3 (2-6), and 4 (1-8), respectively, with statistical significance between groups 4 and 1 (p = 0.031). For the entire cohort of 200 patients, we analyzed the incidence of protein-losing enteropathy for each of the four groups and found protein-losing enteropathy percent occurrences of 0, 2.9, 8.8, and 16.1, respectively, with statistical significance between groups 4 and 2 (p = 0.031) and between groups 4 and 1 (p = 0.025). A history of obstructed pulmonary blood flow at birth, coupled with a functional right ventricle, may predict a poorer long-term Fontan outcome.
我们之前在一小群Fontan术后患者中注意到，肝纤维化评分与出生时肺血流状况之间可能存在关联。为了进一步探究这一观察结果，我们检查了2010年7月至2015年3月期间在我们中心就诊的所有Fontan患者的数据。我们确定了200例患者进行分析。在这200例患者中，56例接受了经静脉肝活检。在这200例患者中，13例（6.5%）患有蛋白丢失性肠病。我们将56例活检患者和200例患者的整个队列分为4组：（1）出生时肺血流无梗阻且左心室功能正常，（2）出生时肺血流无梗阻且右心室功能正常，（3）出生时肺血流梗阻且左心室功能正常，（4）出生时肺血流梗阻且右心室功能正常。对56例肝活检患者组的分析显示，4组的肝总纤维化评分中位数分别为2（0 - 6）、2（0 - 8）、3（2 - 6）和4（1 - 8），第4组和第1组之间具有统计学意义（p = 0.031）。对于200例患者的整个队列，我们分析了四组中每组蛋白丢失性肠病的发生率，发现蛋白丢失性肠病的发生率分别为0、2.9%、8.8%和16.1%，第4组和第2组之间（p = 0.031）以及第4组和第1组之间（p = 0.025）具有统计学意义。出生时肺血流梗阻的病史，再加上右心室功能正常，可能预示着Fontan术后的长期预后较差。
