Children's Heart Center Nevada, Las Vegas, Nevada, USA.
Department of Pediatrics, School of Medicine, Division of Pediatric Cardiology, University of Nevada Las Vegas, Las Vegas, Nevada, USA.
J Card Surg. 2021 Jul;36(7):2329-2335. doi: 10.1111/jocs.15553. Epub 2021 Apr 8.
We investigated a relationship between a composite index comprised of Fontan-circuit anatomical features and hepatic fibrosis scores from biopsy.
We identified living extracardiac Fontan patients, ≥7 years old and ≥5 but <20 years postoperative, that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2020. We divided patients into anatomical groups and applied a risk score to each patient. We compared average anatomical risk scores with average hepatic total fibrosis scores by group.
We identified 111 patients that met inclusion criteria. After excluding four patients, we assigned 107 to one of 12 anatomical variant groups (n ≥ 3). For the 107, the average age at liver biopsy was 14 ± 6 years old. Of the 107, 105 (98%) were New York Heart Association Class 1. We found average anatomical risk scores by group correlated with average total fibrosis scores by group (R = 0.8; p = .005). An average Fontan duration to biopsy of 10 ± 1 years was similar for all 12 anatomical groups. We found no other clinical variables, laboratory, or hemodynamic values that trended with anatomical risk scores or hepatic total fibrosis scores.
In a cohort of relatively young, stable extracardiac Fontan patients, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group. These findings suggest that some anatomical variants in extracardiac Fontan patients are associated with higher Fontan-associated liver disease (FALD)-related hepatic total fibrosis scores than others, despite similar Fontan durations.
我们研究了由 Fontan 循环解剖特征组成的复合指数与活检肝纤维化评分之间的关系。
我们确定了年龄≥7 岁、术后≥5 年且<20 年的活体体外循环 Fontan 患者，并在 2012 年 3 月至 2020 年 9 月期间进行了心导管检查和经静脉肝活检。我们将患者分为解剖学组，并为每个患者应用风险评分。我们比较了各组平均解剖风险评分与平均肝总纤维化评分。
我们确定了 111 名符合纳入标准的患者。排除 4 名患者后，我们将 107 名患者分配到 12 个解剖变异组之一（n≥3）。对于 107 名患者，肝活检时的平均年龄为 14±6 岁。107 名患者中，纽约心脏协会心功能分级 1 级者 105 名（98%）。我们发现组间平均解剖风险评分与组间平均总纤维化评分相关（R=0.8；p=0.005）。所有 12 个解剖组的平均 Fontan 到活检的时间为 10±1 年。我们没有发现其他临床变量、实验室或血液动力学值与解剖风险评分或肝总纤维化评分呈趋势。
在一组相对年轻、稳定的体外循环 Fontan 患者中，平均复合解剖风险评分与按解剖组平均肝总纤维化评分强烈相关。这些发现表明，体外循环 Fontan 患者的某些解剖变异与更高的 Fontan 相关肝病（FALD）相关肝总纤维化评分相关，尽管 Fontan 持续时间相似。
