Objectives: 

Fontan-associated liver disease (FALD) has emerged as a nearly universal chronic comorbidity in patients with univentricular congenital heart disease who undergo the Fontan procedure. There is a paucity of data reporting long-term outcomes and the impact of FALD in this population.

Methods: 

Patients who underwent the Fontan procedure between 1992 and 2018 were identified using California registry data. Presumed FALD was assessed by a composite of liver disease codes. Primary outcomes were mortality and transplant. Multivariable regression and survival analyses were performed.

Results: 

Among 1436 patients post-Fontan, 75.9% studied were adults, with a median follow-up of 12.6 (8.4, 17.3) years. The population was 46.3% Hispanic. Overall survival at 20 years was >80%, but Hispanic patients had higher mortality risk compared with White patients [hazard ratio: 1.49 (1.09–2.03), P=0.012]. Only 225 patients (15.7%) had presumed FALD, although >54% of patients had liver disease by age 25. FALD was associated with later deaths [median: 9.6 (6.4–13.2) years post-Fontan] compared with patients who died without liver disease [4.1 (1.4–10.4) years, P=0.02]. Patients with FALD who underwent combined heart liver transplant had 100% survival at 5 years, compared with only 70.7% of patients who underwent heart transplant alone.

Conclusions: 

In this population-based analysis of long-term outcomes post-Fontan, Hispanic ethnicity was associated with increased all-cause mortality. Further, the prevalence of FALD is underrecognized, but our data confirms that its incidence increases with age. FALD is associated with late mortality but excellent posttransplant survival. This emphasizes the need for FALD-specific liver surveillance strategies in patients post-Fontan.

中文翻译：

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Fontan 相关肝病和 Fontan 手术长期幸存者的种族差异评估：一项基于人群的研究

目标： 

Fontan 相关肝病(FALD) 已成为接受 Fontan 手术的单心室先天性心脏病患者几乎普遍存在的慢性合并症。报告长期结果以及 FALD 对这一人群影响的数据很少。

方法： 

使用加州登记数据识别 1992 年至 2018 年间接受 Fontan 手术的患者。通过肝脏疾病代码的组合来评估推定的 FALD。主要结局是死亡率和移植。进行多变量回归和生存分析。

结果： 

在 Fontan 治疗后的 1436 名患者中，75.9% 是成年人，中位随访时间为 12.6 (8.4, 17.3) 年。人口中 46.3% 是西班牙裔。20年总生存率>80%，但与白人患者相比，西班牙裔患者的死亡风险更高[风险比：1.49（1.09-2.03），P =0.012]。只有 225 名患者 (15.7%) 被推定为 FALD，尽管 > 54% 的患者在 25 岁时患有肝病。与没有死亡的患者相比，FALD 与后期死亡相关[中位值：Fontan 后 9.6 (6.4–13.2) 年]肝脏疾病[4.1（1.4–10.4）年，P =0.02]。接受联合心肝移植的 FALD 患者 5 年生存率为 100%，而单独接受心脏移植的患者只有 70.7%。

结论： 

在对丰坦后长期结果的基于人群的分析中，西班牙裔种族与全因死亡率增加相关。此外，FALD 的患病率尚未得到充分认识，但我们的数据证实其发病率随着年龄的增长而增加。FALD 与晚期死亡率相关，但移植后存活率极好。这强调了在 Fontan 术后患者中采取针对 FALD 的肝脏监测策略的必要性。