Keck School of Medicine, University of Southern California, Los Angeles, CA.
Department of Surgery, University of Southern California, Los Angeles, CA.
Ann Surg. 2022 Sep 1;276(3):482-490. doi: 10.1097/SLA.0000000000005581. Epub 2022 Jun 29.
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.
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.
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.
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.
Fontan 相关肝疾病（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。关于该人群的长期预后和 FALD 的影响，数据报道很少。
使用加利福尼亚州登记处的数据确定了 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评估推定的 FALD。主要结局是死亡率和移植。进行了多变量回归和生存分析。
在 1436 例 Fontan 术后患者中，75.9%为成年人，中位随访时间为 12.6（8.4，17.3）年。该人群 46.3%为西班牙裔。20 年总生存率>80%，但与白人患者相比，西班牙裔患者的死亡率更高[风险比：1.49（1.09-2.03），P=0.012]。尽管 54%以上的患者在 25 岁之前就患有肝脏疾病，但只有 225 例（15.7%）患者被认为患有 FALD。FALD 与 Fontan 后死亡的时间较晚有关[中位数：9.6（6.4-13.2）年]，而无肝脏疾病死亡的患者为 4.1（1.4-10.4）年，P=0.02]。接受心脏肝联合移植的 FALD 患者 5 年生存率为 100%，而仅接受心脏移植的患者为 70.7%。
在这项 Fontan 术后长期预后的基于人群的分析中，西班牙裔与全因死亡率增加有关。此外，FALD 的患病率被低估，但我们的数据证实其发病率随年龄增长而增加。FALD 与晚期死亡率相关，但移植后生存率很高。这强调了在 Fontan 术后患者中需要制定 FALD 特异性肝脏监测策略。
