Diaz-Frias Josue, Conjeevaram Hari S, Norris Mark D, Joynt Michael R, Yu Sunkyung, Duimstra Ashley, Cotts Timothy B
Division of Cardiovascular Medicine, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI, USA.
Division of Gastroenterology and Hepatology, Department of Internal Medicine, University of Michigan Health System, Ann Arbor, MI, USA.
Pediatr Cardiol. 2025 Aug 18. doi: 10.1007/s00246-025-03988-1.
Fontan-associated liver disease (FALD) is an impactful complication for adults with Fontan circulation. The presence of esophageal varices is associated with increased morbidity and mortality in patients with underlying cirrhosis. The diagnostic yield of routine esophagogastroduodenoscopy (EGD) screening in Fontan patients with clinical or radiographic evidence of cirrhosis is not well known with wide practice variation across centers. We performed a retrospective chart review of all patients with Fontan circulation who underwent an EGD from 2020 to 2023 at our center. During the study period, a total of 66 patients (51.5% male) with Fontan circulation underwent a screening EGD at a median age 30.2 years (range 19.8-60.4 years). Esophageal varices were present in 41 (62.1%) patients, of which 35 (53.0%) had grade I esophageal varices, and 6 (9.1%) had grade II. The study showed a high incidence of esophageal varices in our patients with a Fontan circulation, suggesting a role for routine EGD as a screening tool in this population. This diagnostic procedure was well tolerated with no associated complications.
法洛四联症相关肝病(FALD)是法洛四联症循环的成年患者中一种具有重大影响的并发症。食管静脉曲张的存在与潜在肝硬化患者的发病率和死亡率增加相关。对于有临床或影像学肝硬化证据的法洛四联症患者,常规食管胃十二指肠镜检查(EGD)筛查的诊断率尚不清楚,各中心的实践差异很大。我们对2020年至2023年在我们中心接受EGD检查的所有法洛四联症循环患者进行了回顾性病历审查。在研究期间,共有66例法洛四联症循环患者(51.5%为男性)接受了筛查性EGD,中位年龄为30.2岁(范围19.8 - 60.4岁)。41例(62.1%)患者存在食管静脉曲张,其中35例(53.0%)为I级食管静脉曲张,6例(9.1%)为II级。该研究表明,我们的法洛四联症循环患者中食管静脉曲张的发生率很高,这表明常规EGD作为该人群的筛查工具具有一定作用。该诊断程序耐受性良好,无相关并发症。