Over the past decade, as the majority of patients with single ventricle anatomy who have undergone the Fontan operation reach adulthood, a newly recognized disease process, Fontan-associated liver disease (FALD), has emerged. FALD is an extracardiac complication that may lead to substantial comorbid disease and premature mortality. The risk factors, pathophysiology, longitudinal consequences, and therapeutic options related to FALD remain poorly defined. Although we recognize that Fontan circulatory properties are associated with extracardiac organ dysfunction, numerous gaps in our understanding of the nature of this relationship exist. Such extracardiac manifestations, in addition to other late complications of the circulation, can significantly affect quality of life and healthcare use. Therefore, to initiate a formal evaluation of FALD, the American College of Cardiology (ACC) sponsored a stakeholders meeting on October 1 to 2, 2015, in Washington, DC. The goal of the meeting was to bring together subspecialty experts in the fields of adult and pediatric hepatology, congenital cardiology (adult congenital and pediatric cardiology), heart failure/transplant, epidemiology, and cardiothoracic surgery, as well as patient advocates, patients, parents of children and young adults who have had the Fontan procedure, and research organizations and societies to discuss the current state of FALD. Topics included gaps in knowledge, optimal care, research opportunities and barriers, and sound practices to guide providers, patients, and families. This report summarizes findings from the stakeholders meeting and seeks to establish a platform for understanding and addressing FALD.

中文翻译：

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Fontan 相关肝病

在过去的十年中，随着大多数接受 Fontan 手术的单心室解剖患者进入成年期，出现了一种新发现的疾病过程，即 Fontan 相关肝病 (FALD)。FALD 是一种心外并发症，可能导致大量合并症和过早死亡。与 FALD 相关的风险因素、病理生理学、纵向后果和治疗选择仍然不明确。尽管我们认识到 Fontan 循环特性与心外器官功能障碍有关，但我们对这种关系的性质的理解存在许多差距。除了循环的其他晚期并发症外，此类心外表现还会显着影响生活质量和医疗保健使用。因此，要启动对 FALD 的正式评估，美国心脏病学会 (ACC) 于 2015 年 10 月 1 日至 2 日在华盛顿特区主办了一次利益相关者会议。会议的目标是汇集成人和小儿肝病学、先天性心脏病学（成人先天性和小儿心脏病学）、心力衰竭/移植、流行病学和心胸外科领域的亚专科专家，以及患者倡导者、患者、家长接受过 Fontan 手术的儿童和年轻人，以及讨论 FALD 现状的研究组织和学会。主题包括知识差距、最佳护理、研究机会和障碍以及指导提供者、患者和家庭的合理做法。本报告总结了利益相关者会议的结果，并寻求建立一个理解和解决 FALD 的平台。