J Am Coll Cardiol. 2017 Dec 26;70(25):3173-3194. doi: 10.1016/j.jacc.2017.10.045.
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.
在过去的十年中，随着大多数接受过 Fontan 手术的单心室解剖结构患者进入成年期，一种新出现的疾病过程——Fontan 相关肝疾病（FALD）已经出现。FALD 是一种心脏外的并发症，可能导致严重的合并症和过早死亡。FALD 相关的风险因素、病理生理学、纵向后果和治疗选择仍未得到明确界定。尽管我们认识到 Fontan 循环特性与心脏外器官功能障碍有关，但我们对这种关系本质的理解存在许多空白。这种心脏外表现，以及循环的其他晚期并发症，会显著影响生活质量和医疗保健的使用。因此，为了对 FALD 进行正式评估，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。会议的目的是召集成人和儿科肝病学、先天性心脏病（成人先天性和儿科心脏病学）、心力衰竭/移植、流行病学和心胸外科领域的专业专家，以及患者权益维护者、患者、接受过 Fontan 手术的儿童和年轻人的父母，以及研究组织和学会，讨论 FALD 的现状。讨论的主题包括知识空白、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调查结果，并试图为理解和解决 FALD 建立一个平台。
