Rychik Jack, Goldberg David, Rand Elizabeth, Semeao Edisio, Russo Pierre, Dori Yoav, Dodds Kathryn
1 Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
2 Division of Gastroenterology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
Cardiol Young. 2013 Dec;23(6):831-40. doi: 10.1017/S1047951113001650.
The Fontan operation, although part of a life-saving surgical strategy, manifests a variety of end-organ complications and unique morbidities that are being recognised with increasing frequency as patients survive into their second and third decades of life and beyond. Liver fibrosis, protein-losing enteropathy and plastic bronchitis are consequences of a complex physiology involving circulatory insufficiency, inflammation and lymphatic derangement. These conditions are manifest in a chronic, indolent state. Management strategies are emerging, which shed some light on the origins of these complications. A better characterisation of the end-organ consequences of the Fontan circulation is necessary, which can then allow for development of specific methods for treatment. Ideally, the goal is to establish systematic strategies that might reduce or eliminate the development of these potentially life-threatening challenges.
尽管Fontan手术是挽救生命的手术策略的一部分，但随着患者存活至二三十岁及更长时间，它会出现各种终末器官并发症和独特的发病情况，且被认识到的频率越来越高。肝纤维化、蛋白丢失性肠病和塑形支气管炎是涉及循环功能不全、炎症和淋巴系统紊乱的复杂生理过程的后果。这些病症以慢性、隐匿的状态表现出来。正在出现一些管理策略，这为这些并发症的起源提供了一些线索。有必要更好地描述Fontan循环的终末器官后果，以便能够开发出具体的治疗方法。理想情况下，目标是建立可能减少或消除这些潜在威胁生命的挑战的系统策略。
