Montreal Heart Institute Adult Congenital Center, Université de Montréal, Montreal, Québec, Canada.
Can J Cardiol. 2013 Jul;29(7):811-20. doi: 10.1016/j.cjca.2012.12.009. Epub 2013 Mar 6.
Fontan surgery represents a milestone in the evolution of congenital heart disease management. It achieved the seemingly improbable (ie, restoration of a noncyanotic state by entirely bypassing the subpulmonary ventricle). In so doing, it has allowed a generation of children who may have otherwise succumbed to their severe congenital heart defect to survive to adulthood. Perfect univentricular physiology is, however, an elusive goal. The Fontan circulation inherently represents a hemodynamic compromise that results in a catalog of potential multiorgan complications. In this review, we explore current knowledge regarding pathophysiology of the failing Fontan, its varied clinical manifestations, and potential therapeutic options. Failure of the Fontan circulation is broadly divided into 3 overlapping categories: ventricular dysfunction, systemic complications of Fontan physiology, and chronic Fontan failure. As long as the Fontan operation continues to serve as the paradigm of care for patients with univentricular hearts, efforts must be directed toward supporting this dynamic circulation that progressively declines in efficiency with age. Continued research in therapies is needed for univentricular dysfunction and systemic complications of Fontan palliation, including potential uses of mechanical support as a bridge to transplantation or as a neosubpulmonary ventricle. Fontan patients remain a major challenge to the medical and surgical community as a whole. Multicentre and multidisciplinary efforts to improve the density and depth of experiences might lead to a better appreciation for, and management of, Fontan failure and its ramifications.
Fontan 手术代表了先天性心脏病管理演变的一个里程碑。它实现了看似不可能的目标（即完全绕过肺动脉下心室，使非发绀状态得以恢复）。这样一来，它使一代可能因严重先天性心脏病而夭折的儿童得以成年。然而，完美的单心室生理学是难以实现的目标。Fontan 循环本质上代表了一种血流动力学的妥协，导致一系列潜在的多器官并发症。在这篇综述中，我们探讨了心力衰竭的 Fontan 病理生理学、其各种临床表现以及潜在的治疗选择。Fontan 循环衰竭大致分为 3 个重叠类别：心室功能障碍、Fontan 生理学的全身并发症和慢性 Fontan 衰竭。只要 Fontan 手术继续作为单心室心脏患者的护理典范，就必须努力支持这种随着年龄增长效率逐渐下降的动态循环。需要对单心室功能障碍和 Fontan 姑息治疗的全身并发症进行持续的治疗研究，包括机械支持作为移植或新肺动脉下心室的桥梁的潜在用途。Fontan 患者仍然是整个医疗和外科界的一个重大挑战。多中心和多学科的努力可以提高经验的密度和深度，从而更好地了解和管理 Fontan 衰竭及其后果。
