Fontan Circulation: Success or Failure?

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• 作者：Bl ; ine Mond¨¦sert ; Fran?ois Marcotte ; Fran?ois-Pierre Mongeon ; Annie Dore ; Lise-Andr¨¦e Mercier ; Reda Ibrahim ; Anita Asgar ; Joaquim Miro ; Nancy Poirier ; Paul Khairy
• 刊名：Canadian Journal of Cardiology
• 出版年：2013
• 出版时间：July, 2013
• 年：2013
• 卷：29
• 期：7
• 页码：811-820
• 全文大小：1195 K

文摘

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.