title: Fontan circulation: success or failure?
abstract: 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.
authors.affiliation: Montreal Heart Institute Adult Congenital Center, Université de Montréal, Montreal, Québec, Canada.
authors.forename: Blandine
authors.initials: B
authors.lastname: Mondésert
authors.forename: François
authors.initials: F
authors.lastname: Marcotte
authors.forename: François-Pierre
authors.initials: FP
authors.lastname: Mongeon
authors.forename: Annie
authors.initials: A
authors.lastname: Dore
authors.forename: Lise-Andrée
authors.initials: LA
authors.lastname: Mercier
authors.forename: Reda
authors.initials: R
authors.lastname: Ibrahim
authors.forename: Anita
authors.initials: A
authors.lastname: Asgar
authors.forename: Joaquim
authors.initials: J
authors.lastname: Miro
authors.forename: Nancy
authors.initials: N
authors.lastname: Poirier
authors.forename: Paul
authors.initials: P
authors.lastname: Khairy
mesh_terms: D000328:Adult; D002648:Child; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D011183:Postoperative Complications; D017211:Treatment Failure; D018754:Ventricular Dysfunction