title: Clinical Approaches to the Patient with a Failing Fontan Procedure.
abstract: The Fontan operation has been nothing short of revolutionary in its influence on the modern management of patients born with functionally univentricular hearts. The vast majority of these individuals are now surviving well into adulthood. In the 45 years since its introduction, however, there has been increasing recognition of Fontan survivors as a vulnerable population with an altered physiology that has remarkably broad, adverse impact on their long-term health. In this review, the authors discuss the varied manifestations, both cardiac and extracardiac, of the failing Fontan circulation and potential therapeutic options. In addition, a general clinical approach to the patient presenting with Fontan failure is proposed. Ultimately, the key to improving our care and understanding of the Fontan population lies in multi-institutional collaboration and partnership between subspecialty cardiologists and specialists in other organ systems.
authors.affiliation: Section of Pediatric Cardiology, Department of Pediatrics and Internal Medicine, Yale School of Medicine, New Haven, CT, USA.
authors.forename: Robert W
authors.initials: RW
authors.lastname: Elder
authors.affiliation: Division of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. fred.wu@cardio.chboston.org.
authors.forename: Fred M
authors.initials: FM
authors.lastname: Wu
mesh_terms: D018729:Fontan Procedure / Q000379:methods / Q000639:trends; D006330:Heart Defects, Congenital / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D017063:Outcome Assessment, Health Care; D017410:Practice Guidelines as Topic; D012955:Societies, Medical; D018754:Ventricular Dysfunction / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*
keywords: Adult congenital heart disease; Congenital heart disease; Congestive heart failure; Fontan; Hypoplastic left heart syndrome; Single ventricle; Tricuspid atresia