title: End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis.
abstract: 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.
authors.affiliation: 1 Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.affiliation: 1 Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: David
authors.initials: D
authors.lastname: Goldberg
authors.affiliation: 2 Division of Gastroenterology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Rand
authors.affiliation: 2 Division of Gastroenterology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: Edisio
authors.initials: E
authors.lastname: Semeao
authors.affiliation: 4 Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: Pierre
authors.initials: P
authors.lastname: Russo
authors.affiliation: 1 Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: Yoav
authors.initials: Y
authors.lastname: Dori
authors.affiliation: 1 Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.
authors.forename: Kathryn
authors.initials: K
authors.lastname: Dodds
mesh_terms: D001991:Bronchitis / Q000209:etiology*; D002648:Child; D018729:Fontan Procedure / Q000009:adverse effects*; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology*; D011504:Protein-Losing Enteropathies / Q000209:etiology*