title: Clinical Phenotypes of Fontan Failure: Implications for Management.
abstract: Fontan failure has been variably and inconsistently described in the literature, leading to challenges in comparing studies and evaluating treatments. Development of a conceptual framework to describe clinical phenotypes will aid in consistent terminology in the literature. In the heart failure literature, several key concepts have been described-"heart failure" is a clinical syndrome of various etiologies, with phenotype-specific response to therapies. As the congenital heart disease community struggles to grapple with "Fontan failure," these concepts come to light. Fontan failure in the context of four clinical phenotypes, including evaluation, potential management strategies, and future directions is discussed.
authors.affiliation: Department of Medicine, Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA.|Congenital Heart Center of Georgia, Atlanta, Ga, USA.
authors.forename: Wendy M
authors.initials: WM
authors.lastname: Book
authors.affiliation: Department of Medicine, Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA.|Congenital Heart Center of Georgia, Atlanta, Ga, USA.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Gerardin
authors.affiliation: Department of Medicine, Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA.
authors.forename: Anita
authors.initials: A
authors.lastname: Saraf
authors.affiliation: Boston Adult Congenital Heart Disease and Pulmonary Hypertension Program, Harvard Medical School, Boston, Ma, USA.|Department of Cardiology, Boston Children's Hospital, Boston, Ma, USA.|Department of Medicine, Division of Cardiology, Brigham and Women's Hospital, Boston, Ma, USA.
authors.forename: Anne
authors.initials: A
authors.lastname: Marie Valente
authors.affiliation: Department of Medicine, Division of Cardiology, Emory University School of Medicine, Atlanta, Ga, USA.|Congenital Heart Center of Georgia, Atlanta, Ga, USA.|Department of Pediatrics, Emory University School of Medicine, Atlanta, Ga, USA.|Sibley Heart Center, Atlanta, Ga, USA.
authors.forename: Fred
authors.initials: F
authors.lastname: Rodriguez
mesh_terms: D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D008208:Lymphatic System / Q000503:physiopathology; D010641:Phenotype; D011183:Postoperative Complications / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D012039:Regional Blood Flow; D012307:Risk Factors; D013318:Stroke Volume; D017211:Treatment Failure; D017725:Ventricular Pressure
keywords: Cirrhosis; Congenital Heart Disease; Fontan; Heart Failure; Hemodynamics; Portal Hypertension