Clinical Phenotypes of Fontan Failure: Implications for Management. (26th May 2016)

• Record Type: Journal Article
• Authors: Book, Wendy M.; Gerardin, Jennifer; Saraf, Anita; Marie Valente, Anne; Rodriguez, Fred
• Is Part Of: Congenital heart disease. Volume 11:Number 4(2016)
• Page Start: 296
• Page End: 308
• Publication Date: 2016-05-26
• Subjects: Congenital Heart Disease -- Fontan -- Heart Failure -- Cirrhosis -- Portal Hypertension -- Hemodynamics; Congenital heart disease -- Periodicals; 616.1204305
• DOI: 10.1111/chd.12368
• Languages: English
• ISSNs: 1747-079X

Abstract

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.