Authors: Shaddy, Robert E; Webb, Gary

Source: Expert Review of Cardiovascular Therapy, Volume 6, Number 2, February 2008 , pp. 165-174(10)

Publisher: Expert Reviews

Abstract:

There is a growing population of adult patients with congenital heart defects in the developed world. Most have been repaired, but few have been cured. Many have myocardial dysfunction. Most have exercise intolerance. Some have heart failure. As a group, they show neurohormonal activation similar to that seen in an adult heart failure population with acquired heart disease. Currently, the patients at greatest risk of heart failure are those without a systemic left ventricle, such as Mustard and Senning repairs of transposition of the great arteries (TGA), congenitally corrected TGA, and patients who have had a Fontan procedure. Exercise intolerance may predict hospitalization and death in such patients. For those patients with systemic left ventricles, it would seem reasonable to use the heart failure guidelines developed for patients with acquired heart disease. For those patients without a systemic left ventricle (e.g., a systemic right ventricle or single ventricle), there is currently no foundation for evidence-based therapy.

Keywords: adult; congenital heart disease; heart failure

Document Type: Research article

DOI: http://dx.doi.org/10.1586/14779072.6.2.165

Affiliations: 1: Division Chief, Cardiology, The Children's Hospital of Philadelphia, and Professor of Pediatrics, University of Pennsylvania School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA 19104-4399, USA., Email: shaddyr@email.chop.edu

Publication date: 2008-02-01

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• Expert Review of Cardiovascular Therapy provides expert commentary on the clinical applications of the new medicines, therapeutic agents and diagnostics in cardiovascular disease. Coverage inculdes drug therapy, heart disease, vascular disorders, hypertension, cholesterol in cardiovascular disease, heart disease, stroke, heart failure and cardiovascular surgery.
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