Applying heart failure guidelines to adult congenital heart disease patients.
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.
Shaddy
Robert E
RE
Division of Cardiology, The Children's Hospital of Philadelphia, and University of Pennsylvania School of Medicine, 34th Street and Civic Center Blvd, Philadelphia, PA 19104-4399, USA. shaddyr@email.chop.edu
Webb
Gary
G
D000328:Adult; D015415:Biomarkers / Q000378:metabolism; D002319:Cardiovascular System / Q000503:physiopathology; D006330:Heart Defects, Congenital / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D006333:Heart Failure / Q000209:etiology / Q000628:therapy*; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D018377:Neurotransmitter Agents / Q000378:metabolism; D017410:Practice Guidelines as Topic; D016276:Ventricular Function
D015415:Biomarkers; D018377:Neurotransmitter Agents