Applying heart failure guidelines to adult congenital heart disease patients

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

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.