Applying Heart Failure Guidelines to Adult Congenital Heart Disease Patients

Robert E Shaddy; Gary Webb


Expert Rev Cardiovasc Ther. 2008;6(2):165-174. 

In This Article

Abstract and Introduction

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.

More and more patients with congenital heart defects reach adult ages in developed countries. In many countries, there are now more adult patients than there are pediatric patients. This increasing adult congenital heart disease (ACHD) profile brings with it increasing numbers of patients with heart failure and even more who are at risk of developing heart failure. What is known about the assessment and management of these patients? Can heart failure guidelines be applied to these patients? This review will address these questions.