A review of heart failure in adults with congenital heart disease.

The nearly one-million estimated adult congenital heart disease (ACHD) patients in the United States now outnumber children with congenital heart disease (CHD). With continued improvement in survival due to surgical and medical management of patients born with CHD, there is an overall shift in the burden of care from childhood to adulthood. Due to this transitioning population, the probability of heart failure continues to increase with age and represents nearly one-quarter of all mortality in ACHD. Despite these sobering figures adult cardiologist and fellows continue to have limited exposure in the care of patients with congenital heart disease. The syndrome of heart failure represents a complex derangement of neurohormones, natriuretic peptides, and cytokines leading to progressive symptoms of exercise intolerance, dyspnea, and fatigue. Congenital heart patients represent a unique challenge in both categorization and protocol management of heart failure (HF). It remains unclear if the current four-stage ACC/AHA guidelines for diagnosis and treatment of HF in adults can serve as a meaningful framework for congenital heart patients. Additionally, widely used conventional HF therapy of beta-blockers and angiotensin converting enzyme inhibitors (ACE-I) have not demonstrated clear survival benefit in this population. Unfortunately, adequately powered and controlled randomized studies are grossly lacking and remain challenging to conduct. Nonetheless, a review of heart failure associated with ACHD is provided.