ABSTRACT
The adult congenital heart disease population continues to grow and is estimated at 1.2 million in the United States alone. Despite vast improvements in survival afforded by stepwise advances in congenital heart disease surgery and diagnostic, medical, and perioperative care, adults with congenital heart defects often have residual hemodynamic lesions, fibrosis, and surgical scarring, and are at increased risk of arrhythmias, myocardial dysfunction, and pulmonary vascular disease. As a result, more than a third of patients experience heart failure during their lifetime. Specialist training and concentration of expertise in centers of excellence are required to meet the needs of this unique population. The rapid expansion of the adult congenital heart disease population, however, will lead to an increasing number of patients presenting to other specialties and primary care physicians with heart failure. A good working knowledge of the principles underpinning heart failure in adult congenital heart disease is useful for all cardiologists, to facilitate safe triage, initial management, and effective communication with specialists. The aim of this chapter, for specialists and non-specialists alike, is to highlight the most significant aspects of heart failure in this patient group, including epidemiology, heart failure mechanisms, and special considerations in diagnosis and management, including intensive care, mechanical circulatory support, and transplantation.
