Heart failure in adult congenital heart disease

As the adult congenital heart disease population expands and ages due to the success of early diagnosis and intervention, many of those individuals have or will develop myocardial dysfunction and the symptoms of heart failure. Given the incidence of myocardial dysfunction in this population today, expectations are that future numbers of individuals with heart failure over the coming decades may be overwhelming. The clinical characteristics of the adults with congenital heart disease population, in general, are similar to other heart failure populations, including biomarker elevation, exercise intolerance, arrhythmia, and mortality. The etiology of heart failure in adults with congenital heart disease is often multifactorial, resulting from chronic adverse loading conditions, fetal and neonatal events, abnormal ventricular architecture and geometry, and abnormal coronary perfusion, each leading to a final common pathway of myocardial fibrosis. Abundant data demonstrate clear benefit of pharmacotherapy in heart failure from acquired heart disease, including ACE inhibitors, angiotensin receptor blockers, beta-blockers, and aldosterone antagonists. However, in adults with congenital heart disease, the benefit of these medicines has not been clearly shown. As heart failure progresses in this population, ventricular assist devices and cardiac transplantation are being increasingly used; however, experience in utilizing them in adults with congenital heart disease still remains limited.