General principles of heart failure management in adult congenital heart disease

Abstract

The population of adults with congenital heart disease is now an estimated 1.4 million in the United States alone and growing. Unfortunately, survival to adulthood does not equate to a normal life expectancy, and heart failure (HF) has now emerged as the leading cause of death for the adult congenital heart disease (ACHD) patient. As this population continues to grow in number and advance in age, the prevalence of heart failure in the adult with congenital heart disease (ACHD-HF) will undoubtedly continue to increase. However, much of our current understanding of mechanism, diagnosis, and management of ACHD-HF remains limited. We aim to review the current understanding of the proposed definitions, mechanisms, clinical impact, and general management considerations of ACHD-HF while also recognizing the large number of knowledge gaps that persist.

Keywords: Adult congenital heart disease; Fontan; Heart failure; Systemic right ventricle; Tetralogy of Fallot.