Heart failure (HF) in the adult with congenital heart disease (ACHD) is associated with high morbidity and mortality and has been implicated as the leading cause of death in this patient population. The diagnosis of HF in ACHD involves a combination of clinical suspicion from subjective patient history, anatomic imaging, functional diagnostic studies, and rhythm evaluation. Once diagnosed, the approach to management of HF in this population varies widely and by lesion. Unfortunately, there is a paucity of literature available delineating the optimal management of these patients, making clinical decisionmaking extremely challenging. In this review, we aim to summarize available evidence to help guide the diagnosis and management of HF in ACHD.

Introduction

An estimated two-thirds of people with congenital heart disease are adults. This population continues to grow now that nearly 90% of infants born with congenital heart disease survive into adulthood1,2 . With this aging population, the development of heart failure (HF) has become one of the most challenging sequelae of palliated congenital heart disease, often implicated as the leading cause of death in adults with congenital heart disease (ACHD)3–۷ . In a study by Zomer et al. using the Dutch CONCOR Registry, HF admissions occurred in 2.5% of the ACHD population and was associated with a 5- fold higher risk of mortality during a 21-year follow-up compared to ACHD patients who had no previous history of HF admissions8 . The alarmingly high morbidity and mortality associated with HF in ACHD demands attention and current knowledge on its continuously evolving management. In this article, we provide a broad overview of available evidence to help guide the diagnosis and management of HF in ACHD.