Valvular heart disease (VHD) may be congen- ital, so management overlaps with the 2014 AHA/ ACC Guidelines for the Management of Patients With Valvular Heart Disease, S1.4-2 particularly for bicuspid aortic valve (BAV) disease. Where overlap exists, this document focuses on the diagnosis and treatment of congenital valve disease when it differs from acquired valve disease, whether because of anatomic differ- ences, presence of concomitant lesions, or differences to consider given the relatively young age of patients with ACHD. This guideline is not intended to apply to children (<18 years of age) with CHD or adults with acquired VHD, heart failure (HF), or other cardiovas- cular diseases. The prevalence of ACHD is growing because of the success of pediatric cardiology and congenital cardiac surgery in diagnosing and treating congen- ital heart defects in children. Improved survival to adulthood is most striking for those with the most severe disease, with survival to age 18 years now ex- pected for 90% of children diagnosed with severe Patients with ACHD are a heteroge- neous population, both in underlying anatomy and physiology, as well as surgical repair or palliation. Consequently, although the prevalence of ACHD is in- creasing, the population of patients with a given con- genital abnormality or specific repair may be relatively Patients with CHD are not cured of their disease after successful treatment in childhood. Almost all patients with ACHD will have sequelae of either their native CHD or its surgical repair or palliation, although these sequelae can take decades to man- ifest. The heterogeneity of the population and the long, symptom-free intervals constrain the ability to generate data applicable across the population of ACHD or to adults with specific lesions or repairs. 