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A dvances in surgical and clinical management of congenital heart disease (CHD) have allowed patients to survive into adulthood. The number of adult congenital heart disease (ACHD) patients continues to increase by 5% per year with >1 million individuals in the United States. There is a misperception that ACHD patients are cured; residual hemodynamic and electrophysiologic abnormalities are increasingly prevalent and have increased the need for health care utilization and hospitalizations in this population. Residual issues include ventricular dysfunction, valvular disease, shunts, and arrhythmias that have contributed to the increasing numbers of patients with and at risk for heart failure. Significant morbidity and mortality are associated with heart failure– related hospitalizations. Adult comorbidities like systemic hypertension (HTN), diabetes, and coronary artery disease (CAD) will further contribute to the problem of heart failure in ACHD patients. Evidence suggests that physical inactivity, obesity, diabetes, and acquired cardiovascular disease (CVD) may be at least as prevalent in patients with CHD as in the general population. Furthermore, some types of CHD may place patients at increased risk for developing CVD. Therefore, this review will discuss the evaluation and management of cardiovascular risk factors in adults with CHD so that providers may screen and possibly lower their risk of acquired CVD over the long term. Acquired CVD remains the leading cause of death in the United States. Manifestations of CVD include myocardial infarction (MI), stroke, transient ischemic attacks (TIA), aortic aneurysms, and peripheral vascular disease (PVD). More than 80% of adults with CHD have been identified to have ≥1 cardiovascular risk factors. Preventive measures such as smoking cessation, diet and exercise, screening and treatment for HTN, diabetes, and hyperlipidemia may lower their cardiovascular risk over the long term. This review will discuss the epidemiology and pathophysiology of CVD as it relates to adults with CHD. A review of specific CHD lesions that are at highest risk for the development of CVD will be discussed in detail (Table 1).