Patients with tetralogy of Fallot can survive to late adulthood; however, there are few data on cardiovascular outcomes in this population. We conducted a single-center retrospective analysis of cardiovascular outcomes and risk factors in 208 patients with tetralogy of Fallot to better evaluate the burden of cardiovascular disease in this group. Descriptive statistics were used to determine the prevalence of relevant cardiovascular risk factors and outcomes, including a composite analysis of cardiovascular disease. Rates and mean values from the American Heart Association 2011 Heart Disease and Stroke Statistics Update were used as population estimates for comparison. In tetralogy of Fallot patients, cardiovascular disease prevalence was not different from that found in the general population (40% vs 36%, P=0.3). However, there was significantly more cardiovascular disease in tetralogy of Fallot men aged 20 to 39 years (30% vs 14%, P < 0.05) and in tetralogy of Fallot men aged 40 to 59 years (63% vs 29%, P < 0.0001). This was due to higher prevalence of coronary disease (12% vs 7%, P < 0.05) and heart failure (16% vs 2%, P < 0.0001). In particular, the increased prevalence of heart failure (regardless of pulmonary valve disease) accounts for the frequency of cardiovascular disease in tetralogy of Fallot men aged 20 to 59 years. These data support the need to routinely screen young adult male survivors of tetralogy of Fallot for asymptomatic heart failure. Further studies are needed to determine the incidence, severity, and long-term effects of cardiovascular disease in the adult congenital heart disease population