BACKGROUND Aortic coarctation has been associated with generalized vascular disease, yet little is known about retinal vascular patterns and their changes over time. OBJECTIVE The aim of this study is to characterize the nature and extent of retinal vascular disease in adults with aortic coarctation, and explore age-related effects and associations with cardiovascular outcomes. METHODS A prospective cross-sectional seroepidemiological study was conducted on 60 consecutive adults with repaired aortic coarctation, age 42.4±14.1 years, 61.7% male. In addition to detailed questionnaires, imaging studies, and laboratory testing, high-quality retinal images were acquired by 45° nonmydriatic digital funduscopy. RESULTS No patient had evidence of hypertensive retinopathy. A distinctive vascular pattern characterized by bilaterally symmetric tortuosity of retinal arteries and veins was observed. Arterial tortuosity was abnormal in 98.3% of patients and decreased with age (P=0.0005). In patients≥45 years, a 1-point increase in the arterial tortuosity score was associated with a 1.5-fold higher risk of cardiovascular complications (i.e., acute coronary syndrome, stroke, cerebral aneurysm, aortic dissection/rupture) [odds ratio 1.50, 95% CI (1.01, 2.24), P=0.0496]. Abnormal venous tortuosity was present in 75.0% of patients and non-significantly correlated with higher levels of serum inflammatory markers (C-reactive protein, fibrinogen, interleukin-6, and tumor necrosis factor-alpha). A higher venous tortuosity score was likewise associated with an increased risk of cardiovascular complications [odds ratio 1.86, 95% CI (1.03, 3.35), P=0.0392]. CONCLUSIONS Adults with repaired aortic coarctation exhibit a unique retinal vascular pattern characterized by excessive arterial and venous tortuosity that regresses with age. Greater tortuosity is associated with adverse cardiovascular outcomes in patients≥45 years.