The surgical options for significant aortic valve disease include either Ross procedure (RP) or aortic valve replacement (AVR). The exercise stress test is routinely performed in these patients to assess the objective functional capacity. This retrospective study was conducted to evaluate the differences and the longitudinal changes of exercise capacity in patients following the RP and AVR for aortic valve disease. This is an IRB approved retrospective study and included patients who had either RP or AVR performed for aortic valve disease and had at least one exercise stress test performed after the surgical procedure. Patients with other congenital heart disease, pacemaker or defibrillators, and those with inadequate data were excluded. Demographic data including age at surgery, type of surgery and type of aortic valve was collected. Data regarding treadmill cardiopulmonary exercise test (CPET) was also collected. A total of 47 patients met inclusion criteria and were equally represented in each group, i.e. RP [n = 23, 73.9% male, age at surgery 11.2 (4.5–15.9) years] vs. AVR [n = 24, 88% mechanical AVR, 60.9% male, age at surgery 15.1 (12.8–19.4) years]. There was a significant decline in predicted oxygen consumption (%VO₂) at time of first post-operative CPET in patients after AVR compared to RP (79 vs. 88%, p = 0.048) over a similar accrued median interval follow-up (4.6 vs. 6.2 years, p = 0.2). The longitudinal follow-up analysis of following AVR (n = 11, 54.5% male, median inter-test duration of 5 years) showed significant decline in peak exercise capacity or VO₂ (34.2 vs. 26.2 vs., p = 0.006). In contrast, after RP (n = 12 patients [58.3% male, median inter-test duration 7.1 of years], exercise capacity and other key parameters remained preserved. In this small sentinel study, we report a better initial exercise capacity among patients after RP compared to AVR over an intermediate follow-up. During longitudinal follow-up in a subset of patients, exercise capacity remained preserved amongst the RP group while it further declined in the AVR group.