The aim of this study was to evaluate the impact of rheumatic heart disease (RHD) on the long-term outcomes of the Ross operation when compared to patients with bicuspid aortic valves (BAV). We analysed patients with rheumatic disease and BAV who underwent a Ross operation between 1995 and 2020. The primary outcomes were late survival and reoperations on the pulmonary autograft and the right ventricular allograft. We also performed a propensity score analysis to adjust for differences in baseline characteristics. The cumulative incidence of the events in the matched cohorts was compared using the Fine-Gray model. There were 147 patients with rheumatic disease and 314 with bicuspid valves. In the unmatched population, patients with rheumatic disease were younger, more symptomatic, had worse left ventricular function and were more likely to present a preoperative diagnosis of aortic insufficiency. Propensity matching resulted in 86 pairs with similar baseline characteristics. Compared with those with bicuspid valves, rheumatic patients had similar 20-year survival both in the unmatched (RHD = 67.8% vs BAV = 79.7%, P = 0.27] and matched cohorts (RHD = 72.5% vs BAV = 82.9%, P = 0.46). Cumulative incidence of reoperations on the pulmonary autograft after 20 years was also comparable between them in the unmatched (RHD 13.4% vs BAV 14.4%, P = 0.67) and matched patients (RHD 11.6% vs BAV 21.1%, P = 0.47). Pulmonary autograft dysfunction due to rheumatic involvement was detected in only three occasions. Cumulative incidence of reoperations on the right-sided allograft was higher in the unmatched cohort (RHD = 5.5% vs BAV = 16.7%, P = 0.035) but became nonsignificant after propensity score matching (RHD = 9.9% vs BAV = 32.3%, P = 0.051). The cumulative incidence of all Ross-related reoperations was similar in both groups. Among patients undergoing Ross operation, patients with RHD had similar long-term survival and rates of reoperations, when compared to patients with BAV.