Background <p>Transcatheter aortic valve replacement (TAVR) is contraindicated for rheumatic aortic stenosis (RAS). Evidence on the efficacy and safety of TAVR is scarce despite several cases of RAS recorded in China. This study aimed to evaluate the efficacy of TAVR in patients with RAS.</p> Methods <p>A total of 359 patients were enrolled and divided into the RAS ( <i>n</i> = 71) and non-RAS ( <i>n</i> = 288) groups according to the World Heart Federation guidelines for rheumatic heart disease combined with computed tomography imaging. Propensity score matching was performed to ensure comparability between groups. A self-expanding valve was used in all the patients. Primary endpoints included technical success, device success, and 1-year composite outcomes. Short-term complications and anatomical differences were analysed.</p> Results <p>Patients with RAS had higher N-terminal pro-B-type natriuretic peptide levels and Society of Thoracic Surgeons scores, higher atrial fibrillation rates, and worse cardiac metrics (enlargement, valve regurgitation, and lower estimated glomerular filtration rates). The patients in the RAS group had fewer bicuspid valves, less calcification, more elliptical annuli, and larger left ventricular outflow tract/annulus area ratios. The RAS group had a higher prosthesis positioning and oversizing rate. Technical success, device success, and early safety rates were similar between groups. However, the RAS group had a higher pacemaker implantation and transcatheter heart valve displacement (THVD) risk, yet demonstrated superior 1-year outcomes.</p> Conclusions <p>TAVR may be safe and effective for the treatment of RAS. The anatomical characteristics of patients with RAS increased the risk of THVD and pacemaker implantation. A high-release strategy and appropriate oversizing could partially alleviate the risk of THVD.</p> Graphical Abstract <p>TAVR, transcatheter aortic valve replacement; RAS, rheumatic aortic valve stenosis.</p> <p></p>