BACKGROUND: Rheumatic heart disease (RHD) in young people presents a complex management problem. In Australia a significant proportion of those affected are Aboriginal and Torres Strait Islanders. Transcatheter mitral valve-in-valve (TMViV) replacement has emerged as an alternative to redo surgery in high-risk patients with degenerated mitral bioprostheses. The aim of this study is to review outcomes of TMViV replacement in young patients with RHD. METHODS: A single-centre, retrospective review of prospectively collected data on patients undergoing TMViV from December 2017 to June 2021. Primary outcome was major adverse cardiovascular events. Secondary outcome was post-operative trans-thoracic echocardiogram (TTE) results. RESULTS: There were seven patients with a mean age of 33 years and predominantly female (n = 5). Pre-operative comorbidities included diabetes (29%), chronic obstructive pulmonary disease (43%), left ventricular dysfunction (43%) and current smoking status (80%). Post-operative median length of hospital stay was 4 days with no post-operative renal failure, stroke, return to theatre, valve embolization or in hospital mortality. Post-operative TTE showed either nil or trivial central mitral regurgitation, no paravalvular leak and a median gradient of 5 mmHg (IQR 4.5, 7) across the new bioprosthesis; sustained at median follow-up of 22 months. CONCLUSION: Current literature of TMViV replacement is focused on an older population with concurrent comorbidities. This study provides a unique insight into TMViV replacement in a young cohort of patients with complex social and geographical factors which sometimes prohibits the use of a mechanical valve. The prevalence of RHD remains high for Aboriginal and Torres Strait Islanders, planning for future repeat valve operations should be considered from the outset. We consider TMViV as a part of a staged procedural journey for young patients with RHD.