Acute rheumatic fever and rheumatic heart disease remain major global health problems. Although strategies for primary and secondary prevention are well established, their worldwide implementation is suboptimum. In patients with advanced valvular heart disease, mechanical approaches (both percutaneous and surgical) are well described and can, for selected patients, greatly improve outcomes; however, access to centres with experienced staff is very restricted in regions that have the highest prevalence of disease. Development of diagnostic strategies that can be locally and regionally provided and improve access to expert centres for more advanced disease are urgent and, as yet, unmet clinical needs. We outline current management strategies for valvular rheumatic heart disease on the basis of either strong evidence or expert consensus, and highlight areas needing future research and development.