BACKGROUND AND AIMS: Rheumatic heart disease is the commonest acquired cardiovascular disease worldwide. About 20 years have elapsed since the original Euro Heart Survey on valvular heart disease (VHD) was conducted with multiple changes in practice due to advances in treatment techniques. In this study, we aimed to analyse the management of patients with severe native valve disease or those with previous valvular intervention in comparison with existing European Society of Cardiology guidelines. METHODS: The European Society of Cardiology VHD II registry is an international, prospective, longitudinal multicentre, observational study, which was conducted in 222 centres. The registry included patients with severe native VHD or with previous valvular intervention. Follow-up was undertaken at 6 months at the investigating centre or by telephone. RESULTS: Amongst patients recruited in the European Society of Cardiology VHD II registry, 470 had severe rheumatic mitral valve disease and 332 had previous rheumatic mitral valve intervention. Amongst the patients with Class I recommendation for intervention, it was undertaken in only 70%. Adherence to guideline recommendations was more in patients with native VHD than in those with previous intervention. Total mortality was 1.5% in hospital and 3.5% at 6 months follow-up. Independent predictors of death at 6 months were age, chronic pulmonary disease, New York Heart Association Classes III and IV at presentation, liver dysfunction, and previous myocardial infarction. CONCLUSIONS: Compliance with guideline recommendations for intervention is poor overall in patients with rheumatic valve disease. Concerted educational efforts are needed to improve the management of this vulnerable patient cohort.