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The burden of rheumatic heart disease (RHD) continues to be a major contributor to morbidity and premature death in poor and developing countries. We investigated patterns of valvular involvement in patients with RHD as observed in a large tertiary care hospital in eastern Nepal.We retrospectively reviewed transthoracic echocardiography reports from patients diagnosed with RHD between June 1999 and February 2011.Among 10 860 transthoracic echocardiography studies, 1055 female and 658 male patients were diagnosed with RHD, 25.7% of the patients being below 20 years of age. Mitral regurgitation was the most common valvular lesion across all age groups irrespective of sex (n = 1321, 77.1%). Female patients were significantly older as compared to male patients at the time of presentation (32.8 ± 15.2 versus 28.5 ± 15.4 years; P < 0.001) and more commonly presented with mitral stenosis as compared to male patients (62.8 versus 51.5%; P < 0.001), with a peak between the age of 30 and 49 years. Conversely, aortic regurgitation was more common in men as compared to women (55.6 versus 48.9%; P = 0.007). Involvement of both the mitral and the aortic valve was observed in 49.8% of the patients and was more common in men as compared to women (52.7 versus 47.8%; P = 0.047).In this consecutive cohort of patients with RHD in Nepal differential patterns of valvular involvement are observed across sex and age categories.