Isolated tricuspid valve disease is rare. We report the case of a 65-year-old female patient who presented with right heart failure. Initial echocardiographic evaluation revealed a giant right atrium (RA) with severe tricuspid inflow obstruction with regurgitation. A comprehensive two- and three-dimensional echocardiographic evaluation revealed a dysplastic tricuspid valve resulting in the inflow obstruction and regurgitation. Rheumatic and carcinoid etiologies were ruled out by relevant tests. It is very rare for dysplastic tricuspid valve to present in late adulthood. The classic echocardiographic findings are discussed followed by a brief review of the literature.
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