Carney complex (CNC) is an inherited autosomal dominant disorder associated with multiple neoplasms. Myxomas associated with CNC differ from their sporadic forms because the former usually develop at a younger age and they may be multicentric and have a tendency to recur. Furthermore, their localization may be atypical. We report the case of a 57-year-old man, with a huge right atrial myxoma obstructing the tricuspid valve orifice. A diagnosis of CNC was established by genetic analysis. The importance of early diagnosis and an adequate follow-up is emphasized.
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