The authors report a case of a 54-year-old white male with a coronary fistula associated with double mitral valve disease. The patient was studied by invasive and non-invasive cardiac methods including coronary angiogram in order to reach the correct diagnosis and to define the successful surgical treatment that included the closure of the fistula, partial resection of the left atrium and insertion of a mechanical mitral valve prosthesis. It is concluded that this case represents a very rare association between coronary fistula and double mitral valve disease.
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