The study involved 63 patients with an echocardiographic, surgical and histopathologic diagnosis of cardiac myxoma who were seen over a period of 20 years. Tumor recurrence or relapse was documented in five of these patients (7.9%), 3 of whom had a confirmed diagnosis of Carney complex, while one other patient had a probable diagnosis. Genetic studies demonstrated abnormalities in the PRKAR1A gene on chromosome 17 in 2 patients and their immediate family. In 11 of the 58 patients who did not experience relapse of the myxoma, genetic studies failed to show any abnormality. In conclusion, the possible presence of the Carney complex should be investigated in patients with multiple myxomas or with a cardiac myxoma whose location is atypical.