Familial cardiac myxoma. A study of relatives of patients with myxoma.

PURPOSE: Cardiac myxomas are rare benign tumors of the heart. Although most cases are sporadic, rare familial occurrence has been described. The aim of this study was to evaluate tumor involvement in family members of patients with cardiac myxoma and to compare familial vs nonfamilial cardiac myxoma in relation to age, sex, site and multichamber involvement, endocrine abnormality, embolism, presence of vascular aneurysms, and tumor recurrence. PATIENTS AND METHODS: We studied 38 family members of 14 patients with cardiac myxoma by two-dimensional echocardiography and whenever possible on an annual basis. Patients with cardiac myxoma were then divided into familial cardiac myxoma (6 patients) and nonfamilial cardiac myxoma (12 patients) for the above comparison.
RESULTS: Four family members (10.5 percent) were found to have cardiac myxoma from two different families. The first included a brother and a sister, both with acromegaly, and the second included a mother, daughter, and two sons, in one of whom the tumor was detected on the second annual sons, in two-dimensional study. The patients with familial cardiac myxoma were younger (34.8 years vs 54 years, p < 0.001) than the nonfamilial cases. Right chamber involvement was more common in the familial cases (67 percent vs 8 percent, p < 0.05) and had more frequent recurrence (67 percent vs none, p < .05). There was no difference in endocrine abnormality, vascular aneurysms, or embolism between familial and nonfamilial cases.
CONCLUSION: Cardiac myxoma is relatively frequent in family members with a higher yield of detection in family members of patients with right-sided or bilateral myxoma. Patients with familial cardiac myxoma are younger and have more frequent right-side involvement and long-term recurrence. Screening by echocardiography of family members of patients with cardiac myxoma is recommended. Annual studies are recommended for relatives of patients with familial cardiac myxoma.