Familial Atrial Myxoma with Carney's Complex: 1 Case

Kim, Myung Chun; Lee, Jae Young; Park, Choo Chul; You, Seh Young; Cho, Kyu Seok

Abstract

Myxomas are the most common form of intracardiac tumors and are found primarily in the left atrium. In rare cases, Carney and associates have described a syndrome called "the complex of myxoma" consisting of cardiac myxoma, which characteristically is familial, in assocation with two or more of the follow conditions: myxomatous masses (cardiac myxoma, cutaneous myxosma, and mammary myxoid fibroademoma), spotty pigmented lesions of the skin, and endocrine disorders. We report a case of familial atrial myxoma with Carney's complex in a 19-year old woman who has spotty pigmentations on her face, and left atrial myxomas, and myxoma on the right nipple. Her mother and sister share the left atrial myxoma. The myxomas originated in the septum of the left atrium and the anterior leaflet of the mitral valve were successfully excised. In conclusion, family members of affected patients should be screened periodically with echocardiography in an attempt to identify asymptomatic cardiac myxomas. Complete excision and postoperative follow up are necessary to rule out the muticentricity and high rate of recurrent lesions.