Lentiginosis associated with a left atrial myxoma

A 14-year-old white male patient with lentiginosis was in congestive heart failure. He was noted to be redheaded, and the lentigines were especially concentrated on the face, including the lips. A two-dimensional echocardiogram revealed an orange-sized mobile mass in the left atrium. Cardiac surgery for removal of the left atrial myxoma was successful, and a complete recovery was made. This is the second report of lentiginosis associated with a left atrial myxoma and the first in which the immediate family did not have similar pigmentary changes. Lentiginosis and associated cardiac manifestations are briefly reviewed.