Thorac. cardiovasc. Surgeon 45 (1997) H. Akbas, K. Kr rali, B. Daglar, V. Kutay, 6. lsik, and C. Yakut
Thorac.cardiovasc.Surgeon 45 (1997) 148 -150
© Georg ThiemeVerlag Stuttgart NewYork
Surgical Treatment of Left-Atrial
Myxoma in Carney's Complex
H. K. Klrali, B. Doglor, V. Kutoy, 6. ond C. Yokut
kosuyolu Heart and Resarch Hospital, Istanbul, Turkey
_ Although rare, cardiac myxomas are the most frequently
encountered primary neoplasms of the heart. Cardiac myxomas
are typically sporadic, benign, non-recurrent tumors, and they
are usually seen in the left atrium. Patients who have myxoma-
associated spotty pigmented skin lesions, endocrine neoplasms
with overactivity, and noncardiac myxomatous tumors are said
to have Carney's complex. This report presents a 21-year-old
woman with Carney's complex. She was operated for tetralogy of
Fallot and total correction was performed at another center
when she was three years old. Two years ago, she was operated
on for a primary pigmented nodular adrenocortical tumor and
bilateral adrenalectomy was performed. Her present admission
to our clinic was for left-atrial myxoma with associated symp-
toms. Patients with Carney's complex have distinctive clinical
features which separate them from the larger group of patients
with the more common sporadic myxoma. The most important
distinction is that the myxoma syndrome appears to be a multi-
system disease.
Key words: Carney's complex - Myxoma
Introduction
Cardiac myxomas are the most common benign neoplasms of
the heart and are found primarily within the left atrium (5,8).
They are isolated, benign, non-recurring tumors of the left at-
rium without associated pathological conditions (3), and they
are usually attached to the atrial septum (1). In addition, a sub-
set of patients with card iac myxoma are recognized and assoc i-
ated with pigmented skin lesions, benign noncardiac myxoma-
tous tumors , and endocrine tumor s with overactivity (12).This
association has been called "Carney's Complex" by Carney and
his colleagues (4,7,12). These patients have distinctive features
which separate them from the larger group of patients with the
more common sporadic myxomas (1).
Case Report
We present a 21-year-old woman with Carney's complex in
whom the left-atrial myxoma was excised successfully. She was
refe rred to our clinic with a left-atrial myxoma discovered dur-
ing a routine follow-up echocardiography. Our patient had
undergone total correction for tetralogy of Fallot at another
center when she was 3 years old in 1978.ln addition, her surgi-
cal history included gastric banding for obesity in 1995, bilat-
eral adrenalectomy for primary pigmental nodular adrenocor-
tical disease which resulted in Cushing's syndrome in 1995, and
a successful incisional hernia repairment last year.