Lipomatous hypertrophy of the interatrial septum (LHIS) is a histologically benign cardiac lesion that is defined by excessive fat accumulation in the area of the interatrial septum (IAS) that does not include the fossa ovalis. Another unusual illness is lipomatosis, which is defined as a broad overgrowth of mature adipose tissue that involves a large portion of an extremity or trunk. We describe a rare case with significant LHIS accompanied by subcutaneous lipomatosis. Echocardiography revealed a mass in the right atrium in this patient. Magnetic resonance imaging revealed that this mass was composed of the adipose tissue and was an extension of a huge thickened IAS. Furthermore, this significant hypertrophy of the IAS was in direct continuation with the excessive mediastinal and epicardial fat.
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