Cardiac surgery was infrequently performed in patients with systemic lupus erythematosus (SLE), and its clinical outcome was reported only in small series. We sought to evaluate the clinical outcome of cardiac operation in patients with SLE. Between January 1996 and March 2005, 9 patients with SLE underwent cardiac surgery at the authors' hospital. Six patients underwent coronary artery bypass grafting (three conventional and three on-pump beating heart), two patients underwent valve replacement and 1 patient underwent simultaneous heart-kidney transplantation. All 6 patients with coronary artery bypass grafting had saphenous venous grafts and two of them had additional left internal mammary artery graft. The overall in-hospital mortality rate was 11% (1/9). Major postoperative complications occurred in 4 patients (44%) including profuse postoperative bleeding, ventricular tachycardia and early graft thrombosis. There were two late deaths including sudden cardiac death and sepsis. The median follow-up duration was 23 months (range, 1-110). In conclusion, although the postoperative complication was common, cardiac operation could be performed in patients with SLE.