Background : Connective tissue disease (CTD) is an idiopathic autoimmune disorder which causes systemic chronic inflammation. Inflammation causes various cardiovascular diseases. Systemic steroid use, which is usually the sole treatment for CTD, also causes arteriosclerosis. Although cardiovascular surgery is often necessary in patients with CTD, preexisting multiple organ dysfunction related to CTD, in addition to systemic administration of steroids or other immunosuppressants, is thought to increase the risk of surgery. However, little is known about how the disease process of CTD influences early and late cardiovascular surgery outcomes. Methods : To better understand these issues, we reviewed 31 patients with CTD (study group) and compared their outcomes to those of other patients (control group) who underwent cardiovascular surgery at our institution between April 2008 and November 2013. Results : There were 26 women and 5 men, and the average age was 64.4±16.7 years. CTD types included rheumatoid arthritis in 7 patients, systemic lupus erhythematosus in 6, aortitis syndrome in 6, polymyalgia rheumatica in 3, scleroderma in 3, polymyositis in 3, and others. The procedures included 10 valve cases, 10 coronary artery bypass grafting (CABG) or CABG-valve combination cases, and 11 isolated or complicated thoracic aortic surgery cases. Prior to undergoing these procedures, 24 patients (77.4%) were treated with steroids and/or immunosuppressant, and 6 patients had been diagnosed with interstitial pneumonia in the study group. Moreover, the rate of peripheral artery disease and carotid artery stenosis in the study group was significantly higher than that in the control group. There were no perioperative deaths in the study group. There were no significant differences in terms of major complications such as ischemic events, infection, acute kidney injury, lung injury, and others between the groups. We conducted a follow-up survey for the study group with an average period of 27.8±16.0 months. During the follow-up period, there were 4 late deaths. In addition, 8 patients required readmission, 6 for cardiovascular events and 2 for poor wound healing. All the survivors in the study group showed improved cardiac function and were in the NYHA functional class I and II. Conclusion : Cardiovascular surgery for patients with CTD can provide acceptable early and mid-term results.