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.

A 75-year-old man who presented with fever and cough was given a diagnosis with active pulmonary valve endocarditis and transferred to our institution. Blood cultures were positive for Enterococcus faecalis, and transthoracic echocardiography showed a mobile vegetation attached to the pulmonary valve. Despite an 8-week treatment with antibiotics, a relapse of the infection required surgery. During the surgical procedure, we found that the vegetation had destroyed all of the pulmonary valve leaflets. After excising the pulmonary valve leaflets, we implanted a bioprosthetic valve and closed the pulmonary artery with autologous pericardium. The patient completed a 6-week course of intravenous antibiotics and was discharged on postoperative day 68. Postoperative transthoracic echocardiography demonstrated an adequate effective orifice area index. Our case report of isolated pulmonary valve endocarditis without predisposing factors is rare. The implantation of a bioprosthetic valve and enlargement with an autologous pericardial patch is an effective option for achieving a satisfactory hemodynamic profile.

Chylorrhea is a rare complication after cardiothoracic surgery, occurring in 0.5-2% of patients. It is extremely rare after coronary artery bypass grafting. The initial management of chylorrhea is conservative, but if it is unsuccessful, surgical intervention is indicated. Recently, some cases treated with octreotide have been reported. We report two cases of chylorrhea after internal thoracic artery harvest treated with octreotide.

For A 38-year-old male diagnosed a congenital complete atrioventricular block in the neonatal period, epicardial lead and pacemaker was implanted through left thoracotomy. Although we tried to implant a pacemaker through the subclavian vein as an adult, it was unsuccessful because of obstruction of the bilateral subclavian vein. For this reason, we performed a pacemaker implantation with transatrial-endocardial lead through the right thoracotomy due to save the generator electric power. This is one of the useful techniques for cases with obstruction of the upper extremity vein.

An 84-year-old woman with severe aortic stenosis (AS) and coronary artery disease (CAD) was admitted repeatedly with syncope and heart failure. Due to her comorbidities, concomitant transapical transcatheter aortic valve replacement (TAVR) and off-pump coronary artery bypass grafting (OPCAB) were performed. She did well postoperatively. CAD is often found concurrently in patients presenting with severe symptomatic AS. Concomitant TAVR and OPCAB is considered as a less invasive and more feasible treatment option in high-risk patients.

A 76-year-old woman required aortic valve replacement due to severe aortic stenosis. She had a huge thyroid cancer, which invaded the innominate and left internal jugular veins. We planned a two-stage operation : the first involved aortic valve replacement ; and the second involved operation of the thyroid cancer. To avoid median sternotomy, we adopted the right parasternal approach. A 7-cm right parasternal skin incision was made. The third and fourth costal cartilages were cut and bent into the right thoracic cavity, without removal of the ribs. The postoperative course was uneventful, and second operation was performed via the median sternotomy approach on postoperative day 53. The right parasternal approach can be used as an alternative when sternotomy is unsuitable in cases of aortic valve replacement.

A 66-year-old man experiencing fever and dyspnea was transferred to our hospital 2 years ago. He had been on hemodialysis for 30 years due to chronic renal failure and was observed as having aortic stenosis for 5 years. Severe mitral regurgitation and complete atrioventricular block caused by infective endocarditis (IE) were noted. Thus, he emergently underwent double-valve replacement (DVR) and pacemaker implantation. The range of infection extended widely to the right atrium and atrioventricular septum beyond the mitral annulus. The infection was suppressed by perioperative antibiotic therapy. Transthoracic echocardiography (TTE) revealed a cavity approximately 30 mm in diameter in the left ventricle, which was located under the mitral annulus, and it extended to the right atrium 3 months after the operation. A diagnosis of left ventricular pseudoaneurysm associated with IE was then made. Because of progressive expansion of the aneurysm, we performed another operation 2 years after the previous one. The pseudoaneurysm was located in the region of the Koch's triangle, which indicated that it was caused by mitral annular abscess. We closed the orifice of the aneurysm approximately 20 mm in diameter with a polyester patch with a diameter of 35 mm. Postoperative TTE showed that the pseudoaneurysm was thrombosed and had no blood flow. Pathological examination of the wall of the pseudoaneurysm revealed that it consisted of fibrous tissues without myocardium. We encountered a rare case treated by patch closure for the left ventricular pseudoaneurysm after DVR associated with IE.

A 74-year-old woman presented to our hospital with complaints of dysphagia. On examination, we diagnosed extensive thoracic aortic aneurysm and esophageal compression due to a descending thoracic aortic aneurysm. We planned a two-stage approach for repairing the extensive thoracic aortic aneurysm ; the first stage involving the repair of the ascending and arch segments, and the second stage involving the repair of the descending aorta. In the first stage, we performed the Bentall procedure and total arch replacement with a long elephant trunk. Following this, her dysphagia resolved, although the size of the descending aortic aneurysm was the same as that before the procedure (49 mm in diameter). We decided to treat her conservatively in the outpatient clinic without the second stage, because the descending aorta was asymptomatic and not sufficiently large. One year later, she presented with a sudden recurrence of dysphagia and swelling of buttocks. She was diagnosed with an expansion of the descending aortic aneurysm (62 mm in diameter) and a hematoma in the gluteal muscle due to aneurysm-induced disseminated intravascular coagulation (DIC). After emergency admission, she underwent a successful thoracic endovascular aortic repair and was discharged following a smooth recovery from dysphagia and aneurysm-induced DIC. We report this case along with a review of the literature.

A 50-year-old man with an extensive thoracic aortic aneurysm underwent staged surgery which consisted of preceding total aortic arch replacement with the frozen elephant trunk technique using J Graft Open Stent Graft^®, followed by open thoracoabdominal aortic aneurysm repair. During the second operation, the descending aorta was cross clamped along with the preexisting stent graft, and Dacron graft was anastomosed directly to the stent graft using a running 4-0 monofilament suture. The anastomosis site was then covered with a short piece of Dacron graft identical with the stent graft in size to secure hemostasis. We herein discuss our approach in this complex case, focusing on prevention of inadvertent events such as deformation of the preexisting stent graft and unexpected bleeding.

Spontaneous rupture of the thoracic aorta without trauma, aneurysm or dissection is a rare but fatal disease. We reported successful endovascular aortic repair of thoracic aortic spontaneous rupture in 3 patients. Generally, it is difficult to accurately identify the rupture site in the spontaneous rupture. However, by detailed planning based on the data of preoperative CT images, thoracic endovascular aortic repair (TEVAR) can be successfully performed, like surgical repair of spontaneous rupture of the distal aortic arch or descending thoracic aorta. TEVAR should be considered as a first-line therapy, especially, in patients with advanced age or significant comorbidities.