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.

Total anomalous pulmonary venous connection (TAPVC) is rarely associated with remarkably small left heart structures. In these types of cases, the hemodynamics resembles that of hypoplastic left heart syndrome, and the treatment strategy is controversial. We present the case of a 1-day-old girl with infracardiac TAPVC, small left heart structures (hypoplastic left heart complex), bilateral superior vena cava, and aberrant origin of the right subclavian artery. We performed a semi-emergent first-stage open palliation for repair of TAPVC, because of pulmonary venous obstruction. We concomitantly performed atrial septal defect (ASD) enlargement and bilateral pulmonary artery banding (BPAB). The postoperative course was uneventful and the left heart structures did not grow, so we performed the Norwood procedure and placed a right ventricle-pulmonary artery shunt with a 5.0 mm artificial graft. Subsequently, the left heart structures were not suitable for biventricular repair, so we chose univentricular repair. The patient underwent a bilateral bidirectional Glenn operation and Fontan completion at 6 and 23 months of age, respectively. TAPVC repair, BPAB, and ASD enlargement are reasonable surgical options for a patient with borderline small left heart structures and TAPVC, as they enable us to wait for growth in the left heart structures and to determine whether univentricular or biventricular repair is suitable.

Objective : The aim of this study is to describe a series of patients undergoing reoperation due to hemolytic anemia after mitral valve surgery and assess the mechanisms and surgical outcomes. Methods : Between 2009 and 2014, we performed redo mitral valve surgery in 11 patients who had refractory hemolytic anemia after mitral valve surgery at Kyoto University Hospital. The mean age of the patients was 72.2±6.8 years old, and there were 5 men. Results : Preoperative echocardiography demonstrated that only 3 patients had ≥ grade 3 mitral regurgitation (MR), the rest of the patients had only mild to moderate MR. The mechanisms of severe hemolysis included paravalvular leakage (PVL) after mitral valve replacement (MVR) in 8 patients, structural valve deterioration (SVD) after MVR using a bioprosthesis in one, and residual/recurrent mitral regurgitation after mitral valve plasty (MVP) in two. All the patients except one (re-MVP) underwent MVR. The mean interval between previous operation and current operation was 14.1±9.4 years in post-MVR cases, and 2.0±1.9 years in post-MVP cases. There were three late deaths, one of which was due to cardiac death (exacerbation of heart failure due to pneumonia). There was one patient who required re-MVR for recurrent hemolysis due to PVL after MVR. Conclusion : Although hemolytic anemia after mitral valve surgery is rare, it often requires reoperation regardless of the degree of MR at late follow-up period. Thus, patients after mitral valve surgery should be carefully followed-up.

We report a case of 76 year-old woman who had previously undergone coronary artery bypass grafting (CABG) with the right internal thoracic artery (RITA) bypassed to the left anterior descending artery. Six years after CABG, she developed acute type A aortic dissection, and she was medically treated because the false lumen was thrombosed and it was considered that surgical intervention would be high risk for the patent RITA graft crossing between the sternum and the ascending aorta. During follow-up, her aortic aneurysm enlarged to 57 mm in diameter, and finally she was referred to our hospital for surgical intervention. In this case, preservation of the patent RITA graft was thought to be critical because the RITA graft was the only blood source for the left anterior descending artery. Prior to re-median sternotomy, we performed a right anterior minithoracotomy to make sufficient space between the sternum and the RITA graft, and then instituted peripheral cardiopulmonary bypass to decompress the heart. After re-sternotomy, we ensured minimum dissection of the RITA graft, and we successfully accomplished graft replacement of the ascending aorta to the aortic arch without injuring the patent RITA graft. In cases with a patent RITA graft and an ascending aortic aneurysm close to the sternum, our strategy is considered to be efficient for re-median sternotomy.

Although there have been several studies that compared the efficacy of percutaneous coronary intervention (PCI) and coronary artery bypass grafting (CABG), the impact of off-pump CABG (OPCAB) has not been well elucidated. Among the 9,877 patients undergoing first myocardial revascularization enrolled in the CREDO-Kyoto Registry (a registry of first-time PCI using bare-metal stents and CABG patients in Japan), 6,327 patients with multivessel and/or left main disease were enrolled in the present study (PCI 3,877/CABG 2,450). Median follow-up was 3.5 years. Propensity-score-adjusted all-cause mortality after PCI was higher than that of CABG (hazard ratio [95% confidence interval] : 1.37 [1.15-1.63], p< 0.01). The incidence of stroke was lower after PCI than that after CABG (0.75 [0.59-0.96], p=0.02). The predicted risk of operative mortality (PROM) of each patient of on-pump/off-pump CABG was calculated by the logistic EuroSCORE. Patients were divided into tertiles based on their PROM. The hazard ratio of the incidence of stroke in on-pump CABG compared with off-pump CABG in the high-risk tertile was 1.80 ([1.07-3.02], p=0.03). The adjusted overall mortality was not significantly different between the two procedures even in the high-risk tertile (1.44 [0.98-2.11], p=0.06). In patients with multivessel and/or left main disease, CABG was associated with better survival outcomes than PCI using bare-metal stents. Off-pump CABG as opposed to on-pump CABG is associated with short-and long-term benefits in stroke prevention in patients with higher risk as evaluated by the EuroSCORE. No survival benefit of OPCAB was shown, regardless of preoperative risk level.

Extensive calcification of the mitral annulus presents a formidable technical challenge to surgeons and increases the risk of serious complications such as intractable hemorrhage, atrioventricular disruption, and ventricular rupture during mitral valve surgery. We present a case of aortic and mitral valve replacements for a patient with extensive calcification of an intervalvular fibrous body. A 76-year-old woman was admitted with dyspnea on effort, leg edema and syncope. Transthoracic echocardiography showed severe aortic stenosis, and mitral stenosis with regurgitation, and extensive mitral annular calcification. Decalcification was performed with CUSA and we selected a trans-aortic-valve approach for decalcification of the intervalvular fibrous body. The calcification was left to a certain extent in order to preserve annular strength. Postoperative echocardiography showed no perivalvular leakage from either prostheses. The patient was transferred to a local hospital for further rehabilitation.

A 60-year-old man was admitted to our institution with abnormal ECG findings. Coronary CT and angiography showed coronary aneurysms from the left main trunk to the bifurcation of the left anterior descending artery, and the left circumflex artery, with severe stenosis and complete obstruction of the proximal right coronary artery. Morphological evaluation findings strongly suggested that the coronary aneurysms were highly related to childhood Kawasaki disease. We successfully performed triple vessel coronary artery bypass grafting. Here, we report a very rare case of coronary aneurysms presumed to be due to childhood Kawasaki disease in an elderly man.

No abstract available.
Mitral Valve Insufficiency

A 77-year-old woman was referred to our hospital for treatment of a ruptured thoracoabdominal aortic aneurysm (TAAA) with a maximum diameter of 7cm. Considering her age and level of daily activity, the placement of an endovascular stent graft was performed as an emergency rescue procedure. For termination of the endoleak from the distal portion of the stent graft detected by CT the next day, another stent graft placement was added after establishment of blood supply to the superior mesenteric and celiac arteries by placing a Y-shaped graft from the abdominal aorta to each artery with success. The patient was discharged from our hospital 25days after surgery with disappearance of endoleak and good graft patency. A hybrid technique with grafting to abdominal branches, followed by placement of stent graft, can be an alternative treatment for such high-risk patients with ruptured TAAA.