Aortic Root and Pulmonary Artery Reconstruction in a Patient with Pulmonary Artery Intimal Sarcoma / 日本心臓血管外科学会雑誌

A 52-year old woman was referred to our hospital because of abdominal pain and restlessness. A chest contrast-enhanced CT showed huge pericardial effusion and intraluminal defects in the main pulmonary artery. We could not make a diagnosis based on the cytology of the pericardial effusion and histopathology of the mass with a sample taken by a catheter. Therefore, we undertook biopsies of the mass by median sternotomy, which led to the diagnosis of pulmonary intimal sarcoma. The tumor resection was performed to release the right ventricular outflow stenosis. We tried to resect the tumor as much as possible, and reconstructed the pulmonary artery and aortic root. She was discharged to home and survived 5 months after surgery.

Cardiovascular Surgery in Patients with Connective Tissue Disease / 日本心臓血管外科学会雑誌

<p><b>Background</b> : 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. <b>Methods</b> : 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. <b>Results</b> : 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. <b>Conclusion</b> : Cardiovascular surgery for patients with CTD can provide acceptable early and mid-term results.</p>

Total Aortic Arch Replacement in a Patient with a Patent Right Internal Thoracic Artery Graft Crossing between the Sternum and the Ascending Aortic Aneurysm / 日本心臓血管外科学会雑誌

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.

Hemolytic Anemia after Mitral Valve Surgery / 日本心臓血管外科学会雑誌

<b>Objective</b> : 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. <b>Methods</b> : 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. <b>Results</b> : 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. <b>Conclusion</b> : 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.

Staged Repair for a Patient with Infracardiac Total Anomalous Pulmonary Venous Connection Complicated by Hypoplastic Left Heart Complex / 日本心臓血管外科学会雑誌

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 <i>vena cava</i>, 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.

Ascending-to-Descending Aortic Bypass through a Median Sternotomy for Residual Coarctation of the Aorta / 日本心臓血管外科学会雑誌

A 11-year-old boy was admitted to our hospital with a diagnosis of the progressive residual coarctation of the aorta, severe left ventricular hypertrophy and dilatation of the ascending aorta. He had previously undergone 3 operations for coarctation of the aorta. We performed ascending-to-descending aortic bypass through a median sternotomy for residual coarctation of the aorta. Partial cardiopulmonary bypass (CPB) was established via the right femoral artery and right atrium. A cephalad retraction of the heart with a heart positioner and a longitudinal pericardial incision over the descending aorta allowed excellent exposure of the aorta through the posterior pericardium. The graft was anastomosed to the ascending aorta and descending aorta. The graft was brought around the right lateral aspect of the right atrium and through to the anterior aspect of right pulmonary veins and inferior vena cava. The bypass graft size was 14 mm in diameter. The CPB time was 134 min, and operation time was 232 min. The postoperative course was uneventful, and he did not suffer from paraplegia. His blood pressure postoperatively normalized without medication. He was discharged 20 days after surgery. The ascending-descending aortic bypass through a posterior pericardium approach is a safe and effective option for relieving residual coarctation and improving hypertension, for patients who have complex coarctation requiring surgical correction. However, because of his young age (II) it is necessary to follow him up carefully.

Coronary Aneurysms in an Elderly Man Presumed to Be due to Childhood Kawasaki Disease / 日本心臓血管外科学会雑誌

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.

Comparison of Clinical Outcomes Using EuroSCORE for Coronary Artery Bypass Grafting with or without Cardiopulmonary Bypass / 日本心臓血管外科学会雑誌

We reviewed 223 cases of isolated coronary artery bypass grafting (CABG) during the past 6 years, and used the EuroSCORE to assess the differences in clinical outcomes between off-pump CABG (OPCAB) and on-pump CABG (conventional CABG: CCABG). After March 2000, our first choice has been OPCAB, with CCABG selected only for cases with unstable hemodynamics. The total of 223 isolated CABG cases consisted of 129 OPCAB and 94 CCABG, but after March 2000, 94 OPCAB and 42 CCABG were performed. Mean EusoSCORE was 5.8 for OPCAB and 4.1 for CCABG, and corresponding expected survival rates were 7.20% and 5.04%. The 3 cases of hospital death (mortality, 1.3%) all belonged to the earlier CCABG groups and were not related to cardiac death. After March 2000, no hospital deaths occurred in either group. Midterm results showed 5 deaths, but these were not related to cardiac death, either. There were no significant differences between the 2 groups in terms of hospital complications other than long mechanical ventilation time, which was markedly longer only for the OPCAB groups (<i>p</i><0.01). Mean number of grafts was significantly high for patients in the CCABG groups (OPCAB 2.1 vs. CCABG 2.8; <i>p</i><0.05). We have therefore been using OPCAB for high-risk cases, and midterm results of our CABG patients were satisfactory.

Successful Surgical Repair of Prosthetic Valve Dehiscence Associated with Aortitis Syndrome in the Healing Phase / 日本心臓血管外科学会雑誌

A 43-year-old woman underwent aortic valve replacement for aortic regurgitation causing aortitis syndrome. The postoperative course had been uneventful and inflammation was controlled by steroid therapy. She developed a moderate degree of dyspnea with cardiomegaly. Two years after the first aorta valve replacement (AVR), severe aortic regurgitation was observed on both echocardiography and aortography. Dehiscence of the prosthetic valve was suspected and an emergency operation was performed. To secure the reimplanted prosthetic valve, we applied the technique of passing felt-pledgeted sutures through the aortic wall in the vicinity to the right coronary cusp and the noncoronary cusp and others through the left coronary cusp with everting mattress sutures. The postoperative course of the second AVR has been uneventful for two months. Since prosthetic valve detachment can occur even if inflammation of aortitis is well controlled, strict management of inflammation is recommended for a prolonged period to prevent reccurence of aortitis and subsequent valve dehiscence.

Reoperation of Obstructed Extracardiac Valved Conduits / 日本心臓血管外科学会雑誌

Obstruction of right ventricle-pulmonary artery bioprosthetic valved conduits can result from valvular degeneration and calcification or neointimal peel formation. From 1968 through 1989, 38 patients underwent repair of congenital heart malformation with a porcine xenograft extracardiac valved conduits from right ventricle to pulmonary artery. Of 27 patients who survived after initial repair, 14 patients (8 males and 6 females) were reoperated for conduit obstructions. Ages of patients at the reoperation ranged 5 to 20yr (mean age 11.8±3.6yr) and the interval between initial repair and reoperation ranged 3 to 9yr (mean 6.6±1.7yr). The obstructed conduits were replaced with mechanical valved conduits (4 patients), nonvalved conduits (7 patients) or outflow patches (3 patients). In a half of patients, obstructions occured at multiple levels within the conduits. Obstructions mainly resulted from valvular degeneration, neointimal peel formation and anastomotic narrowings. There was no operative death but one late death due to the infective endocarditis. The systolic pressure ratio of right ventricle to left ventricle (or aorta) decreased from 0.81±0.13 preoperatively to 0.48±0.10 postoperatively. From our experience, it is recommended to use adequate sized bioprosthetic valued conduits for patients' body weight at the initial repair and replace obstructed conduits to the large sized nonvalved conduit at reoperation if possible.

Pulmonary stenosis after arterial switch operation for complete transposition of the great arteries(TGA) / 日本心臓血管外科学会雑誌

Pulmonary stenosis is the most frequent problem after arterial switch operation for TGA. We experienced four cases of late severe pulmonary stenosis out of twelve patients. All four had supravalvular stenosis either at anastomotic site or at previously banded segment. One patient had associated valvular stenosis and another had bilateral branch stenosis. It is possible that valvular stenosis was due to retraction of equine pericardial patch and branch stenosis was due to overdistension. All four cases were successfully reoperated on 13∼39 months after switch operation. To prevent late pulmonary stenosis, we now alter technique of switch operation in two points. First, the great arteries are anastomosed with interrupted U-shaped sutures from outside of the vessels in whole circumference. Second, both coronary arteries are transferred with punched-out method to save tissue of Valsalva sinus, and the defects are closed with autologous pericardial patch.