A five-year-old boy with a univentricular heart, inferior vena cava interruption, and azygos connection was admitted to our hospital to undergo a staged Fontan-type procedure. Pectus excavatum had developed after he underwent total cavopulmonary shunt at the age of three years. Computed tomography revealed that the hepatic vein was just behind the recessed sternum. We performed simultaneous Nuss and Fontan-type procedures because we were afraid of the compression of the Fontan pathway from the hepatic vein to the pulmonary artery by the recessed sternum. A cardiopulmonary bypass was established and the hepatic vein and pulmonary artery were bypassed with a 16-mm expanded polytetrafluoroethylene graft. After removing the cardiopulmonary bypass, the Nuss procedure was performed. Although the bilateral thoracic cavities were diffusely and densely adhered, adhesiotomy was safely performed under direct visualization. The postoperative course was uneventful. Postoperative computed tomography showed that the pectus excavatum was well repaired and the Fontan pathway was not compressed by the sternum. Although there are few reports of Fontan-type and Nuss procedures being simultaneously performed, this method is useful for securing the space of the Fontan pathway and for preserving good Fontan circulation in the long term.

Aortic dissection during cardiac operation is a rare but serious complication. Early detection and adequate repair is essential in this situation. A 69-year-old man in whom an aortic valve sparing operation for aortic root dilatation with aortic regurgitation had been begun, had an intraoperative aortic dissection 10min after the start of right axillary artery perfusion. Intraoperative transesophageal echocardiography and direct epi-aortic echo revealed acute aortic dissection extending from the aortic root to at least the descending aorta. The dissection was successfully repaired by a Bentall operation and hemiarch replacement using hypothermic circulatory arrest, selective cerebral perfusion, and antegrade perfusion from an anastomosed graft.

Re-do open cardiac surgery may sometimes require complete ablation around the pericardium for the 2 major reasons of attaining better myocardial protection and obtaining effective DC cardioversion. However, this ablation may increase postoperative hemorrhage which may require blood transfusion. Hypothermia is based on the concept of myocardial protection during open heart surgery by suppressing myocardial metabolism, but recently the approach has been changed to maintaining myocardial metabolism with aerobic or anaerobic energy production. We have already reported that histidine-buffered cardioplegia which promote anaerobic glycolysis, provided an excellent functional recovery of myocardium post-ischemia with lower inotropic requirements in a range from 10°C to 37°C of myocardial temperature. Based on our theoretical background and clinical data, we tested the efficacy of this type of cardioplegia in patients receiving multiple surgical procedures with minimum ablation after sternotomy. First case, who had undergone a Bentall procedure for annulo-aortic ectasia 14 years previously had a thrombotic valve and mitral regurgitation. Aortic valve plasty and mitral valve replacement (MVR) was performed. The second case who had undergone MVR 15 years previously had malfunction of the prosthetic valve and underwent re-MVR. The third and fourth cases had ventricular septal defect (VSD) which were closed using Teflon patches. The third case had patch closure during second operation for residual shunt. The fourth case received tricuspid valve replacement (TVR) for tricuspid regurgitation due to a pacemaker lead implanted into the right ventricle through the left subclavian vein. The fifth case received coronary artery bypass surgery in a second operation for restenosis of the graft and progressing atherosclerosis. All hearts started beating spontaneously without DC cardioversion after the aortic unclamp. Ventricular fibrillation occurred in the first case while the patient was weaned from cardiopulmonary bypass and treatment was performed by aortic cross clamp, infusion of the cardioplegia followed by aortic unclamp to start own beat again. Two of 3 patients who were able to donate their own blood preoperatively did not require homologous blood transfusion. Due to advantages such as excellent myocardial protection under hypothermic or normothermic condition, ease of use and relatively lower potassium concentration, histidine-buffered cardioplegia can be an excellent candidate for myocardial protection in re-do cases with less ablation technique.

We report a case of vascular ring with tracheal stenosis, which might be related to a prolonged endotracheal intubation. A symptomatic 2-month-old boy was admitted to our institution after prolonged intubation without a definite diagnosis. His symptoms were stridor and dyspnea, but not dysphagia. Echocardiography detected a vascular ring and this was confirmed by computed tomography and magnetic resonance imaging (MRI) (Edwards IA type). The left anterior aortic arch was divided distal to the left subclavian artery through left thoracotomy and the ligamentum arteriosus was not identified. On postoperative day (POD) 2, endotracheal extubation was unsuccessfully attempted. Further examination such as MRI and bronchoscopy revealed intimal hyperplasia of the trachea with mild compression of the trachea from the outside. We performed aortopexy and division of the small long ductus which might not be a mechanism of the tracheal compression through right thoracotomy in the second operation with successful extubation on POD 3. The patient has been discharged from the hospital and followed up at the outpatient clinic without any symptom. Tracheomalacia was a common associated anomaly in vascular ring. However, other mechanisms such as inflammatory reaction associated with prolonged intubation should be considered and be avoided in the pediatric population.

This paper reports on a case in which a heavily-calcified so-called “porcelain aorta” (including the ductus arteriosus) was observed, together with a patent ductus arteriosus and aortic stenosis associated with a bicuspid aortic valve. A 76-year-old man had been referred to our hospital on a diagnosis of aortic stenosis. Since angiography revealed slight contrast in an area on the right side of the heart, echocardiography was performed and revealed patent ductus arteriosus. Severe circumferential calcification of the ascending aorta and aortic arch was observed on CT scans. Almost no calcification was observed in other areas. Aortic valve replacement and closure of the ductus arteriosus (transpulmonary approach) were performed by means of a balloon to temporarily occlude the aorta, as surgical clamping was impossible due to calcification. Hypothermic systemic perfusion and antegrade selective cerebral perfusion were used. The postoperative progress of the patient was good. Bicuspid aortic valve and patent ductus arteriosus are highly likely to be present in combination in cases of congenital cardiac anomaly, and it is therefore necessary to be particularly attentive when diagnosing such cases. It was considered that our patient, an adult suffering patent ductus arteriosus, was a rare case in which the calcified ductus arteriosus was observed and the calcification had spread to the ascending aorta.

Objective : Transarterial or transapical aortic valve replacement (TAVR) procedures have been performed for high-risk patients with severe aortic valve stenosis (AS) in western countries. A high-risk patient is defined as having an STS score greater than 10%. In Japan, aortic valve replacement (AVR) with cardiopulmonary bypass (CPB) is standard care for AS, even if the patient is at high risk of developing complications. We calculated an expected operative risk of patients using a JAPAN score established by Japanese Adult Cardiovascular Surgery Database (JACVSD). Patients and Methods : Patients were divided into three groups : score less than 5%, low risk (LR) ; score 5-10%, moderate risk (MR) ; score more than 10%, high risk (HR). We also evaluated the efficacy of conventional AVR in each group. Between January 2002 and May 2011, we performed conventional AVR in our hospital and 116 patients who underwent AVR for symptomatic AS were enrolled in this study. Results : There were 79 patients in the LR group, 30 patients in the MR group and 7 patients in the HR group. The mean score was 2.6±1.1% in the LR group, 6.8±1.4% in the MR group and 23.3±16.8% in the HR group respectively. The mean follow-up period was 7.6±0.3 years. Preoperative co-morbidity was not statistically significant among three groups, however more octogenarians were found in the HR group. The aortic valve area and left ventricular ejection fraction (LVEF) were significantly smaller in the HR group. There were 4 cancer patients. The HR group had significantly longer operation and CPB times than the LR group. The operative mortality in all cases was 1.6%. Overall survival at 5 years was 78%. Actual survival at 5 years was 77% in the LR group, 82% in the MR group and 71% in the HR group. The major adverse cardiac and cerebrovascular event (MACCE)-free ratio at 5 years was 85%. Absence of death caused by MACCE at 5 years was 93%. All cancer patients died after AVR due to advancement in cancer. Conclusion : The results of conventional AVR with CPB were satisfactory in each group. Cancer patients may be good candidates for TAVR in the future.

We performed endovascular stent-graft placement on 39 patients with abdominal aortic aneurysms between 1996 and March 2002-a period of approximately 5 years (first half: until the end of June 1998, second half: July 1998 onward). Three patients in the first half of the period and 8 patients in the second half were 80 years or older. Two cases of mycotic aneurysm were observed. During the second half, we encountered high-risk cases in which the patients had complications such as coronary artery disease (5 patients), COPD (1 patient) and thoracic aortic aneurysm (4 patients). Although we had to switch to surgery in 3 patients during the first half of the period, we successfully placed stent-grafts in the other 36 cases (92%). Endoleaks were observed in 6 patients, and dissection of the iliac artery was observed in 5 patients (stents had been placed in all patients). In 50% of all cases in the first half of the period and 89% of all cases in the second half, stent-graft placement was successful and no endoleak was observed. During the follow-up period, 3 cases required additional treatment, and another 4 cases required surgery. Four patients died in hospital during the first half of the period, and 3 patients died during the following 3 years. The 3-year survival rate was 82%. It was considered that stent-graft placement for abdominal aortic aneurysms is particularly effective for high-risk patients, and that the results of this type of therapy will improve in the future.

The patient was a six-month-old girl with an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). She had fever and visited a family physician at 5 months of age. Because of poor oxygenation, she was referred to our pediatric department and intubated soon after hospitalization. Echocardiography showed that the orifice of left coronary artery was just above pulmonary commissure, the left ventricular ejection fraction was 9%, and the level of mitral regurgitation was moderate. Right coronary angiography showed that the left coronary artery contrasted against the collateral arteries. The left coronary artery originated from the left side of the pulmonary trunk. After recovery of the general condition with medical therapy, the patient underwent coronary artery reconstruction by the modified spiral cuff technique on the 21st day of hospitalization. The temporary detachment of pulmonary valve and its commissure for making a margin around the left coronary artery enabled us to make the spiral cuff in almost the usual manner. She was moved to the intensive care unit with the support of extracorporeal membrane oxygenation (VA-ECMO) and was successfully weaned off the VA-ECMO 5 days after the surgery. The postoperative course was good, and she was discharged from our hospital 3 months after the surgery. The echocardiogram one year after the surgery showed a left ventricular ejection fraction of 30%, mild mitral regurgitation, and mild pulmonary regurgitation. Our experience indicates that the spiral cuff technique is a useful coronary reconstruction method for the treatment of ALCAPA, especially in cases presenting a considerable distance between the origin of the left coronary artery and the transplantation site. There are few reports regarding the surgical treatment of infantile ALCAPA showing reduced left ventricular function. Coronary artery reconstruction using the spiral cuff method and planned VA-ECMO are useful surgical procedures in such cases. Our experience also suggests that the establishment of a treatment strategy including mechanical support is essential to improve the results in severe ALCAPA cases.

A 17-days-old-girl with pulmonary artery (PA) sling, patent ductus arteriosus (PDA), and persistent left superior vena cava was admitted to our hospital. Despite good respiratory status just after birth, the respiratory status gradually worsened, and tracheal intubation was performed on 13th day after birth. Emergency division of the PDA was conducted on day 16. Although preoperative computed-tomography did not demonstrate tracheal stenosis, bronchoscopy after tracheal intubation revealed progression of tracheal stenosis with difficulty for stable anesthetic management. Therefore, operation was postponed to re-evaluate tracheal stenosis. Bronchoscopy and computed-tomography revealed the worsening of tracheal stenosis. Eventually, we performed total correction of the PA sling and tracheal stenosis on day 17. Cardiopulmonary bypass was established after median sternotomy. After transection of the PDA, the left PA originating from the right PA was also transected, and transplanted to the main PA. Then, sliding tracheoplasty was performed following the division of the tracheal stenotic region. Her respiratory condition improved after operation, and postoperative computed-tomography showed successful correction of tracheal stenosis. Although few such cases were reported for neonatal PA sling requiring concomitant tracheoplasty, this case suggests that total correction of PA sling and tracheal stenosis is feasible and useful surgical procedure for such cases.

A 74-year-old man was diagnosed with infective endocarditis (IE) involving the aortic and tricuspid valves, ventricular septal defect (VSD), and complete atrioventricular block. He was admitted to a previous hospital with complaints of fever and neck pain, and he developed complete atrioventricular block during the course of his illness. An echocardiogram revealed severe aortic regurgitation, aortic valve vegetations, and a ventricular septal defect. He was then transferred to our hospital, and he underwent emergent surgery. The aortic valve cusps were calcified and thick, with significant cusp destruction. The vegetations partly extended to the subvalvular area of the right and non-coronary cusp. The vegetations also extended from the atrial septum to the tricuspid valve septal leaflet and perimembranous VSD. Ventricular septal reconstruction using the sandwich technique with two bovine pericardial patches, aortic valve replacement, and tricuspid valve replacement were performed. Postoperatively, he received antibiotic therapy for six weeks and was discharged from our hospital after the implantation of a cardiac resynchronization therapy pacemaker. Echocardiography showed no residual shunts. Our case suggests that the sandwich technique can be a useful method of septal reconstruction for IE with extensive destruction of the ventricular septum.