We present a 70-year-old woman who underwent a classic Blalock-Taussig shunt for tetralogy of Fallot (TOF), followed by intra-cardiac repair at the age of 25 years. She developed heart failure due to aortic regurgitation with aortic root dilatation and pulmonary regurgitation 45 years after the surgery. She was successfully treated with concomitant biventricular outflow tract reconstruction (aortic valve, ascending aorta, and pulmonary valve replacement). The treatment strategy for aortic regurgitation with aortic root dilatation after TOF repair is unclear. With a transient increase in the number of elderly patients who have undergone the classic Blalock-Taussig shunt as palliative surgery, the number of complex cases of both right and left ventricular outlet tract involvement will also increase. With patients' advanced age and situation of complex reoperation taken into consideration, aortic valve and ascending aorta replacement may be useful options for cases of aortic regurgitation and aortic root dilatation.

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.

An aortoesophageal fistula is a critical condition with high operative mortality. A case of aortoesophageal fistula following thoracic endovascular aneurysm repair is reported. The patient was a 72-year-old man complaining of dysphagia who underwent stent grafting for a saccular aneurysm of the descending aorta that was compressing the esophagus four months earlier. Endoscopic examination showed perforation of the aneurysm into the esophagus with severe stenosis. The aneurysmal sac was filled with thrombus. Aortography demonstrated a type I endoleak from the lesser curvature of the aortic arch, draining into the aneurysmal sac. The patient was afebrile with moderate elevation of C-reactive protein, and the white blood cell count was normal. The patient underwent closure of the aneurysmal entry with healthy aortic wall and replacement of the descending aortic aneurysm with a prosthetic graft. The graft was isolated from the fistula by an omental flap. The patient's postoperative course was uneventful. Computed tomography performed 4 years after the surgery showed shrinkage of the aneurysmal sac. The patient has had a healthy life for 9 years since the operation.

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.

Pulmonary hypertension persisted in a 57-year-old man after mitral and tricuspid valve replacement to treat mitral and tricuspid regurgitation. Heart failure gradually worsened after surgery. Pulmonary hypertension was initially considered as the major reason for the heart failure, and inhaled nitric oxide was administered. Thereafter, the heart failure improved and mechanical circulatory assist could have been avoided. We believe that inhaled nitric oxide is a less invasive and effective method for improving pulmonary hypertension and hemodynamics after mitral valve replacement.

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.

A 75-year-old man with underlying arteriosclerosis obliterans presented with acute heart failure secondary to rest pain of the right lower extremity. Echocardiogram showed severe mitral regurgitation, moderate tricuspid regurgitation and a low cardiac function (ejection fraction : 27%). Right toe gangrene developed in association with continuous acute heart failure. He underwent mitral valve replacement, tricuspid annuloplasty, right common femoral artery-posterior tibial artery bypass and amputation of the right toes in single-stage surgery. There were no major complications during his hospital stay. After surgery, his symptoms significantly improved.

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.

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.

In cases of stent-grafting for ruptured aneurysm, endoleak is a serious problem. We report 2 cases of ruptured aneurysms that were treated with endovascular stent-graft placement. Case 1: A 79-year-old woman had a ruptured thoracic aortic aneurysm that was treated with endovascular stent-grafting from the distal arch to the descending aorta. Although her infra-operative course was uneventful, she died suddenly the day after operation. Autopsy revealed re-rupture of the aneurysm due to endoleak from the proximal site. Case 2: An 84-year-old woman was treated with endovascular stent-grafting for ruptured abdominal aortic aneurysm. The stent-graft was inserted from the infra-renal abdominal aorta to the right common iliac artery with femoro-femoral crossover bypass placement. There was evidence of type II endoleak that occurred via the left internal iliac artery (IIA) and inferior mesenteric artery (IMA) 16 days after surgery. A CT scan performed 6 months after surgery revealed an increase in aneurysm size and persistent type II endoleak. Both embolization of the aneurysmal sac through the IMA and surgical ligation of the IMA failed, and endoleak from the IMA persisted. Re-rupture of the aneurysm occurred 10 months after initial surgery and emergency open surgery was performed. In stent-grafting for ruptured aneurysms, only the thrombus outside the graft resists the pressure caused by the endoleak. We conclude that endoleak after stent-grafting for ruptured aneurysm should be treated completely as soon as possible because of the risk of re-rupture.