A 79 year-old woman was given a diagnosis of acute myocardial infarction and was immediately transferred to our hospital by a helicopter. Cardiologists successfully revascularized the occluded left anterior descending artery which was considered to be the care of this case. After that, they detected a large ventricular septal perforation by transthoracic echocardiography. We performed repair of the ventricular septal perforation 4 days later, with a modified infarct exclusion technique. Residual shunt flow was not seen by echocardiography after the operation. This patient recovered uneventfully and was discharged on postoperative day 55.

We encountered a rare case of a 75-year-old woman who fell into right ventricular failure and shock with a comparatively rapid course due to a huge primary right atrial angiosarcoma occupying the right atrium. An emergency surgical excision of the tumor was performed and the right atrium was reconstructed with an EPTFE patch under cardiopulmonary bypass. On account of the positive margin, postoperative radiotherapy was added. There was no local recurrence, but adjuvant chemotherapy was performed for multiple lung and liver metastases 14 months after surgery. Primary cardiac angiosarcomas are extremely rare and have dismal prognoses. Although a complete surgical resection is the cornerstone of treatment, multidisciplinary therapy may improve patient outcomes.

Background: Everolimus is a molecularly targeted drug for renal cell carcinoma. It is also approved for pancreatic neuroendocrine tumors ("PNET") and tuberous sclerosis complex ("TSC") in Japan and frequently associated with stomatitis, one of the most common adverse reactions. However, the mechanism of the onset of stomatitis has not been elucidated, and no reports have been published on appropriate prophylaxis against everolimus-induced stomatitis. Method: In the Department of Urology, Yamagata University Hospital, gargling with sodium azulene sulfonate has been used prophylactically since October 2010, when oral treatment with everolimus was first administered at our hospital. In this study, the preventive effect of gargling with sodium azulene sulfonate on stomatitis was evaluated in 21 patients receiving everolimus. Result: The incidence of stomatitis was 53.3% (Grade 3/4: 0%) in the intervention group and 83.3% (Grade 3/4: 16.7%) in the non-intervention group; patients treated in October 2010 or earlier. The grade of severity was significantly lower in the intervention group (Median grade: 1 vs. 2, p<0.05; Mann-Whitney’s U test). However, there was no difference in the time of onset between the two groups. Discussion: Sodium azulene sulfonate effectively prevented stomatitis probably because it not only has anti-inflammatory and mucosal protective effects, but also promotes wound healing. Further detailed analysis will be required in more patients.

A 72-year-old woman underwent a double aortic valve replacement with the Freestyle aortic bioprosthesis and subcoronary implantation with the Mosaic mitral bioprosthesis because of rheumatic multivalvular heart disease in 2000. During her annual follow-up, her Sinotubular junction was observed to have gradually increased in diameter on echocardiography and computed tomography. Therefore, 9 years after surgery we performed a reoperation for severe aortic regurgitation. Intraoperatively, the stentless bioprosthesis was found to be structurally intact. We believe that the dilation of the Sinotubular junction associated with a stentless bioprosthesis in the subcoronary position have caused her severe aortic regurgitation.

A 6-year-old boy who had been found to have hypertrophic obstructive cardiomyopathy presented with severely limited symptoms of heart failure due to progressive left ventricular outflow obstruction. Cardiac catheterization revealed the peak systolic pressure gradient of 87mmHg at left ventricular outflow, and systolic anterior motion of the anterior mitral leaflet with concomitant mitral regurgitation was observed by echocardiography. Transaortic septal myectomy was performed using transesophageal echocardiography guidance before, during and after surgery. Although the patient needed permanent pacemaker implantation for postoperative complete heart block, the procedure reduced the left ventricular outflow obstruction and relieved his symptoms.

A baby girl with a low birth weight was given a diagnosis of congenital bicuspid aortic stenosis and mitral valve prolapse. At the age of 40 days, she underwent balloon aortic valvotomy, but significant aortic regurgitation appeared afterwards. Another surgical intervention became necessary by the age of 20 months (weight, 5.7 kg), because of intractable heart failure mostly caused by exacerbated mitral regurgitation. We performed a leaflet extension valvuloplasty for the small bicuspid aortic valve using an autologous pericardium treated by glutaraldehyde. The mitral valve was replaced with an ATS-16AP valve. Although her postoperative course was complicated with mitral paravalvular leakage and poor left ventricular function, she was discharged from hospital 6 months post operatevely. Leaflet extension valvuloplasty is a surgical option for infants with a small aortic annulus, but the procedure could be the only solution in cases when Konno or Ross techniques are not suitable.

A 78-year-old woman underwent mitral valve replacement (MVR) with bioprosthesis in 1984. By 1997 the valve had become dysfunctional and was replaced with a Mosaic valve. Dyspnea on exertion occurred in 2005 and a systolic murmur was detected at that time. Echocardiography revealed severe mitral regurgitation (MR). The mitral valve was replaced for the third time. The explanted valve showed commissural dehiscence at the stent position and calcified leaflets. The mitral valve of a 70-year-old man was replaced with a bioprosthesis in 1986, and again with a Mosaic valve in 1997 because the original bioprosthesis had become dysfunctional. Seven years later, a systolic murmur appeared and echocardiography revealed severe MR. The valve was replaced for the third time. A leaflet tear was found in the removed valve. The Mosaic valve is a third generation porcine bioprosthesis that reportedly has excellent long-term durability. However, in these cases, the Mosaic valves deteriorated prematurely, and no obvious causes of this early structural deterioration could be identified. Continued long-term follow up is necessary, and the possibility of premature deterioration should be considered when selecting bioprostheses.

Vasculo-Behçet disease (VBD) is a special type of Behçet disease (BD) involving some vascular disorders like aneurysmal formation, arterial occlusion, and venous thrombosis in various vessels. VBD has a poor prognosis due to aneurysmal rupture or recurrence of vascular disorders despite optimal treatment. However, definite diagnosis in BD is made on the basis of clinical features, and early diagnosis is difficult. We report 2 patients whose first clinical symptoms were femoral-pseudoaneurysms. They received a diagnosis of VBD after surgery. The first patient was a 69-year-old man, who underwent autologous-vein patch closure of a perforated region in the left femoral artery. One year later, he had a pseudoaneurysm of the right profunda femoris artery, which was ligated. The second patient was a 51-year-old man, who underwent the interposition of the saphenous vein for defective artery due to left superficial femoral-pseudoaneurysm.

A cyanotic baby boy was given a diagnosis of single right ventricle, double outlet right ventricle, hypoplastic aortic arch, mitral atresia, atrial septal defect and pulmonary-ductus-descending aorta trunk. On day 4, extended aortic arch anastomosis and pulmonary artery banding were undertaken. At age 70 days, severe cyanosis and respiratory distress appeared and advanced rapidly. Angiography revealed critical subaortic stenosis and pulmonary hypertension, and the patient required urgent Damus-Kaye-Stansel anastomosis with concomitant right modified Blalock-Taussig shunt. Patients with single ventricle and hypoplastic aortic arch are a high-risk subgroup of progressive subaortic stenosis after initial pulmonary artery banding, and therefore need careful observation and may require early relief of subaortic stenosis.

Supravalvular aortic stenosis is a rare obstructive lesion of the left ventricular outflow tract localized at the level of sinotubular junction. It has been recognized that supravalvular stenosis may occur as a part of Williams syndrome and is sometimes complicated by obstruction of the left main coronary artery. We successfully performed single patch augmentation for supravalvular aortic stenosis and left coronary ostial stenosis with concomitant aortic valvotomy in a child without Williams syndrome. The patient had been followed as congenital bicuspid aortic valvular and supravalvular stenosis. At the age of 3 years, cardiac catheterization revealed an increased pressure gradient of 90mmHg at the left ventricular outflow and newly developed ostial stenosis of the left coronary artery. An oblique incision on the ascending aorta was made above the sinotubular junction and extended leftward onto the left main coronary artery, and this incision opened the fibrous ridge at the left coronary artery. After commissurotomy for the bicuspid valve, both the supravalvular and ostial stenosis were augmented with a single autologous pericardial patch treated by glutaraldehyde. The pressure gradient was significantly reduced and the ischemic left ventricular dysfunction was eliminated.