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.

Recurrence of seminoma in the scrotum after inguinal orchidectomy is extremely rare. Herein, we report the second case in the literature of recurrence of seminoma in the scrotum following inguinal orchidectomy for stage I seminoma.
Seminoma ; Recurrence ; Disease of scrotum ; Benign neoplasm of scrotum ; Orchiectomy

Hemolytic uremic syndrome (HUS) is a heterogeneous disorder characterized by hemolytic anemia, thrombocytopenia and renal failure that occurs predominantly in infants and young children. However, HUS in adults has also been described as a complication of various chemotherapy regimens with a relatively poor prognosis. Since cisplatin is now widely used for treatment of solid cancers, it is necessary to take into account the possibility of cisplatin-induced hemolytic uremic syndrome as a rare but potentially fatal side- effect. Herein, we describe our experience with a 67-year old woman being treated for a urothelial carcinoma of the bladder who suffered chemotherapy-induced HUS after a cisplatin-based regimen. Plasmapheresis was carried out five times; however, her serum platelets remained depressed, and she subsequently died. We conclude that there is a high risk potential for HUS in patients undergoing intensive chemotherapy for advanced-stage bladder cancer.
Hemolytic-Uremic Syndrome ; Chemotherapy-Oncologic Procedure ; Cisplatin ; Cancer of Bladder ; regimen

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 27-year-old woman was given a diagnosis of infectious endocarditis with severe tricuspid regurgitation. Despite adequate antibiotics therapy, her general condition did not improve, and moreover multiple pulmonary abscesses were detected by computed tomography. Therefore surgery was indicated. Surgery consisted of removal of vegetation and tricuspid valve plasty with autologous pericardial patch augmentation of the anterior leaflet. Tricuspid valve plasty was carried out without prosthetic materials. Her postoperative course was uneventful with only mild tricuspid regurgitation. One year after surgery, neither recurrence of infection nor worsening of tricuspid regurgitation was noted. This method could be a useful technique for young patients with severe infection.

We described a patient with free wall rupture followed by papillary muscle rupture due to acute myocardial infarction. A 69-year-old man was transferred complaining of transient unconsciousness. His clinical history, electrocardiogram, and chest CT showed myocardial infarction with free wall rupture indicated that several days had passed since the onset. Coronary angiography showed occlusion of the right coronary artery and severe stenosis of the left anterior descending artery. Since cardiac rupture was at inferior wall and hemorrhage wasn't active, repair of the rupture using fibrin glue and fibrin sheet and coronary artery bypass grafting to the left anterior descending artery was performed without cardiopulmonary bypass. On the 10th postoperative day, his arterial oxygen saturation suddenly deteriorated. Transesophageal echocardiography revealed papillary muscle rupture and severe mitral regurgitation. Emergency mitral valve replacement was performed. After two emergency operations, he gradually recovered and were discharged to home. In three months after discharge, he was admitted again due to congestive heart failure with left ventricular aneurysm at inferior wall and recovered in response of conservative treatment. Surgical experience of double rupture is rare. Based on this case, it may be necessary to perform reperfusion therapy toward even this case of recent myocardial infarction, to prevent papillary muscle rupture. It also may be better to use a patch on free wall rupture to prevent cardiac aneurysm.

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 20-year-old man was admitted to our hospital with persistent cough and dyspnea. He had bilateral distention of the jugular veins, and swollen lymph nodes were palpable in the right subclavicular region. Plain X-ray and computed tomography (CT) of the chest showed a solid soft tissue mass in the upper mediastinum, with leftward displacement of the trachea and complete obstruction of the superior vena cava. Mediastinal radiotherapy (1.8 Gy/day) and methylprednisolone (100 mg/day) were started immediately. Biopsy of the right subclavicular lymph nodes revealed metastatic seminoma. The patient was referred for chemotherapy, which was performed with a combination of cisplatin, bleomycin and etoposide (BEP). A partial response was observed after completion of 3 cycles of chemotherapy, but there was no further tumor shrinkage after additional salvage chemotherapy. The patient is being followed up on an outpatient basis and has been free of recurrence for 32 months after intensive treatment.

Cardiovascular manifestations of acromegaly include cardiomegaly and very often hypertension, coronary atherosclerosis, and diabetes. Primary valvular disease is less commonly observed. A 62-year-old woman had acromegaly associated with mitral regurgitation (MR) resulting from prolapse of the posterior mitral leaflet, which was successfully repaired. At the age of 57 years, the patient was admitted due to heart failure without valvular disease. Acromegaly was diagnosed and a pituitary tumor was removed surgically. At the age of 62, a heart murmur was found, and moderate to severe MR was diagnosed. MR was successfully corrected by quadrangular resection of the posterior leaflet, including the prolapsed portion, and prosthetic ring annuloplasty. Histological examination showed myxomatous degeneration. The patient recovered uneventfully. During the last 2 decades, only 21 surgical cases of valvular disease associated with acromegaly were reported in the literature; mitral valve lesions in 10 patients (all with regurgitation), aortic valve lesions in 10 patients (7 with regurgitation and 3 with stenosis), and one with combined lesions of mitral and aortic valves. Since histology did not show specific changes in many reports, it is still unclear whether valve lesions are caused by a high GH hormone level. Although mitral valve replacement was recommended in the 1990s due to the fragility of valvular rings and their apparatus, mitral repair was performed in 5 recent cases and no recurrence has been reported.

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.