We present a case of juvenile type dermatomyositis and severe retinopathy. A 10-year-old girl presented with progressive weakness of proximal muscles, generalized rash, including heliotrope-type eyelid erythema, and bilateral visual disturbance. Laboratory data showed a markedly elevated serum creatine kinase and electromyography revealed a myogenic pathology. Funduscopic examination showed numerous cotton wool spots and macular edema. She developed massive rhabdomyolysis, generalized skin lesions, systemic edema, renal failure, and respiratory failure. After she received steroid pulse therapy, plasma exchange, and high-dose immunoglobulin, her general condition and visual symptoms improved. She remained well when we followed up her condition 5 years after the discharge.

Drug refractory atrial flutter (AF) with secundum atrial septal defect (ASD) and pulmonary valvular stenosis was treated by surgical correction and intraoperative radiofrequency (RF) current ablation. Supraventricular arrhythmia, especially AF, is frequently found in aged patients with ASD. Perioperative managements for this arrhythmia were difficult because of drug refractoriness. We performed this ablation combined with intracardiac corrections, and sinus rhythm has been maintained without any drugs for 18 months. This case indicated that RF current ablation during open-heart surgery is useful and safe method of treatment of AF.

A 64-year-old man with congenital factor V deficiency and hereditary spherocytosis was attending our hospital for type II diabetes and stage 4 diabetic nephropathy. Coronary angiography performed to assess chest pain revealed severe triple-vessel disease, including total occlusion of the right coronary artery. The patient required surgical coronary revascularization. In the preoperative examination, the activated partial thromboplastin time (APTT) and prothrombin time-international normalized ratio (PT-INR) were high (89.5 s and 1.95) and factor V activity was low (6% ; normal range, 70-135%). Hemodialysis was performed on the day of the operation, and 6 units of fresh frozen plasma (FFP) were administered, which reduced immediately the preoperative PT-INR to 1.33. We performed off-pump coronary artery bypass grafting (OPCAB) and perioperatively administered 6 units of FFP with 4 units of red blood cells (RBC) transfusion. The postoperative course of the patient was uneventful, and he was discharged on postoperative day 22. Here we report the case of a patient with a very rare disease of congenital factor V deficiency and hereditary spherocytosis complicated with stage 4 diabetic nephropathy who required OPCAB.

Objective : With clinical application in mind, we developed a method to measure the movement of a selected joint three-dimensionally as a conic domain (joint sinus cone). The method was applied to shoulder joint motion during daily living activities exercises in a group of hemiparetic poststroke patients. The results were compared to the exercise range of physically unimpaired persons. Methods : The subjects dressed in jackets and performed certain other tasks in a sitting position and the range of motion of the shoulder joint in three dimensions was measured using a 6-dimensional electromagnetic tracking system. Measurement results were analyzed with a plane display using a Lambert Azimuthal equal area. Results : This method was able to determine findings difficult to see with the naked eye, such as the narrow range of motion in a paralyzed shoulder joint and poor functionality also in the contralateral shoulder. Conclusion : The dressing measurement was achieved using magnetic measurement equipment. By displaying the results through a map projection it was possible to appreciate the whole range of the motion. Because this method facilitates the expression of complex joint exercise ranges, it will be useful in the analysis of daily behavior restrictions due to impairment.

We report an unusual case of a 71 year-old man who developed chronic consumption coagulopathy caused by an abdominal aortic aneurysm. He was diagnosed as having the dissecting aortic aneurysm (DeBakey type IIIa) and the abdominal aortic aneurysm in 1989, and had been attending to our hospital as an outpatient since then. He developed macrohematuria in March 1990. The laboratory data showed the decrease in platelet, fibrinogen, plasminogen and α₂ plasmin inhibitor and the increase in FDP. The bleeding tendency was controlled by the administration of gabexate mesilate and heparin, but the laboratory data revealed that consumption coagulopathy continued. The abdominal aortic aneurysm was successfully replaced with a prosthetic vascular graft in June 1992. Postoperative hematological findings revealed the improvement, and he discharged 32nd day and doing well after operation.

A 5-year-old boy with tricuspid atresia who underwent the Björk procedure died due to right atrial thrombus and left pulmonary embolus 37 days after operation. It is suggested that thromboembolism may be a frequent complication after the Björk procedure due to the turbulent blood flow at the right atrio-ventricular anastomosis and also due to congestive blood flow. Anticoagulation therapy seems to be essential for postoperative management.

A 6-year-old boy was admitted with infective endocarditis and aortic regurgitation. Clinical signs of infection were severe. The leukocyte count was 13, 100/μl and the C-reactive protein (CRP) was elevated to 17.2mg/dl. Blood culture was positive for Staphylococcus aureus. Echocardiography showed a vegetation 3mm in diameter on the aortic valve, and a perforation of the right coronary cusp with moderate aortic regurgitation. With antibiotic therapy, clinical signs and laboratory data of infection improved at an early stage. We decided to operate after his complete recovery from infection. Laboratory data normalized completely in 6 weeks, but echocardiography demonstrated aneurysmal change of the right coronary sinus and severe aortic regurgitation. The Ross operation was performed on the 44th day. At operation, it was noted that the non-coronary cusp was destroyed completely leaving only strings of fibrous tissue. A perforation of 3mm in diameter was also found on the right coronary cusp. There was a mural aneurysm near the right coronary orifice without abscess formation in the surrounding structure. A pulmonary autograft was transplanted to the aortic root after resection of the destroyed aortic cusps, aortic root and the mural aneurysm. The right ventricular outflow tract was reconstructed using an autologous pericardium as a posterior wall and the Monocusp ventricular outflow patch (MVOP) #22 as an anterior transannular patch. The postoperative course was uneventful. Postoperative echocardiography revealed no aortic regurgitation.

We report a case of syphilitic aortitis (SA) associated with severe right coronary ostial stenosis, aortic regurgitation (AR), and annuloaortic ectasia (AAE). A 48-year-old man presented to a regional hospital with easy fatigability and nocturnal dyspnea. Echocardiography revealed Seller's grade 3 AR. A computed tomography scan showed AAE, dilatation of the ascending aorta, and calcification of both coronary ostia. Coronary angiography demonstrated that the left coronary artery was intact ; however, the right coronary artery was obscure. Active syphilis was detected on routine blood tests on admission. Therefore, the patient was started on a course of ampicillin/sulbactam (ABPC/SBT). Subsequently, he underwent the Bentall procedure and coronary artery bypass grafting with the right internal thoracic artery. The intraoperative findings showed degeneration of the aorta and severe right coronary ostial stenosis. The pathological findings of the aortic wall and aortic valve were consistent with SA. The postoperative course was uneventful. The patient continued receiving ABPC/SBT for 3 weeks postoperatively, and was then switched to oral amoxicillin.

Background : Several studies have shown that Fontan circulation may lead to liver congestion and possible structural liver alteration. The aim of this study is to analyze the relationships between biochemical fibrosis markers and hemodynamic parameters in the long term after the Fontan operation.
Methods : The study enrolled 51 patients who underwent total cavopulmonary connection between March 1994 and July 2010. We analyzed the relationships between the 5 liver fibrosis markers (hyaluronic acid, retinol-binding protein, procollagen type III peptide, type IV collagen 7S, type IV collagen) and the 6 hemodynamic parameters (pulmonary artery pressure, pulmonary artery index, pulmonary vascular resistance, ejection fraction, atrioventricular valve regurgitation, cardiac index).
Results : Hyaluronic acid and type IV collagen 7S positively correlated with pulmonary artery pressure. Hyaluronic acid negatively correlated with ejection fraction, and type IV collagen 7S positively correlated with atrioventricular valve regurgitation in patients followed up for more than 8 years after Fontan completion. Pulmonary artery pressure was significantly higher in patients in whom type IV collagen 7S was elevated. Hyaluronic acid correlated with pulmonary vascular resistance (p=0.0035) and ejection fraction (p=0.014), as well as type IV collagen 7S with pulmonary artery pressure (p=0.0001) by multiple regression analysis.
Conclusion : Hyaluronic acid and type IV collagen 7S reflected the degree of hepatic congestion, and cardiac function, in the long term after the Fontan operation.

The cases of neonates and infants who underwent successful delayed sternal closure (DSC) using silastic rubber after open heart surgery were reviewed. The indication for DSC was cardiac dilatation with tamponade-like behavior upon attempted sternal closure in all. In 7 of 10 cases, DSC were possible within 4 days after operation. There were statistical decrease in heart rate (HR), left atrial pressure (LAP), cardiothoracic ratio (CTR) and inspiratory oxygen concentration of the respirator at the time of DSC compared to those in the early postoperative period. None of the patients had mediastinitis or other severe infection in the postoperative course. One patient each died of progressive pulmonary venous obstruction and of non-cardiac disease late postoperatively and 8 patients are long-term survivors. It is concluded that DSC is recommended whenever there is any hemodynamic deterioration due to attempted sternal closure following open heart surgery in infancy as it could be safely performed within 3 to 4 days after operation without any complication.