[A case of Down syndrome with ventricular septal defect and hemodynamical Eisenmenger syndrome].

A 10 month-old infant with Down syndrome having ventricular septal defect and pulmonary hypertension performed cardiac catheterization, which resulted in a slight increase in the pulmonary vascular resistance from 10.8 to 11.3 unit.m2. Lung biopsy findings showed at most an early grade 2 Heath-Edwards classification, and an index of pulmonary vascular disease of 1.1, both of which indicated operability for total correction. He underwent surgical correction and the pulmonary arterial pressure significantly decrease. Although he suffered chylothorax in 5th postoperative day, he did not developed pulmonary hypertension crisis.

Aortobronchial fistula late after transverse arch replacement.

We report an unusual case of aortobronchial fistula late after transverse arch replacement caused by the remnant of a temporary bypass near the ascending aorta. In reconstructive surgery of the ascending aorta, antegrade perfusion is preferably performed through a side branch after completion of the distal anastomosis by some surgeons. This report suggests possible risk of a serious late complication unless the side branch is placed and tailored properly.

[Coronary artery bypass grafting with arterial grafts in the elderly patients].

One hundred six consecutive patients aged 75 years and over (mean age 77.1, 75-84) underwent coronary artery bypass. Controlled group is 287 patients undergoing CABG in 1996. The number of bypass graft, arterial graft, Ao-cross clamp time, CPB time, operation time in aged group and controlled group were as follows 3.3 and 3.3, 1.9 and 2.5 (p < 0.01), 106 and 104, 333 and 328. The number of anastomose per patients with LITA, SVG, GEA, RITA, IEA, RA, as follows 1.0 and 1.0, 1.5 and 0.5, 0.6 and 0.5, 0.1 and 0.14, 0.03 and 0.03, 0.2 and 3.3 SVG were used significantly high and RA were used low in aged group. Sequential bypass were under went 30.1% in aged group and 35.5% in controlled group. There was no operative death in elective operation, 5 (4.9%) was died in emergent operation. A favorable outcome may be expected when coronary bypass surgery with arterial graft is performed in 75 years and over aged group.

[Two cases of mitral stenosis associated with ball thrombus in the left atrium].

Two patients (55-year-old female and 77-year-old female) were operated on for mitral stenosis associated with left atrial ball thrombus. The first case had the episode of cerebral infarction and second case had syncopal attack. Both of them presented with systemic arterial embolism. After confirmation of the diagnosis by echocardiography, removal of ball thrombi, OMC and MVR were carried out urgently. Considering high risk of "hole in one sudden death" and multiple-episodes of systemic embolization, ball thrombus should be removed as urgent following confirmation of diagnosis.

Aneurysm of a branch of the subclavian artery with multiple arteriovenous fistulae.

A rare case is reported of an 83-year-old woman with an aneurysm of a branch of the subclavian artery with multiple arteriovenous fistulae. The patient was admitted to our hospital with a pulsatile mass in the supraclavicular space and a prominent continuous murmur which radiated to the anterior chest, right forearm and right neck. She first noticed a pulsatile 2 cm mass in 1972, 1 year following a subtotal gastrectomy. At that time, she had intravenous therapy through a right neck vein. In 1993, the mass became larger, and she developed a shunt murmur. Digital subtraction angiography (DSA) demonstrated an aneurysm of the right subclavian artery and an arteriovenous fistula between the right subclavian artery and vein. The right common carotid artery and right subclavian artery arose from the aortic arch separately. The aneurysm arose from a branch of the subclavian artery which may be the costocervical trunk. The 5 x 4 cm aneurysm was resected and the arteriovenous fistula was divided. On postoperative day 5, a new murmur was ausculated. A repeat DSA detected a new fistula between the axillary artery and vein. Reoperation was performed to ligate and divide the fistula. Pathological examination revealed an atherosclerotic aneurysm.

[Tricuspid valve replacement by right thoracotomy with minimal dissection in reoperations].

We report two cases of tricuspid valve replacement for tricuspid valve insufficiency as reoperations following mitral valve replacement through midline sternotomy. A right thoracotomy was used to approach the tricuspid valve. To avoid the risk of cardiac laceration, cardiopulmonary bypass was instituted after cannulation of the femoral artery and of superior vena cava through right atrium with balloon caval occlusion and inferior vena cava through the femoral vein with balloon caval occlusion. Without aortic cross clamping under mild hypothermia, right atriotomy was performed through adherent parietal pleura, pericardium, and right atrial wall without dissection. Tricuspid valve was replaced utilizing the bioprosthetic valve with good clinical results. These new measures were expeditiously carried out without dissection of the heart, which has been deemed to be the risk of reoperations.

[Histological study for endothelial seeded vascular prostheses-experimental study on caine].

Endothelial seeded graft has been investigated for a small-caliber vascular graft. In this report, we established endothelial lining onto ultrafiber graft by the filtration method and implanted it to caine abdominal aorta. The grafts were harvested in 1 to 4 months and examined endothelialization and subendothelial structure by light microscopy and electron microscopy. The cell counts of pre and post flush through the graft were calculated. pre:9.8 +/- 10(6)/ml post: 1.2 +/- 0.60 x 10(6)/ml trap ratio: 86.4 +/- 4.6%. The thickness of inner capsule of seeding part were significantly low than that of control part. But there were no significant difference of inner capsule of anastomosis between seeding part and control part. In the seeded part endothelial lining and well incorporation were observed by light microscopy. Electronmicroscopic study revealed that seeded endothelium had desmosome and secreted a basement membrane. In the control part there was only small endothelial inoculation and thrombus formation which caused stenosis of graft. The filtration method revealed the efficacy to seed on to vascular prosteses for long time period. The seeded graft had antithrobogenity and well incorporation.

[Surgical management of coronary artery fistula with tricuspid stenosis in a three-month-old infant].

This is a case report of a 3-month-old infant who had been suffering from progression of cardiac failure. Two dimensional echo-cardiography revealed a fistula between the right coronary artery to the right heart. He was operated upon using extracorporeal circulation. Symbas procedure was carried out to close the fistula. Upon opening the right atrium, there noted tricuspid stenosis (TS). Then, ASD was created to reduce the right atrial pressure to prevent right heart failure secondary to TS. He is doing well postoperatively.

[A case of anti-glomerular basement membrane antibody (AGBMA) nephritis associated with myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)].

A 68-year-old patient visited our hospital, because of progressive renal failure. Laboratory data were serum creatinine 13.3 mg/dl, BUN 74.3 mg/dl, AGBMA 134U, MPO-ANCA 37% and CIC 1.6 g/dl. Hemodialysis was performed on admission and renal biopsy was conducted. Though the histological findings showed fibrocellular crescents and global sclerosis in many glomeruli, renal function was gradually ameliorated by administration of prednisolone (40 mg/day) and cyclophosphamide (50 mg/day). After 3 weeks of therapy, hemodialysis was withdrawn temporarily. Because of the complication of pneumocystis carini, prednisolone and cyclophosphamide were discontinued, and maintenance hemodialysis was resumed. In spite of progressed histological findings, hemodialysis was withdrawn at least temporarily through the use of pharmacological therapy. Thus, the presence of ANCA may indicate a relatively good prognosis of AGBMA nephritis.

Perianastomotic findings in canine endothelial cell seeded grafts at 3 months.

Endothelial cell seeding is one of the methods commonly used to prepare an antithrombogenic surface for small caliber vascular prostheses. In this study, we investigated the perianastomotic tissue reaction of seeded grafts, using a canine model. Eight dogs were used to harvest endothelial cells (ECs) enzymatically from their external jugular veins, with one dog being excluded because of infection. ECs were cultured and seeded, using a rotation method, onto a small caliber prosthesis woven with ultrafine polyester fibers. Each graft was implanted into the carotid artery of its cell donor dog, with another implanted contralaterally as a non seeded control. Two of seven seeded grafts were patent at 3 months, while all controls were occluded. Histologic examination revealed a continuous lining of ECs in the patent grafts, but thickening of the intima at the anastomoses was also observed. The occluded seeded grafts had organized hard thrombi and thick fibrosis at the perianastomotic area, however, with few thrombi in the midportion. These observations suggest that the investigation of a small caliber prosthesis with the ultimate aim of producing better patency should focus not only on antithrombogenicity of the surface of the lumen, but also on the perianastomotic biologic reaction.

[A case of Fabry's disease associated with lupus nephritis].

A 36-year-old woman was hospitalized because of nephrotic syndrome. On admission, laboratory studies revealed total protein 5.9g/dl, total cholesterol 381mg/dl, urine protein 2-4g/day, C3 68mg/dl(90-185mg/dl) and the immunological tests showed that antinuclear factor, anti-DNA antibodies and the LE cell phenomenon were positive. Renal function was within normal range. After admission, renal biopsy was done. Light microscopic finding showed diffuse membranous glomerulonephritis, and vacuolization of epithelial cells. Immunofluorescent microscopic finding showed a granular specific staining for IgG, IgM, C3 and C1q along the capillary loops. Electron microscopic finding showed subepithelial and subendothelial dense deposits, and visceral epithelial cell cytoplasm containing osmiophilic multilamellar lipoid bodies. In the studies of the enzyme activities, the patient's fibroblast extract demonstrated a partial deficiency of alpha-galactosidase, and urine ceramide trihexoside was positive. But the patient's leukocyte extract did not demonstrate a deficiency of alpha-galactosidase. So Fabry's disease associated with lupus nephritis was diagnosed. It seems that the case of Fabry's disease which is an X-linked disorder caused by deficiency of the lysosomal enzyme alpha-galactosidase, associated with lupus nephritis, is extremely rare.

Esmarch's bandage technique in distal bypass surgery.

Esmarch's rubber bandage technique has been applied to 49 distal bypass surgeries in 46 patients during the past ten years. The primary and secondary patency rates at 5 years after femoro-tibial bypass surgery were 82% and 92%, respectively. This technique has the following advantages: (1) it minimizes surgical injury of the arterial wall because there is less dissection around the anastomotic site; (2) it decreases scar formation in the anastomotic area after surgery; (3) it maintains abundant muscular blood flow by preserving small branches to muscles, and (4) it provides a bloodless surgical field and easy handling for fine sutures without using vascular clamps. We consider that the avoidance of long circumferential dissection of the artery may play an important role in improving long-term patency in distal bypass surgery.

False aneurysm as a late complication of division of a patent ductus arteriosus.

A 58-year-old male patient who had a huge false aneurysm as a late sequela of division of patent ductus arteriosus was surgically managed with success. It is noteworthy that 24 years had elapsed from the initial operation until recognition of the aneurysm. The pathogenesis and method of the surgical treatment are discussed.