[Scimitar syndrome; report of a case].

A 17-year-old woman with scimitar syndrome without an atrial septal defect was operated by intra-cardiac conduit repair. Computed tomography (CT) and magnetic resonance imaging (MRI) showed resolution images of anatomical findings of scimitar vein. Surgical procedures for the scimitar syndrome have varied according to the anatomic features presented in each case. The detection of precise anatomy of scimitar syndrome is important for determining the appropriate surgical procedure. Images of 3-dimensional (3-D) CT and MRI of scimitar syndrome were demonstrated.

Newly developed left main coronary artery lesion after coronary stenting.

Percutaneous transluminal coronary stenting is a proven nonoperative method of direct myocardial revascularization. We encountered a case of iatrogenic significant subacute left main coronary artery stenosis in a patient who had undergone prior percutaneous transluminal coronary artery stenting of the left anterior descending (LAD) artery. Coronary artery bypass grafting was performed. To our knowledge, this is the first report of a surgical case of left main coronary artery stenosis worsened by changes secondary to earlier coronary stenting in the mid portion of left descending coronary artery in Japan.

[A case of protruding atherosclerotic plaque with mobile projections in the sino-tubular junction].

A 66-year-old woman who received maintenance hemodialysis for the last 14 years was referred to our hospital due to the sudden onset of ischemic stroke and systemic emboli. Transthoracic echocardiography before surgery showed large protruding masses measuring 2 cm which projected into the lumen of the aortic root. The plaque originated on a wide base of the posterior aspect of the ascending aorta at its junction with the sinus of Valsalva. The mobile plaque was surgically removed by endarterectomy from the posterior wall of sino-tubular junction under cardiopulmonary bypass. Pathologic examination of the masses removed at the time of surgery showed that they were atherosclerotic plaque with superimposed thrombi. During operation, transesophageal echocardiography appears to be useful for the rapid detection of a protruding aortic atheroma especially in the initial period of cardiopulmonary bypass until aortic cross clamp.

Recurrent aortic valve endocarditis caused by Gemella morbillorum--report of a case and review of the literature.

Gemella morbillorum (G. morbillorum) is part of the commensal flora of the oropharynx and intestinal tract, and on rare occasions causes infective endocarditis. A 55-year-old man with massive aortic regurgitation caused by recurrent infective endocarditis with G. morbillorum had a history of prior endocarditis caused by alpha-hemolytic streptococcus and multiple antibiotic allergies 5 years prior, and was successfully treated by aortic valve replacement. Almost all the reported cases of endocarditis caused by G. morbillorum have been bacteriologically cured with antibiotics and this is the first reported case of recurrent endocarditis caused by G. morbillorum in which the initial infection was bacteriologically cured by antibiotics and the secondary infection treated with valve replacement. This organism can be one of the causes of infective endocarditis and prompt surgical repair is mandatory if the infection is refractory or there is progression of congestive heart failure under antibiotic cover.

[Surgical treatment for aortic aneurysms associated with coronary artery disease-selection of bypass conduit].

Graft replacement for arch aneurysms and concomitant coronary artery bypass grafting (CABG) were performed in four consecutive patients over a three-year period between March 1995 and October 1998. The etiology of the aneurysms was atherosclerosis in all the patients. One early death as a result of a cerebellar infarction occurred on the 74th postoperative day. In all cases, respiratory failure frequently occurred after surgery. In a recent case, the internal mammary artery was used as a graft conduit to the left anterior descending artery (LAD). Both artery and vein grafts were anastomosed to coronary arteries during the initial core cooling. Selective cerebral perfusion was carried out during the reconstruction of the transverse aortic arch and its branches. The left subclavian artery was anastomosed first to secure perfusion to the LAD. To achieve sufficient myocardial protection and obtain good postoperative hemodynamics, CABG was simultaneously performed at the time of aortic aneurysm repair in cases complicated with coronary artery disease.

[Surgical treatment for chronic pulmonary thromboembolism in a patient with protein C deficiency].

Medical therapy for chronic pulmonary thromboembolism is limited, and surgical treatment has become more frequent recently. We have performed pulmonary thromboendarterectomy on a patient with chronic pulmonary thromboembolism accompanied by protein C deficiency. The patient was a woman aged 68 years who had protein C deficiency. The preoperative condition was New York Heart Association functional class IV. Hypoxemia, marked pulmonary hypertension, and low cardiac output were observed. After a median sternotomy, moderate hypothermia was induced using a cardiopulmonary bypass, and thromboendarterectomy in the pulmonary artery was performed. The arterial blood oxygen concentration improved, and the mean pulmonary pressure decreased. The cardiac output also increased, and New York Heart Association functional class improved to I. Pulmonary thromboendarterectomy under cardiopulmonary bypass was effective for chronic pulmonary thromboembolism accompanied by protein C deficiency.

Papillary fibroelastoma in association with thrombosis on a mechanical valve.

A patient with a thrombosed mechanical valve underwent valve re-replacement during which a tumor of the left ventricular outflow tract with the typical macroscopic and microscopic characteristics of a papillary fibroelastoma was successfully removed surgically. The 60-year-old woman had undergone isolated mitral valve replacement with a St Jude Medical 29-mm valve for mitral regurgitation 15 years ago. The present admission was for investigation of dyspnea on exertion. Two-dimensional transthoracic echocardiography demonstrated a posteroseptal, pedunculated mass, measuring 1.3x1.0 cm, in the outflow tract of the left ventricle, mild mitral regurgitation and slight aortic stenosis.

Revascularization of femoropopliteal artery occlusion in Werner's syndrome. Report of a case and review of surgical literature in cardiovascular lesions.

A patient with Werner's syndrome suffering from a chronic ulcer on the right ankle joint underwent femoropopliteal bypass and patch angioplasty combined with endarterectomy of the distal popliteal artery. Postoperative angiography showed satisfactory graft patency and distal run-off, and the ulcer improved. Femoropopliteal occlusive disease in Werner's syndrome tends to have poor run-off and the internal diameter of the popliteal artery with diffuse arteriosclerotic lesion is often too small to facilitate distal anastomosis. Therefore, a conventional bypass procedure is not always effective and an aggressive attitude is essential to obtain sufficient ankle blood pressure and improve the leg ulcer in Werner' syndrome.

Sudden deterioration of aortic regurgitation due to rupture of a raphal cord on the conjoined cusp.

A 57-year-old man was admitted to hospital for acute myocardial infarction associated with mild aortic regurgitation, which was successfully treated by intracoronary thrombolysis. Twenty-four days later, he suffered from another chest pain attack without any electrocardiographic ST-T changes. The coronary angiogram did not show any significant lesions, but the aortic root angiogram showed massive aortic regurgitation. Surgery revealed a bicuspid aortic valve with a conjoined cusp that had a fenestrated raphe torn away from the aortic wall and prolapsing into the left ventricle. The aortic valve was successfully replaced with a St Jude Medical mechanical valve prosthesis. The pathological significance of the intact raphal cord and the rupture remains an unsolved problem. This is the first reported case in which an increase of aortic regurgitation due to a ruptured raphal cord supporting the conjoined cusp was confirmed by a serial root angiogram.

Unusual complications in an inflammatory abdominal aortic aneurysm.

An unusual case of an inflammatory abdominal aortic aneurysm (IAAA) associated with coronary aneurysms and pathological fracture of the adjacent lumbar vertebrae. The associated coronary lesions in cases of IAAA are usually occlusions. In the present case, it was concluded that a possible cause of the coronary aneurysm was coronary arteritis and the etiology of the pathological fracture of the lumbar vertebrae was occlusion of the lumbar penetrating arteries due to vasculitis resulting in aseptic necrosis. Inflammatory AAA can be associated with aneurysms in addition to occlusive disease in systemic arteries. The preoperative evaluation of systemic arterial lesions and the function of systemic organs is essential.

[Post aortic left innominate vein in a patient with a surgically corrected double outlet right ventricle].

We report the presence of a post aortic left innominate vein (PALIV) in a patient with a surgically corrected double outlet right ventricle. A 30-year-old male was admitted to our hospital with exertional dyspnea. The patient had undergone right ventricular outflow tract reconstruction and closure of ventricular septal defect at the age of 14. Echocardiography and cardiac catheterization showed severe pulmonary regurgitation and a residual ventricular septal shunt. After resternotomy, right ventricular outflow tract reconstruction and residual shunt closure were performed. During the operation, the left innominate vein was not found in front of the aorta. Postoperative cardiac catheterization and computed tomography showed that the left innominate vein was positioned behind the ascending aorta draining to the superior caval vein.

Multiple aortocaval fistulas associated with a ruptured abdominal aneurysm in a patient with Ehlers-Danlos syndrome.

Aortocaval fistula (ACF) is a rare complication of spontaneous abdominal aortic aneurysm (AAA) rupture, with an incidence of 2-4%. A unique case of ruptured AAA complicated by multiple aortovenous fistulas involving the inferior vena cava and left internal iliac vein is presented, and is the first published report of a patient with Ehlers-Danlos syndrome undergoing surgical treatment for an ACF.

[Two cases of thoracic aneurysm with aberrant origin of the aortic branches: diagnosis and strategy].

We present two cases of thoracic aortic aneurysms with anomalous origin of the aortic arch branches. One was a 72-year-old female with a ruptured descending thoracic aneurysm and aberrant origin of the right subclavian artery. The other was a 64-year-old male with a saccular distal arch aneurysm and aberrant origin of the left vertebral artery. Preoperative examinations included angiography, computed tomography (CT), three dimensional enhanced CT (3DCT), digital subtraction angiography (DSA), and magnetic resonance imaging (MRI). Understanding the structure of neck vessels is important in deciding where to clamp or to reconstruct in surgical repair of the aortic arch. 3DCT was the most useful examination for this understanding.

[Coronary artery bypass grafting in an octogenarian with chronic myelomonocytic leukemia].

Chronic myelomonocytic leukemia is a disease of the elderly. It tends to have a variable clinical course, as the patient's state is immunologically dysunctional. There has been reluctance to perform open cardiac procedures because of concern about early postoperative sepsis leading to death. A 84-year-old man was admitted for the management of effort angina. PTCA was performed twice. He had left nephrectomy for Grawitz tumor nine years ago and additionally, he had been diagnosed as having chronic myelomonocytic leukemia since the next year. Preoperative laboratory assessment revealed that the total white blood cell counts were 2500 with 25 per-cent of granulocytes, a hematocrit of 31.1%, and platelet counts were 10.0 x 10(4). At the night of the treatment of his granulocytopenia with injection of granulocyte stimulating factor, he complained of continuous anterior chest pain with ST depression on ECG. Emergency single CABG was performed using a saphenous vein graft under the diagnosis of impending myocardial infarction. Postoperative course was uneventful. This is the first case report of CABG in octogenarian with chronic myelomonocytic leukemia in the world.

[A case report of aortic valvuloplasty by rasping technique for aortic stenosis with small annulus simultaneously performed with mitral valve replacement].

The patient was a 48-year-old woman with aortic stenosis and regurgitation and mitral stenosis. Preoperative cardiac catheterization revealed LV-Ao pressure gradient of 30 mmHg and regurgitation of Sellers III. The aortic annulus was measured less than 19 mm. As operative findings, the aortic annulus seemed to be too small to be replaced with 19 mm prosthetic valve. Aortic valvuloplasty (AVP) with rasping technique was performed for the aortic valve and valve replacement was carried out for the mitral valve. After aortic declamping and occurring her beat, the transesophageal echocardiographic evaluation for AVP was effective. Postoperative course was uneventful. Postoperative cardiac catheterization have shown decreased transvalvular pressure gradient up to 10 mmHg and aortic regurgitation of Sellers I.

[A case of video-assisted thoracoscopic surgery for patent ductus arteriosus under transesophageal echocardiography].

A six-year-old boy with patent ductus arteriosus was successfully treated by thoracoscopic surgery under transesophageal echocardiography, by which interruption of the ductal flow was confirmed. Postoperative course was uneventful and the patient was discharged on the 3rd postoperative day. The transesophageal echocardiography was effective to confirm disappearance of the flow in the operating room real-time. Video-assisted endoscopic surgery have reduced operative trauma in adult thoracic and general surgery, and can be safely applied to pediatric patients with patent ductus arteriosus. This technique may be an effective addition to the staged management of more complex forms of congenital heart disease.

[Aortic valve replacement due to Libman-Sacks endocarditis combined with infectious endocarditis].

A 40-year-old female had a history of fever, arthralgia, proteinuria, and dyspnea on effort twenty years ago, and was diagnosed as SLE, renal failure, and aortic regurgitation. She also suffered from pyelonephritis and sepsis due to the infection of E. coli. Preoperative examination revealed non-active phase of SLE. Echocardiography and aortography showed massive aortic regurgitation and operation was recommended. Operative findings showed fresh vegetation on the aortic leaflets, and aortic valve replacement (Tekna-Edwards 19 mm) was performed. Histological findings of the vegetation showed Libman-Sacks endocarditis and infectious endocarditis. Predonisolone was infused intravenously to prevent the acute deterioration of SLE after the operation. She was discharged from the hospital three weeks after the operation.