We report on a rare case of Marfan syndrome with giant dissecting descending thoracic and abdominal aortic aneurysms associated with poor left ventricular function and severe mitral regurgitation. Before the anesthetic induction, a partial extra-corporeal circulation was established to prevent a collapse of the circulation. Descending aortic graft replacement and following abdominal aortic graft replacement were performed safely using the partial extra-corporeal circulation to relief the afterload for the severely deteriorated left ventricle with severe mitral regurgitation. Intra-aortic balloon pumping was also promptly used to assist the poor circulation in the postoperative period. Despite the admission to a specialized institute, he died from irreversible heart failure with a developing renal failure. Even for a difficult patient with Marfan syndrome with severe left ventricular dysfunction and mitral regurgitation, graft replacement was feasible with meticulous perioperative circulatory management using partial extra-corporeal circulation and intra-aortic balloon pumping. However, a prompt registration for heart transplantation and an aortic surgery concomitant with implantation of left ventricular assisted device should have been considered to save the patient.

An 84-year-old man with a thoracic aortic aneurysm underwent total arch replacement with selective antegrade cerebral perfusion. Immediately after the operation, respiratory distress and hypotension developed and Chest X-ray films and computed tomography showed bilateral lung edema. Echocardiography showed a small, underfilled left ventricle, but with preserved systolic function. We suspected transfusion-related acute lung injury (TRALI), and started sivelestat and steroid pulse therapy. His respiratory condition gradually improved, and he was discharged on postoperative day 78. The diagnosis of TRALI was confirmed by positive test results of an HLA class I antibody in the transfused fresh frozen plasma and T- and B-cells of the patient. TRALI should be considered as a cause of acute lung injury after surgery with blood transfusion.

A 61-year-old man fell into out-of hospital cardiopulmonary arrest due to rupture of an abdominal aortic aneurysm, and was resuscitated onsite. On arrival at the emergency room, a fusiform type abdominal aortic aneurysm and massive hematoma in the retro-peritoneal space were detected by ultrasonography. Quickly, an aortic occlusion balloon catheter was placed at the proximal site of abdominal aorta through the left brachial artery, and then graft replacement of the aneurysm was carried out. The inferior mesenteric artery was occluded, and was not reconstructed. Five hours after the operation, left hemi-colectomy was carried out for ischemic necrosis of the descending to sigmoid colon. Although he was complicated by multiple organ failure; renal failure, liver dysfunction, severe infection, and brain infarction, he survived without a fatal disability. A rare case with ruptured abdominal aortic aneurysm who fell into cardiopulmonary arrest outside the hospital but survived after bowel necrosis and multiple organ failure is reported.

The elephant trunk procedure is used to close the false lumen of the distal aorta in the surgical treatment for aortic dissection. We examined the state of the false lumen thrombus and measured the diameter of the aortic dissection, using postoperative digital subtraction angiography and computed tomographic scanning. We performed the elephant trunk procedure in 24 cases in the period, between January 1995 to December 1999. Total aortic arch replacement was performed in Stanford type A dissection, and descending aorta replacement was performed in Stanford type B dissection. In all patients, thrombotic closure around the elephant trunk graft was confirmed. Thromboexclusion of the false lumen of the descending aorta was observed in 18 cases (75.0%). The secondary operation may be unnecessary, because there was a tendency towards reduction of the diameter of dissecting aorta. These data revealed that this procedure was effective. In 6 cases (25.0%), residual dissection was recognized in the thoracoabdominal aorta, but there was no case of expansion requiring further operation. Nevertheless, careful follow-up is necessary, because aneurysms could expand in the future.

A 59-year-old man presented with severe abdominal pain. CT scan showed a type A aortic dissection and pericardial effusion. As cardiac tamponade was present, emergency total arch replacement was performed. Because of his symptom, we added an exploratory laparotomy, which revealed intestinal necrosis. Therefore, necrotic intestine 4.5m in length was resected. After intensive care, he began oral feeding on the 25th day and was discharged on the 76th day postoperatively.

A 52-year-old man who had liver cirrhosis sufferred ruptured thoraco-abdominal aortic aneurysm. This patient was classified as having Child's class B liver cirrhosis preoperatively. The thoracoabdominal aorta was successfully replaced with reconstruction of the renal arteries, superior mesenteric artery, celiac artery, and 10th intercostal artery. Omentopexy was added. As persistent ascites continued postoperatively, peritoneovenous shunting was performed on the 29th postoperative day. Ascites disappeared and 20 days later the patient was discharged from hospital and has been well for two years.

Ten patients, aged 3 to 43 years, with the tetralogy of Fallot underwent in situ pulmonary valve replacement (PVR) 13 times. The implanted valves were a St. Jude Medical prosthesis (3 times) and a bioprosthetic valve (10 times). In 5 patients PVR was performed at the time of radical repair and in the remaining 5 patients PVR was performed after radical repair. Three patients underwent re-PVR at 6 to 13 years after the first PVR. There was one operative death in re-PVR 14 years after the first PVR and one patient died from congestive heart failure 4 years after PVR. In the patients with the tetralogy of Fallot, the rate of PVR in those who had undergone open Brock's operation were significantly higher than that of the patients without open Brock's operation (p<0.05). Actuarial survival rates at 5 years and 10 years were 88.9% and 88.9%, respectively. Rates of freedom from reoperation at 5 years and 10 years were 88.9% and 59.3%, respectively. Although the early operative results are satisfactory, re-PVR is mandatory in the future. Thus the indications of PVR should be considered carefully.

A 64-year-old woman with dyspnea on exertion was referred to our hospital. CT revealed type B aortic dissection with 7cm of aneurysm including a thrombus in the false lumen at the distal aortic arch. Four intimal tears at the distal aortic arch were closed directly during hypothermic circulatory arrest, and the descending thoracic aorta was tailored without a prosthetic graft after fixation of the dissecting adventitia to the intima at the distal portion of the false lumen. The postoperative course was uneventful and this patient was discharged on the 22nd postoperative day. Three years after surgery, the postoperative CT revealed no evidence of dilatation of the descending thoracic aorta as far as the abdominal aorta although the dissection of thoracoabdominal aorta remained. This technique is effective as an surgical option for chronic type B aortic dissection to minimize operative stress and complications.

We present a rare case of acute type A dissection which developed compression of the true lumen after starting cardiopulmonary bypass (CPB) with femoral arterial return. In this case, the entry was located in the proximal descending thoracic aorta, and the dissection expanded up to the ascending aorta in a retrograde direction. After starting CPB, the false lumen suddenly enlarged and the true lumen was compressed. We observed those changes by intraoperative transesophageal echocardiography, so the perfusion was stopped immediately. A long arterial cannula (Wessex) was inserted from the left ventricular apex with the tip of the cannula remaining in the true lumen of the ascending aorta, and antegrade perfusion was restarted. After that we could maintain adequate extracorporeal perfusion and the replacement of the total aortic arch was completed uneventfully.

The case presented is a 76-year-old woman with a ruptured abdominal aortic aneurysm. We tried to pass a Fogarty balloon catheter from the left subclavian artery for proximal occlusion of the ruptured aneurysm but failed to inset the balloon into the descending aorta. Although the aneurysm was safely replaced with a gelatine coated dacron graft, she developed cerebral embolism and never regained consciousness and died two months later. Balloon insertion through the subclavian artery may cause complication through dislodgement of atheromatous plaque and may induce cerebral embolism.