Aortic dissection with multiple familial members is rare. It is commonly associated with Marfan syndrome. Several authors have reported familial aortic dissection without Marfan syndrome. We encountered 4 cases of aortic dissection in a family. The aortic dissection occurred in the mother and all of her children. No case had clinical manifestations of Marfan syndrome or other connective tissue disease. Histopathological examination of the aorta did not show cystic medial necrosis in 2 operated cases. Many members in the family had systemic arterial hypertension. The presence of multiple incidence of aortic dissection in one family suggests underlying connective tissue disease, irrespective of the absence of typical features of Marfan syndrome. Therefore we propose that other close relatives as well as the members with aortic dissection should be followed-up in the same way used for families with typical connective tissue disease like Marfan syndrome.

Anticoagulation management in cardiac surgery can be difficult in patients with heparin-induced thrombocytopenia (HIT). We report a patient who underwent reoperation of cardiopulmonary bypass (CPB) using argatroban in combination with nafamostat mesilate. A bolus of 0.25 mg/kg argatroban was administered, followed by continuous infusion of 5-10 μg/kg/min argatroban and 100 mg/h nafamostat mesilate. No complications such as thrombosis were observed during either CPB or the perioperative period. Although we used argatroban and nafamostat mesilate, which has a shorter half-life than argatroban, the anticoagulant effect was prolonged, and the patient had an uneventful postoperative course despite requiring substantial blood transfusion.

In 1984, a 67-year-old man had aortic valve replacement surgery for aortic regurgitation; he returned with chest pain on May 15, 2003. Emergency coronary angiography was performed because electrocardiogram revealed ST segment depression in leads V₄ to V₆. However, coronary angiography, echocardiogram and chest computed tomography finding were normal. Therefore the patient was discharged the following day. However, he was re-admitted for chest pain, followed by loss of consciousness 4 days after his initial release. Echocardiogram and chest computed tomography revealed perforation in the lateral wall of his left ventricle (LV) and a “blow-out” type rupture was diagnosed. The patient fell into cardiogenetic shock in the emergency room, and emergency left ventricular free wall rupture (LVFWR) surgical repair was performed under percutaneous cardiopulmonary support (PCPS). A round perforation measuring about 10mm in diameter was observed in the lateral LV wall along the course of LCx # 12. The perforation was closed using Teflon strip reinforced mattress sutures. The hemostasis was reinforced with fibrin glue sheet (TachoComb) and polyglygolic acid surgical mesh (Dexon Mesh), with fibrin glue extensively applied. He was discharged on July 17, 2003 without major complications. In this case, the precise cause that led to LVFWR was unknown. Emergency PCPS insertion enabled the LVFWR surgical repair and extensive adhesion due to the previous AVR prevented the massive bleeding to pericardial cavity and the catastrophic hemodynamic deterioration: both factors positively contributed to patient recovery.

A 66-year-old woman complained of dyspnea due to congestive heart failure, and was given a diagnosis of severe aortic insufficiency. Antiphospholipid syndrome and idiopathic thrombocytopenic purpura (ITP) had been diagnosed with 9 years previously. We planned preoperative plasma exchange and steroid pulse infusion to reduce the level of auto-antibodies for phospholipids. The aortic valve replacement was performed safely. Anticoagulant therapy with low molecular weight heparin and oral steroid therapy was administered after the operation to avoid thrombosis or bleeding. The patient's postoperative course was stable. She was discharged without any complication. In conclusion, preoperative plasma exchange and steroid pulse infusion, postoperative anticoagulant therapy and oral steroids resulted in a favorable outcome in a case of heart surgery for a patient with antiphospholipid syndrome.

The patient was 62-year-old woman was brought to the emergency room with chest pain and dyspnea. Computed tomography revealed a hematoma around the ascending aorta, a notch in the aortic wall, pericardial effusion and a hematoma around the pulmonary artery. We diagnosed early thrombotic type of acute aortic dissection. An ascending aorta replacement was performed via median sternotomy under hypothermic circulatory arrest. Upon operation, there was a 1.0 cm intimal tear just above the left main trunk and there was no specific evidence of aortic dissection. So we diagnosed spontaneous aortic rapture. Her postoperative course was uneventful and she was discharged 18 days after surgery.

A 52-year-old man underwent a medical examination, including abdominal computed tomography (CT). Abdominal CT revealed a common hepatic artery aneurysm (25 mm in diameter) ; a portion of the aneurysm depressed the pancreas. The gastroduodenal artery branched off the common hepatic artery aneurysm. We planned coil embolization for the common hepatic artery aneurysm. However, we could not avoid occluding the proper hepatic artery ; therefore, we performed abdominal aortic-proper hepatic artery bypass with coil embolization. The patient's postoperative course was uneventful.

A 42-year-old woman with Turner syndrome was admitted to our hospital due to severe aortic stenosis. Transthoracic echocardiography demonstrated severe aortic stenosis with a bicuspid aortic valve. Enhanced computed tomography revealed that the left upper pulmonary vein connected to the innominate vein, and the ascending aorta was enlarged (maximum diameter of 41 mm). Surgical intervention was performed though median sternotomy with cardiopulmonary bypass. After achieving cardiac arrest by antegrade cardioplegia, we performed an anastomosis to connect the left upper pulmonary vein to the left atrial appendage. Then, aortic valve replacement was performed with an oblique aortotomy in the anterior segment of the ascending aorta. The aortic valve was a unicaspid aortic valve. Following completion of aortic valve replacement with a mechanical valve, reduction aortoplasty was performed on the ascending aorta. The postoperative course was uneventful.

An unconscious 79-year-old woman was admitted. Echocardiography showed cardiac tamponade with pericardial effusion. Enhanced computed tomography revealed pericardial effusion and a coronary artery aneurysm (maximum diameter of 16 mm) on the left side of the main pulmonary artery. Emergency coronary angiography confirmed the aneurysm, which originated from a branch of the left anterior descending artery. Emergency surgery was performed through median sternotomy with cardiopulmonary bypass. After cardiac arrest by antegrade cardioplegia, the aneurysm was opened and two orifices of the arteries were observed. The orifices were ligated, and the remaining aneurysmal wall was closed with a continuous suture. A pathological examination of the aneurysmal wall demonstrated an atherosclerotic true aneurysm.

A 71-year-old man underwent a medical checkup at another hospital with principal complaint of chest discomfort, and was diagnosed by computed tomography (CT) to have a left subclavian artery aneurysm. The CT revealed a 33-mm saccular aneurysm that was located at the proximal portion of the left subclavian artery. Because of the high risk of surgery, we planned to insert a stent graft into the left subclavian artery. We could not avoid occluding the ostium of the left vertebral artery with commercially-available stent grafts, and so decided to insert a surgeon-modified stent graft, in which the fabric of the distal portion of the stent graft was stripped away. The surgeon-modified stent graft was inserted from the conduit of the left axillary artery. Postoperative course was uneventful and the postoperative examination confirmed complete exclusion of the aneurysm and patency of the left vertebral artery.