Immunoglobulin G4 (IgG4)-related disease can occur in various organs, most of which comprise glandular or ductal tissue. We report a case of IgG4-related disease which occurred in a cardiovascular lesion. A 69-year-old man was found to have a tumorous lesion around the coronary artery. Open chest biopsy showed the diffuse lymphoplasmacytic infiltration, occasional eosinophils and numerous IgG4-positive plasma cells within the lesions. The serum concentration of IgG4 in the postoperative period was 1,080 mg/dl (reference range, <135). We diagnosed IgG4-related periarteritis manifesting as a tumor around the coronary artery. This case suggests that IgG4-related disease can occur around the coronary artery and manifest as a periarterial mass lesion.

Seven patients underwent surgical repair of the distal aortic arch aneurysm from January 1990 to October 1997. They were 5 men and 2 women ranging from 63 to 78 years of age (mean, 72.7 years). All patients were operated with a median sternotomy only. There was one operative death, which was ruptured case. However, there were no major complications in non-ruptured cases. This retrospective study suggests that it is possible to repair the distal aortic arch aneurysm through a median sternotomy approach alone, when 1) descending aorta originates with normal size just distal to sacciform aneurysm, 2) the maximum diameter of the aneurysm is over 70mm and 3) distal involvement of the aneurysm does not extend beyond the bifurcation of the trachea. It is useful to retract descending aorta proximally by three threads with pledget for distal anastomosis in inclusion technique.

Direct coronary artery reimplantation to the aorta and mitral valve repair were successfully performed in a 29-year-old female with Bland-White-Garland syndrome (BWG syndrome). Under cardiopulmonary bypass, the main pulmonary artery was completely transected and the left coronary artery was excised with a cuff of pulmonary artery wall. Then the left coronary artery was directly anastomosed to the ascending aorta. Mitral regurgitation was repaired with valvulo-annuloplasty. The post operative course was excellent.

The patient was a 52-year-old man with a history of antiphospholipid syndrome (APS), renal dysfunction and myasthenia gravis (MG). On May 2, 1998, he had sudden chest pain while sleeping. Enhanced computed tomography revealed acute aortic dissection (DeBakey type I). We performed emergency graft replacement of the ascending aorta and the aortic arch under extracorporeal circulation. Because of perioperative anuria, we used peritoneal dialysis (PD) just after the operation. Two days after the operation, we performed re-intubation nine hours after the extubation of the tracheal tube, and performed re-extubation three days later. For a while, his postoperative course was uneventful, but because of gradual worsening of APS, we administered more prednisolone, but 74 days after the operation, he died of multiple organ failure caused by an opportunistic infection, sepsis, and disseminated intravascular coagulation. This was very rare case of acute aortic dissection with MG and APS. After administration of more glucocorticoids, it is important to be wary of opportunistic infections.

No abstract available.
Acanthosis Nigricans* ; Hyperandrogenism* ; Insulin Resistance* ; Insulin* ; Proliferating Cell Nuclear Antigen*

No abstract available.
Acanthosis Nigricans* ; Hyperandrogenism* ; Insulin Resistance* ; Insulin* ; Proliferating Cell Nuclear Antigen*

  On July 7, 2010, a 74-year-old man came to our hospital, complaining that he had a nagging pain in his chest that started the preceding day. After performing electrocardiography, blood tests and electrocardiography, we diagnosed the case as acute myocardial infarction. At first, it was thought that blood flow could be restored in due course of time, antiplatelet and anticoagulant agents were used. Intracardiac catheterization was not included in our initial treatment plan. Three days after the initiation of the treatment, the patient had pain in his left inguinocrural region. Computed tomography and magnetic resonance imaging reveled hematoma in his left iliopsoas muscle. We stopped administering antiplatelet and anticoagulant agents to him. But anemia progressed from Hb14.1g/dL to 9.8 g/dL, so blood transfusions had to be given. After that, the patient underwent a rest cure. With the passage of time, the pain and swelling of the left iliopsoas muscle went down. Regarding the cardiac condition, however, the pain in the chest did not abate even when he was taking a rest. The antiplatelet therapy was resumed, with one type of agent given at first and then with another type added. Examinations using a coronary CT and a cadiac catheter found 90% stenosis at the proximal left anterior descending coronary artery. So, a bare metal stent was placed in the near-closed artery. Ever since, there has been no recrudescence of chest pain and no recurrence of iliopsoas muscle hematoma. The extravascated blood mass seemed to be dissolved spontaneously.