Total correction for a chronic aortic dissection, producing progressive enlargement of the false lumen of the aorta involving wide range of aorta and aortic manifestation of Marfan's syndrome is a very difficult procedure. However, with the recent development in surgical techniques and management, it became possible to replace total or subtotal aorta with the prosthetic graft. Recently, we treated a 24 y/o male patient with annuloaortic ectasia, DeBakey type II+IIIb aortic dissection, and obstruction of right common iliac artery, associated with Marfan's syndrome with a two-staged operation. For the first stage, we performed Cabrol's procedure on his lesions in ascending aorta. About 2 years after that, for the second stage, replacement of total descending and abdominal aorta was pertformed.

In Kampo therapy for rheumatoid arthritis (RA), Keishi-ka-ryojutsubu-to, Keishini-eppi-itto and Keishi-shakuyaku-chimo-to are considered to be the primary formulas. However, it is often difficult to control arthritis with the primary formula alone. In this study, we administered 7.5g/day of Ji-daboku-ippo to 12 patients with RA, who had not responded sufficiently to the primary formula alone. Administration of the primary formula and other anti-rheumatoid drugs was also continued.
After three months of this supplemental administration of Ji-daboku-ippo, the mean±SE of the Lansbury's index significantly decreased from 45.3±5.8% to 33.3±3.8% (p<0. 01). After treatment for one year, a decrement in the Lensbury's index (of more than 20%) was seen in the four patients. These results suggest that supplemental administration of Ji-daboku-ippo is effective for patients who fail to respond sufficiently to the primary Kampo formulas used for RA.

This paper reports on a case in which a heavily-calcified so-called “porcelain aorta” (including the ductus arteriosus) was observed, together with a patent ductus arteriosus and aortic stenosis associated with a bicuspid aortic valve. A 76-year-old man had been referred to our hospital on a diagnosis of aortic stenosis. Since angiography revealed slight contrast in an area on the right side of the heart, echocardiography was performed and revealed patent ductus arteriosus. Severe circumferential calcification of the ascending aorta and aortic arch was observed on CT scans. Almost no calcification was observed in other areas. Aortic valve replacement and closure of the ductus arteriosus (transpulmonary approach) were performed by means of a balloon to temporarily occlude the aorta, as surgical clamping was impossible due to calcification. Hypothermic systemic perfusion and antegrade selective cerebral perfusion were used. The postoperative progress of the patient was good. Bicuspid aortic valve and patent ductus arteriosus are highly likely to be present in combination in cases of congenital cardiac anomaly, and it is therefore necessary to be particularly attentive when diagnosing such cases. It was considered that our patient, an adult suffering patent ductus arteriosus, was a rare case in which the calcified ductus arteriosus was observed and the calcification had spread to the ascending aorta.

We performed endovascular stent-graft placement on 39 patients with abdominal aortic aneurysms between 1996 and March 2002-a period of approximately 5 years (first half: until the end of June 1998, second half: July 1998 onward). Three patients in the first half of the period and 8 patients in the second half were 80 years or older. Two cases of mycotic aneurysm were observed. During the second half, we encountered high-risk cases in which the patients had complications such as coronary artery disease (5 patients), COPD (1 patient) and thoracic aortic aneurysm (4 patients). Although we had to switch to surgery in 3 patients during the first half of the period, we successfully placed stent-grafts in the other 36 cases (92%). Endoleaks were observed in 6 patients, and dissection of the iliac artery was observed in 5 patients (stents had been placed in all patients). In 50% of all cases in the first half of the period and 89% of all cases in the second half, stent-graft placement was successful and no endoleak was observed. During the follow-up period, 3 cases required additional treatment, and another 4 cases required surgery. Four patients died in hospital during the first half of the period, and 3 patients died during the following 3 years. The 3-year survival rate was 82%. It was considered that stent-graft placement for abdominal aortic aneurysms is particularly effective for high-risk patients, and that the results of this type of therapy will improve in the future.

Studies have shown that postoperative disseminated intravascular coagulopathy (DIC) occurs in some patients with cardiac disease, acute aortic dissection, and ruptured abdominal aortic aneurysm. The specific pathophysiology of DIC in these settings are related to low cardiac function, shock, infection and sepsis as well as activation of coagulation cascade in the aneurysm sac or dissected aorta. A soluble form of recombinant human thrombomodulin (rhsTM) was approved in 2008 for the treatment of DIC. This report describes the safety and efficacy of rhsTM for the treatment of DIC in patients with cardiovascular disease operated in our department. Between October 2010 and March 2012, 35 patients with postoperative DIC were treated with rhsTM. Diagnosis of DIC was based on the diagnostic criteria for DIC of the Japanese Association for Acute Medicine (JAAM). During the first 6 months of the study period, after a diagnosis of DIC was made, the patients were treated with gabexate mesilate and antithrombin III, and if patients showed no improvement with conventional treatment, they received rhsTM for 6 days. During the last 10 months of the study period, patients received rhsTM soon after a diagnosis of DIC was made. Twenty seven patients survived for 28 days after rhsTM treatment, and the mortality rate was 22.9% (8/35). Patients who survived showed improvement in acute phase DIC scores, FDP levels, D-Dimer, fibrinogen and platelet counts during rhsTM treatment, but no improvement was observed in patients who died. No serious adverse events were found up to 28 days after the start of rhsTM administration. In conclusion, this study showed no adverse events of rhsTM, and further studies are needed to confirm that rhsTM administration is an effective therapeutic modality in the management of DIC after cardiovascular surgery.

Recently, arterial keton body ratio (AKBR) has attracted attention as a new indicator of liver function which is in equibilium with the ratio between oxidized and reduced forms of free nicotinamide-adenine dinucleotides (free NAD⁺/NADH ration) in the mitochondria. There are few reports on whether AKBR contributes to the hepatic energy charge in the open heart surgery with extra corporeal circulation (ECC) or not. This study was undertaken to clarify the contribution of AKBR to the hepatic energy charge during ECC and the relationship between AKBR and hepatic blood flow. AKBR was determined before, during and after ECC in the open heart surgery for 14 patients. Furthermore, the blood flow in hepatic artery, portal vein and liver microcirculation was measured before, during and after ECC in canine models. Finally, the pulsatile perfusion was performed in canine models and compared with the conventional non-pulsatile perfusion for the blood flow and AKBR. In clinical cases, AKBR was decreased in all cases during the ECC. AKBR which was measured at 2 or 3hr after weaning from the ECC was negatively correlated to the total perfusion time with -0.57 as the correlation coefficient. Six patients who were on the ECC over 180min did not show a good recovery of the AKBR after weaning from the ECC. Especially, three patients presented a clinical picture of acute hepatic failure with jaundice, elevation of the serum levels of transaminase and direct hyperbililubinemia, but only one showed hypoglycemia. These patients showed no improvement in clinical data and AKBR. The patient with improved AKBR recovered clinically. In our experiment, the blood flow in the hepatic artery, portal vein was measured by electromagnetic blood flow meter and the liver microcirculation was measured by laserdoppler flowmeter. The blood flow was decreased remakably in the non-pulsatile group at all sites of measurement: it recovered after ECC in hepatic artery and portal vein, but liver microcirculation did not improve well. AKBR was decreased during ECC and did not recover after ECC in the non-pulsatile perfusion. When the pulsatile perfusion was performed, liver circulation was maintained well, and AKBR recovered well after ECC. The above results suggest that AKBR reflects the liver microcirculation and pulsatile perfusion is beneficial to the liver microcirculation. Pulsatile circulation, however, involves several problems, hemolysis, the decrease of platelets, and so on, but these problems have been improved gradually. We think that the pulsatile perfusion will be used in clinical operations to maintain the good hepatic circulation.