A 23-year-old man had had a fever of unknown origin for a month. Aggravation of shortness of breath brought him to our hospital. After a close inspection, transthoracic and esophageal echocardiography (TEE) showed severe aortic valve regurgitation (AR) with vegetation extending for 25mm. The valve was bicuspid and the vegetation was on the left side valve. TEE also revealed a streak of mitral valve regurgitation (MR). In spite of continuous antibiotic therapy, congestive heart failure developed with progressive MR, so we performed an emergency operation. The aortic valve was bicuspid composed of an agglutinated left and non-coronary cusp, and 15×30mm vegetation was attached on the left. Checking the mitral valve after resection of aortic valve, we found a perforation 3mm in size at the center of the anterior mitral leaflet. After resection of the infected area, we repaired it with a Xenomedica patch 10mm in size through the aortic orifice. Two abscesses located beneath both leaflets were eradicated and finally aortic valve replacement was done with an SJM 23.

Objectives : Pharmaceutical companies in Japan can attach pharmacoeconomic (PE) data to their application for new drug prices covered by the National Health Insurance (NHI) system since 1992. To examine the present state of PE studies in Japan by investigating the situation of PE data attachments and their details and also to identify problems concerning how to reflect the PE information in new drug pricing, a questionnaire-based survey of pharmaceutical companies was conducted.
Method : The survey covered 115 drugs filed by the members of the Japanese Pharmaceutical Manufacturers Association (JPMA), which are among the 137 drugs listed between June 20, 1997, when the NHI drug price formula was published, and November 17, 2000. Questionnaires were returned on 114 of the 115 drugs studied and the response rate was 99%.
Results : PE data was attached to 37 of the 114 drugs (32%) at the time of application. The ratio of applications accompanied by PE data tended to be decreased from 1998. No significant relation was observed between a premium for a drug and the attachment of PE data. The most common method used in the attached PE data was cost-effective analysis (14 of 37 drugs ; 38%), followed by cost-benefit analysis (7 drugs) and cost-minimization analysis (6 drugs). Cost-utility analysis was not applied to any of the 37 drugs.
Discussion : Compared with overseas PE studies, Japanese studies were found to be less comparable with one another due to the lack of a uniform system of research and reporting results. Other problems observed included insufficient epidemiological data for analysis and difficulty in the cost data collection. The questionnaire-based survey revealed that Japan needs to set guidelines for PE studies and to establish epidemiological and cost databases for these research activities. It also suggested the necessity of reviewing the present method of clinical tests so that economic parameters for PE analysis and QOL may be obtained from these tests.

A 6-month-old baby boy had undergone the Jatene procedure at 4 days. Four months later, catheter intervention (balloon angioplasty) was performed because of severe stenosis at the bifurcation of the pulmonary arteries. Twenty days later, several episodes of cyanosis occurred and he was readmitted. The existence of shunt flow between the sinus of valsalva and the pulmonary bifurcation was detected by echocardiography and examination by 16-row MDCT revealed 2 holes at this site. Under a diagnosis of aortopulmonary (AP) window, the patient was placed on cardiopulmonary bypass and the pulmonary artery was opened after aortic clamping. There was a ridge between the bifurcation of the pulmonary arteries. After removing it, 2 holes were visualized that resembled the findings on 16-row MDCT. These holes were closed with Xenomedica patches and the main pulmonary artery was also extended with a Xenomedica patch. AP window is a rare complication after balloon angioplasty for pulmonary stenosis, but we must take great care to prevent this complication.

The patient was a 71-year-old man who had been treated for Parkinson's disease for 21 years. He was admitted because nocturnal dyspnea occurred several times. Echocardiography revealed congestive heart failure because of combined mitral and aortic regurgitation. Double valve replacement was planned. There was a risk of the occurrence of neuroleptic malignant syndrome (NMS) if his drugs for Parkinson's disease were stopped suddenly, so careful control of drug doses was required. Although the patient developed aggravation of his Parkinson's symptoms, careful observation and adjustment of medications prevented the occurrence of NMS.

Objective : Although dissection extending to the aortic root is a common finding, it is potentially fatal in patients with acute type A aortic dissection. The purpose of this study was to evaluate surgical results of acute type A aortic dissection with proximal involvement. The proximal extension of dissection, types of aortic root procedure and its feasibility were investigated. Methods : Between 1997 and 2011, 80 patients with acute type A aortic dissection underwent emergent operation. Results : Dissection reaching around the coronary artery orifice was observed in 28 patients. In 11 patients, both left and right coronary arteries were involved with aortic dissection. Aortic root replacement was performed in 4 patients. In 7 patients, the dissected aortic root was reinforced by GRF glue and proximal aorta was replaced with a graft. Among these patients, postoperative aortic root redissection with severe aortic regurgitation was observed in 5 patients during postoperative long-term periods. All of them required surgical re-intervention of the aortic root. In 17 patients, dissection was extended to the right coronary artery. Aortic root reconstruction was performed in 2 patients due to pre-existing annulo-aortic ectasia. The remaining 15 patients underwent proximal reinforcement with GRF glue. No patient showed dissection extending to the left coronary artery alone. Operative mortality was 11% and other types of complications concerning the aortic root was not observed. Conclusion : An acceptable outcome was demonstrated with our surgical strategy of proximal aortic dissection. For patients, in particular, with proximal involvement to both the left and right coronary arteries, redissection of the aortic root should be noticed as a late complication with considerable frequency. Special care should be taken for precise recognition of the proximal extension of dissection and appropriate surgical procedure including simultaneous aortic root replacement.

Preoperative evaluation of cardiac anatomy is essential to determine the correct surgical procedure for congenital heart disease. Multi-detector row CT (MDCT) is a useful alternative imaging modality to cardiac catheterization and echocardiography. Sixteen patients (12 with total anomalous pulmonary venous return (TAPVR) and 4 with aortic arch anomalies) underwent 16-slice multi-detector row CT scanning. Three-dimensional reconstruction by MDCT was useful to determine the type of TAPVR and the presence of pulmonary venous obstruction (PVO) in TAPVR patients, as well as to detect postoperative PVO in patients who underwent intracardiac repair. In 2 patients who had asplenia associated with TAPVR III and I a, MDCT enabled an intra-atrial approach for TAPVR repair by precise preoperative determination of the relationship between the common PV chamber and single atrium. In patients with aortic arch anomalies, MDCT was useful to determine the type of anomaly, the presence of arch hypoplasia, and any associated rare vascular anomalies (including isolated subclavian artery, and the right-sided descending aorta with left aortic arch). In conclusion, MDCT provides reliable preoperative evaluation of pulmonary venous return and aortic arch anatomy, and therefore is extremely useful for surgical management of congenital heart disease.

A 9-month-old boy who had been given a diagnosis of double outlet right ventricle (DORY), partial anomalous pulmonary venous return (PAPVR), ventricular septal defect (VSD), pulmonary hypertension (PH) and polysplenia with azygos connection, underwent pulmonary artery banding at the age of 6 months. At 2 months after surgery, a chest computed tomogram revealed a main pulmonary artery aneurysm and a main pulmonary artery-right pulmonary artery fistula caused by bacterial endocarditis due to a methicillin-resistant Staphylococcus epidermidis. We performed pulmonary arterioplasty and re-pulmonary artery banding for acute aggravation of cardiac insufficiency and obtained good results. This is an extremely rare case that was treated infectious pulmonary artery aneurysm and fistula after pulmonary artery banding.

We describe a case of postoperative aortic valve regurgitation (AR) after arterial awitch operation (ASO) successfully managed by the modified Konno procedure. A 4-year-old girl with complete transposition of the great arteries (TGA, Type II) had undergone the ASO (LeCompte maneuver) at 10 days of age. Because of progression of moderate AR 4 years after ASO, the modified Konno procedure with aortic valve replacement (SJM 21mm) was successfully performed. She remains in good clinical condition at the last follow-up at 5 years.

Protein C (PC) deficiency is an inherited thrombotic disorder with a prevalence of 0.19% among the general population. PC deficiency is associated with an increased risk of thrombosis when other risk factors are present, such as trauma, surgery, or infection, and is an important cause of mechanical valve thrombosis. We performed tricuspid valve replacement with a 29mm Carpentier-Edwards Perimount valve in a 20-year-old man with PC deficiency. The patient had corrected transposition of the great vessels with severe tricuspid insufficiency, as well as a history of cerebral infarction. In the perioperative period, we used only heparin sodium as the anticoagulant. When we restarted administration of warfarin, changing over from heparin, transient increases of serum plasmin inhibitor-plasmin complex (PIC) and thrombin antithrombin complex (TAT) levels were observed. Despite an increased dose of heparin, an appropriate activated partial thromboplastin time (APTT) was not obtained. This suggested a hypercoagulatory state, but the postoperative course was uneventful. Management of perioperative anticoagulation, prevention of late thrombotic events, and prosthetic valve selection in this particular situation are discussed.

Chronic disseminated intravascular coagulation (DIC) complicates 5.7% of thoracic aortic aneurysm. DIC with thoracic aortic aneurysm is characterized by hyperfibrinolysis, but usually shows a stable condition in a state of compensated non-overt DIC with limited hemorrhagic symptoms. However, in some cases, hemorrhage caused by external factors may induce uncompensated overt DIC and lead to serious hemorrhagic tendencies. In the present study, we report a patient with a thoracic aortic aneurysm complicated by DIC who exhibited marked hemorrhagic tendencies. DIC remarkably improved following administration of recombinant human soluble thrombomodulin.