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Hereditary angioedema (HAE) is a rare disease that causes serious health problem and affects on quality of life for patient due to recurrent episodes of angioedema in various body such as the skin, larynx, digestive tract, and limbs. Many HAE patients have deficiency or dysfunction of C1 inhibitor, impaired regulation of plasma kallikrein activity and overproduction of bradykinin, resulting in leading to episodes of increased capillary hyper permeability and angioedema. Therapy of HAE consists of on-demand treatment for acute attack and prophylactic treatment by suppressing the onset of acute attack in the short and long term. However, no drug has been approved for long-term prophylaxis in Japan. Berotralstat hydrochloride (ORLADEYO Capsules 150 mg) is an oral, selective plasma kallikrein inhibitor approved for the suppression of the onset of acute attacks in HAE in Japan in January 2021. Preclinical studies demonstrated that Berotralstat is a potent and highly specific inhibitor of human plasma kallikrein activity. Berotralstat suppressed bradykinin production in the HUVEC system. Clinical studies demonstrated that oral administration of Berotralstat to HAE type I or type II patients at a dose of 150 mg once daily showed a reduction of HAE attack rate and clinically significant change in angioedema quality of life score. The most common side effect was gastrointestinal symptoms. In conclusion, preclinical and clinical data indicated that Berotralstat is an effective treatment for long-term prophylactic treatment by suppressing the onset of acute attack in HAE patient and is considered to be a useful treatment option for patients.

A 70-year-old man visited a private hospital with the chief complaint of right lower limb pain. Fluorodeoxyglucose-emission tomography (FDG-PET) showed abnormal uptake in the pubic bone, right femur, and ascending colon. The patient was referred to our hospital for further evaluation. The following tumor marker levels were found : prostate-specific antigen (PSA) 20.57 ng/ml, carcinoembryonic antigen (CEA) 108.5 ng/ml, carbohydrate antigen 19-9 (CA19-9) 1,002.1 U/ml. An open pubic bone biopsy was performed. The pathological diagnosis was metastatic adenocarcinoma from prostate cancer. Prostate and ascending colon cancers were clinically diagnosed as T2bN0M1b and T2N0M0, respectively. Laparoscopic colectomy was performed. Androgen deprivation therapy started immediately and the serum PSA level was maintained at <0.2 ng/ml during the follow-up period. However, the CEA and CA19-9 were higher than the normal level 2 years after the surgery. In addition, the FDG-PET revealed abnormal uptake in the pubic bone. Thus, a pubic bone biopsy was performed again. The histological diagnosis was metastatic adenocarcinoma from the ascending colon cancer. Although the patient received combination chemotherapy, he died of colon cancer.

Ten years ago, a seventy-year-old female underwent extirpation of a left retroperitoneal tumor that was 58×36 mm in size. The pathological diagnosis was malignant peripheral nerve sheath tumor (MPNST) at that time. The patients visited our hospital with the chief complaint of back pain at ten years after surgery. Computer tomography (CT) showed recurrent tumors at the pancreas and the left kidney. Fine-needle aspiration biopsy was performed because of the possibility of pancreatic tumor. The pathological diagnosis was the recurrence of MPNST. The patient underwent extirpation of the recurrent tumors along with the pancreatic body and tail, transverse colon, spleen and left kidney. The definitive diagnosis was dedifferentiated liposarcoma with murine double minute 2 (MDM2) gene amplification and positive of p16Ink4 (p16). The previously resected tumor also revealed MDM2 gene amplification and positive of p16. Based on these results, our diagnosis in this case was recurrence of dedifferentiated liposarcoma. At 6 months after surgery, the patient had no local recurrence or distant metastases.

The patient underwent laparoscopic left radical nephrectomy for clear cell renal cell carcinoma (ccRCC). After surgery, the patient had multiple lung metastases and underwent the combination therapy of radiofrequency ablation, interferon-alpha, and inteleukin-2. Thereafter, computed tomography showed multiple lymph node and brain metastases. The patient was administered targeted therapy and radiation. Eventually, the patient suddenly complained of dyspnea. An echocardiogram, coronary angiography and magnetic resonance imaging suggested acute heart failure and pericardial effusion due to a metastatic tumor in the cardiac anteroseptal and posterior wall. Nivolumab was administered for cardiac metastases. The patient has been in stable condition with no progression of cardiac metastases after the administration of nivolumab for 22 months.

Allergen immunotherapy is the only curative treatment for IgE-mediated allergic diseases in contrast to symptomatic treatment such as anti-histamine agents. Subcutaneous immunotherapy (SCIT) has been introduced in Japan for treatment of allergic rhinitis and/or asthma caused by pollens and/or house dust mites (HDM) in early 1960s, and the clinical efficacy has been well-known. However, the major drawbacks of SCIT are necessity of repeated painful injections as well as the risk of severe systemic adverse reactions. Sublingual immunotherapy (SLIT) was developed to resolve these issues. In Japan, Japanese cedar (JC) pollen SLIT-drop was developed initially for treatment of JC pollinosis, and approved for patients of 12 years of age and older in 2014. For adolescent and adult patients with HDM-allergic rhinitis, HDM SLIT-tablet was launched in 2015 and subsequently approved to be also available for pediatric patients (<12 years of age) in 2018. Moreover, JC pollen SLIT-tablet for JC pollinosis was approved in 2018 for all patients with no age limit. Here, we also describe that the formulation technologies of SLIT tablets and distribution of allergens after sublingual administration as well as the development of SLIT drop/tablets.

A 61-year-old woman with a breast tumor detected by mammography examination was admitted to our hospital. Ultrasonography showed a 15.5×7.2mm sized irregular mass at the left BD area. Vacuum-assisted biopsy did not reveal any malignant cells. After 3 months, ultrasonography reexamination showed that the irregular mass had increased to 24.2×16.5mm in size, and it had spread to multiple axillary lymph nodes. The patient was diagnosed with breast cancer by core needle biopsy of the axillary lymph node. Total mastectomy with axillary lymph node dissection was performed. The pathological diagnosis was solid-tubular carcinoma with infarcted necrosis. The number of metastatic axillary lymph nodes was confirmed to be 23 in total. This case was considered very rare and important because there have been very few reports of breast cancer with infarcted necrosis.

In recent years, breast micro-lesions such as ductal carcinoma in situ(DCIS)were detected with progress of the image diagnosis. We investigated the usefulness of vacuum-assisted biopsy(VAB)for initial biopsy of breast tumors. We analyzed 32 cases of VAB performed for breast tumors. The pathological diagnosis of the biopsy specimens was malignant lesions in 10 cases, border-line lesions in 1 and benign lesions in 21 cases. 11 cases underwent surgery and the final histopathological diagnosis was the same in 10 of them. One case histopathology varied from DCIS to invasive ductal carcinoma(IDC). It was suggested that VAB at initial biopsy was a useful biopsy method.

A 35-year-old man wasreferred to our hospital for treatment of a right adrenal tumor detected by ultrasonography during a physical check-up. Contrast-enhanced abdominal computed tomography revealed a poorly enhanced 74 mm tumor situated adjacent to the upper pole of the right kidney. The tumor consisted of fat with peripheral calcification. Magnetic resonance imaging also revealed a right retroperitoneal tumor with fatty contents and well-circumscribed capsule. The endocrine examination revealed the tumor as non-functioning. These findings were suggestive of a right adrenal myelolipoma. We performed laparoscopic right adrenalectomy because of its large size and malignant potency. The pathological examination revealed the retroperitoneal tumor asa mature teratoma existing apart from the adrenal gland. Primary retroperitoneal teratomasare relatively rare. Herein, we report thiscas e of adult mature teratoma occurring in the retroperitoneum.

A combination of tetralogy of Fallot( TOF) and total anomalous pulmonary venous return(TAPVR) is rare and results in chronic volume and pressure load of the right side of the heart and underfilling of the left heart. We report a successful 2-staged surgical correction of TOF associated with TAPVR and atrial septal defect. The patient was unsuitable for total primary intracardiac correction because the volume of the left ventricle was considered to be small. First, repair of anomalous pulmonary venous return and palliative right ventricle outflow tract reconstruction were simultaneously performed in 2 months of birth. One year after 1st operation, cardiac catheterization revealed that normalization of left ventricle volume, so 2nd operation was planned. Total correction of ventricular septal defect and right ventricle outflow reconstruction was performed and the patient was discharged on the 21st postoperative day with good hemodynamic status.

Pneumonia is a major and life-threatening complication after cardiovascular surgery. The objective of our study was to describe epidemiology, clinical characteristics, and risk factors of pneumonia after cardiovascular surgery. From January 2007 to December 2011, 511 consecutive patients (age 67.3±11.9;336 men, 175 women) were enrolled in this study. Pneumonia was diagnosed according to Centers of Disease Control and Prevention surveillance criteria for healthcare associated infection. Data collection included preoperative, intraoperative, and post-operative variables. The overall incidence of pneumonia was 72 cases(14.0%). The mortality in pneumonia group was significantly higher than that in non-pneumonia group (16.6% vs 4.3%, Odds ratio 4.4 p<0.001). Multi-logistic analysis revealed that elderly patient, preoperative congestive heart failure, preoperative hemodialysis, and operation of the thoracic aorta were independent risk factors for pneumonia after cardiovascular surgery.

Although many patients with a ventricular septa defect undergo primary intra-cardiac repair surgery, some patients require 2-stage repair with pulmonary artery banding. Patients with 21-trisomy, who exhibit fragility of the pulmonary vasculature and respiratory systems, may be candidates for this approach. Herein, we report the case of a 21-trisomy patient with ventricular septal defect associated with pulmonary hypertension. She underwent pulmonary artery banding at 4 months of age, and a subsequent radical operation at 27 months. Serial pathological findings between pre- and post-pulmonary artery banding revealed that changes of pulmonary vasculature and respiratory systems were reversible. Her Heath-Edwards grade changed from 0 from II and index of pulmonary vasculature disease changed from 1.1 to 1.0. In conclusion, pulmonary artery banding in a case with pulmonary hypertension prevented the lung from becoming irreversible pathological changes.

Biliary tract infection (BTI) including acalculous cholecystitis is a rare but life-threatening complication after cardiovascular surgery. The objective of our study was to describe epidemiology, clinical characteristics, and risk factors of BTI after cardiovascular surgery. From January 2007 to December 2011, 586 consecutive patients(age68±11;397 men,189 women)were enrolled in this study. BTI was diagnosed according to Centers for Disease Control and Prevention (CDC) surveillance criteria for healthcare associated infection. Data collection included preoperative, intraoperative, and post-operative variables. The overall incidence of BTI was 3.9%. The mortality in BTI group was significantly higher than that in non-BTI group (17.1% vs 5.5%, p<0.05). Multi-logistic analysis revealed that operation of the thoracic aorta( p<0.05) and massive transfusion(p<0.01) were independent risk factors for BTI after cardiovascular surgery.

A 65-year-old female was admitted to our hospital with mitral regurgitation (MR). Transthoracic echocardiography showed severe mitral valve prolapse and subaortic septal hypertrophy with no pressure gradient. Mitral valve plasty consisted of artificial chorda implantation and ring annuloplasty was performed. During intensive care unit( ICU) stay after operation, systolic murmur and low cardiac output syndrome were noted and echocardiography revealed left ventricular outflow tract obstruction (LVOTO) without systolic anterior motion and MR. Cessation of catecholamine, volume administration, beta-blocker and negative inotropic drug like cibenzoline rapidly reduced LVOTO and the hemodynamic condition was improved. Even in a case of subaortic septal hypertrophy with no pressure gradient, emergence of LVOTO should be considered when new systolic murmur and low cardiac output syndrome appeared.

The patient was a 53-year-old woman with a large left breast tumor. Triple-negative breast cancer with bilateral axillary lymph-node metastasis and bone metastasis was diagnosed. The primary treatment consisted of chemotherapy with a combination of paclitaxel(PTX 80mg/m 2)and bevacizumab(Bev 10 mg/kg). After chemotherapy, the tumor shrunk and showed an obvious decrease in size, eventually disappearing. After therapy, an ulcer was detected on the left breast, and mastectomy and axillary lymph node dissection were performed. The patient's postoperative course was uneventful and she has been receiving chemotherapy at our outpatient clinic. Thus, combination therapy with PTX and Bev may be useful for the local control of advanced breast cancer.

Background: Procalcitonin( PCT) is a new diagnostic marker of severe bacterial infection and sepsis., Purpose: To evaluate the usefulness of PCT in patients with suspicion of bacterial infection after cardiovascular surgery., Methods: From January 2012 to December 2012, 150 consecutive patients after cardiovascular surgery were studied retrospectively. Postoperative infection was diagnosed under Centers for Disease Control and Prevention (CDC) guideline for healthcare associated infection, and biomarker levels and microbiological specimen were evaluated., Results: Only blood stream infection group revealed higher PCT levels( median 5.0 ng/ml) than non blood stream infection group( median 0.1 ng/ml)[p<0.01]., Conclusion: PCT is the best biomarker available for the clinical diagnosis of blood stream infection after cardiovascular surgery.

A 46-year-old woman was admitted to our hospital because of congestive heart failure and atrial fibrillation. She had undergone atrial septal defect repair at the age of 7 years. Computed tomography( CT)scan revealed right upper pulmonary vein returned to superior vena cave and the left upper pulmonary vein returned to the innominate vein. Cardiac catheterization showed Qp/Qs of 2.6. Pulmonary venous drainage repair and right-sided maze procedure was planned. The right upper pulmonary vein was led to the left atrium using the modified Warden Procedure. The left upper pulmonary vein was divided and anastomosed to the left atrial appendage. Concomitantly right-sided maze procedure was performed. Postoperative course was uneventful and CT scan revealed no stenosis at anastomotic sites. Sinus rhythm was retained and she was discharged on postoperative day 30.

Background: Aortic valve replacement( AVR) is a definitive treatment in patients with severely calcified aortic stenosis (AS). However, elderly patients with multiple comorbidities are considered to be unsuitable for AVR. Percutaneous transcatheter aortic valvuloplasty( PTAV) has been performed as a palliative option to relieve symptoms or to be a bridge use toward definitive therapy. This study aimed to examine the effectiveness and clinical outcome of surgical AVR subsequent to PTAV., Patients and Methods: Since 2010, 5 symptomatic patients have undergone AVR after PTAV in our institution. PTAV was performed as a bridge to definitive AVR in 2 patients. Other 2 patients developed symptomatic aortic valve restenosis during a follow-up, and required AVR. In the last patient,AVR was carried out after successful noncardiac surgery subsequent to PTAV., Results: AVR improved cardiac hemodynamics in all patients. There was no operative death. Postoperative course was uneventful and all patients were discharged on foot except for 1 patient in whom metastatic liver tumor from advanced gastric cancer was noted., Conclusion: AVR might be performed safely even in high-risk patients with the history of PTAV. PTAV is useful as a palliation or a bridge to definitive therapy for treatment of patients with severe AS unsuitable for surgery.

We described a case of a neonate of 13 trisomy with hypoplastic aortic arch and multiple ventricular septal defect (VSD). The patient presented symptoms of congestive heart failure due to pulmonary high flow. Because of ductal patency without prostaglandin infusion, bilateral pulmonary artery banding was performed for regulation of pulmonary high flow. Surgical intervention improved the clinical symptoms of the patient and permitted the patient to be discharged. We concluded that palliative surgery for the patient contributes to improve the quality of life both the patient and the family. This is the 1st report of bilateral pulmonary artery banding for a patient with 13 trisomy.

A 74-year-old female was admitted to our hospital due to hyperkinetic congestive heart failure. Noninvasive positive pressure ventilation, continuous hemodialysis and inotropic support were needed preoperatively. Computed tomography revealed saccular aortic arch aneurysm with aortopulmonary fistula toward the left pulmonary artery. Cardiac catheterization revealed severe left to right shunt with the pulmonary flow/systemic flow (Qp/Qs) ratio of 6.2.Emergency operation was performed under hypothermic circulatory arrest followed by selective cerebral perfusion and lower body perfusion. The aortopulmonary fistula was directly closed, reinforced with the aneurysmal wall, and total arch replacement was performed. Although the patient temporarily recovered by endoscopic suction of the blood from the lungs and continuous hemodialysis filtration, she died because of multiple organ failure due to mesenteric ischemia on postoperative day 34.

A 40-year-old man had undergone total arch replacement using the elephant trunk technique. Three years after the initial operation, stenosis of the elephant trunk due to enlargement of a false lumen was detected. Replacement of the descending thoracic aorta was performed. A narrow segment of the elephant trunk was compressed by old hematoma and lapped around the connective tissue. A new graft was anastomosed to the narrow segment of the elephant trunk in an end-to-side fashion. Postoperative course was uneventful, and he was discharged 18 days after the operation.

Port-access minimally invasive cardiac surgery (MICS) is complicated by limited skin incision and surgical field. In order to optimize the set-up and port placement in this surgery, we have employed image overlay technique prior to operation consisted of three-dimentional computed tomography (3DCT)image projection on the patient surface. With the aid of this technique, we successfully performed port-access MICS in 12 patients and mitral valve replacement in 1 patient with severe mediastinal displacement after previous pneumonectomy. Image overlay projection could allow surgeons to view underlying anatomical structures virtually and obtain a good operative field. Our proposed technique would be expected to make port-access MICS easier and probably safer in this particular circumstance.

A 46-year-old woman with homozygous familial hypercholesterolemia was referred due to aortic regurgitation. The patient was introduced selective low density lipoprotein cholesterol(LDL)apheresis 20 years ago. Echocardiogram revealed severe aortic regurgitation, and computed tomography revealed thoracoabdominal aortic aneurysm. We considered 2 staged operations were necessitated. Firstly, aortic valve replacement was performed. Emergent coronary artery bypass grafting was also done because intraoperative myocardial ischemia was strongly suspected from left ventricular hypokinesis. One year later, replacement of thoracoabdominal aorta was performed. Post-operative course was uneventful and the patient was discharged at post-operative day 21. The patients with homozygous familial hypercholesterolemia must be strictly followed up because systemic atherosclerosis frequently exacerbates despite selective LDL apheresis.

Background: Cardiac resynchronization therapy (CRT)has been accepted broadly as an alternative to medical treatment in managing severe heart failure patients. Despite advance in CRT, the presence of a significant valvular heart disease was currently specific exclusion criteria and a response to this therapy remains unclear. The purpose of this study was to determine the effectiveness of CRT in heart failure patients undergoing valvular operation simultaneously., Patients and Methods: Between July 2010 and May 2012, 8 heart failure patients who underwent CRT in conjunction with valvular surgery were experienced. Right and left ventricular and atrial epicardial leads were implanted after completion of valvular procedures. In patients with chronic atrial fibrillation(Af),maze procedure was performed in order to eliminate Af. To evaluate the improvement of ventricular mechanical dyssynchrony, the echocardiographic assessment was repeated on admission and 1 month after the CRT implantation., Results: There was no operative death. One patient of ischemic cardiomyopathy died of sustained ventricular tachycardia 2 months after the operation. Postoperative course of severe heart failure patients was uneventful and all patients except 1 discharged on foot with improved New York Heart Association (NYHA) class. Echocardiographic parameters of dyssynchrony did not reach to statistical significance, but several parameters, left ventricular( LV)-pre-ejectionperiod( PEP) and interventricular mechanical delay (IVMD) showing time delay of cardiac contraction, tended to be improve, suggesting contribution to satisfactory postoperative course., Conclusion: The acceptable outcome was demonstrated with our concept to recover the intraventricular and atrioventricular synchrony. Although it might be difficult to establish the patient selection criteria for concomitant CRT and valvular surgery, our strategy is considered to be a feasible procedure to improve the morbidity and mortality in patients with severe heart failure due to valvular disease.

To minimize the risk of malperfusion, axillary arterial cannulation which avoids retrograde flow, has been widely used in patients undergoing operations for various types of aortic pathologies. Despite several advantages of this technique, cerebral malperfusion is getting known and might be due to newly developed arterial damage or dissection. Eighty-four patients underwent cannulation of the axillary artery in recent 5 years. Malperfusion occurred in 3 patients with significant regional cerebral oxygen desaturation during operation. Axillary arterial perfusion was stopped and oxygen saturation was elevated immediately. The perfusion was switched to the femoral artery and following procedure was completed. Although axillary arterial cannulation is an attractive alternative, neither axillary nor femoral arterial perfusion is perfect to avoid the cerebral malperfusion. Whichever perfusion site is used, special care should be taken for intraoperative finding and appropriate use of each techniques.

We describe a case of replacement with a cryopreserved homograft for graft infection. A 48-year-old man had been performed ascending aorta replacement using woven dacron graft for type A aortic dissection. Two months ago, methicillin-resistant Staphylococcus aureus infection was suspected because of high-grade fever. Computed tomography (CT) scan revealed abscess formation around the prosthetic graft and redo operation using cryopreserved homograft was scheduled to avoid re-infection into mediastinum at Tohoku University Hospital, an institute cooperating with University of Tokyo tissue bank. However, Catastrophic Great East Japan Earthquake occurred the day before the scheduled date, and the patient could not have an operation at the institution. He was transferred to our hospital by helicopter. Then homograft was successfully implanted in ascending aorta after complete excision of infectious graft.

The Cabrol technique has been used to reimplant coronary arteries at the time of aortic root replacement. We describe a case of coronary stenosis after Cabrol procedure. A 42-year-old female with Marfan syndrome and chronic type B dissection who underwent aortic root replacement with Cabrol procedure complained of chest pain. Computed tomography( CT) showed extended thoraco-abdominal aortic aneurysm. Angiography revealed bilateral Cabrol limb stenosis at anastomotic sites. Re-do operation was performed under median re-sternotomy. Pannus around the coronary anastomosis was resected and patch extension using bovine pericardium was performed. Reconstruction of coronary arteries was completed by Piehler procedure and total arch replacement with elephant trunk technique was concomitantly performed. Post-operative course was uneventful. CT revealed the release from coronary stenosis.

Background: The aortic valve-sparing operation has been accepted as an ideal procedure for the treatment of aortic root aneurysm, especially in young patients. However, this operation is thought to be technically difficult due to lack of conceptual understanding of the procedure for many surgeons. In order to overcome the surgical problem, we have developed a simple valve-sparing aortic root replacement focused on standardization and reproducibility., Methods: Seven consecutive patients underwent the aortic valve-sparing operation in our institution.Our surgical strategy to restore valvular competency is based on the concept of inserting a dilated aortic annulus into small sized graft. Regardless of annular diameter, the Valvalva graft of 26 mm was used in all patients. Proximal end of the graft is secured on the outside of the left ventricular outflow tract with 12 mattress sutures. The 3 commissures are suspended upward inside the graft as possible. The remnants of aortic sinuses are sutured to the graft in U-shaped manner using continuous suture line. If necessary, aortic cusp repair was performed. Coronary arteries are reconstructed by Piehler's method., Results: There was no operative death. Valve-sparing procedure was completed without conversion to valve replacement and no significant postoperative aortic insufficiency was noted., Conclusions: The excellent outcome was demonstrated with our simple modification. Although we recommend this technique of the aortic valve-sparing procedure which is reproducible and technically less demanding, close observation would be mandatory concerning with valvular durability in this particular circumstance.

We present a rare case of papillary fibroelastoma arising from the pulmonary valve. A 45-year-old man had been suffered from palpitation. Transthoracic echocardiography clearly demonstrated a mobile rounded mass in the right ventricular outflow tract. He underwent surgical extirpation of the tumor. A fragile, yellow tumor on the pulmonary valve was recognized at operation. The pulmonary valve was repaired with autologous pericardium. Operation was performed successfully. The diagnosis as a papillary fibroelastoma was made by histopathological examination. The post-operative course was uneventful and echocardiography showed no residual tumor of the heart.

Persistent 5th aortic arch (PFA) is a rare congenital cardiac anomaly that was firstly reported by Van Praagh et al, in 1969. A 3-month-old boy was referred to us with no symptom but heart murmur. Cardiac echocardiography and the following 3-dimensional computed tomography (3D-CT) scan revealed PFA with coarctation of aorta. There was pressure gradient of 50 mmHg between upper and lower limbs. He was carefully observed at the outpatient clinic because of no afterload mismatch. At the age of 9 months, coarctectomy and end-to-end anastomosis was performed between the PFA and distal arch below left vertebral artery. A histological examination of resected tissue revealed the thickened intima and rough elastic fiber that is absolutely different from normal aorta. Careful observation is necessary with the possibility of future aneurysmal change or re-coarctation, although the postoperative course has been so far uneventful.

We describe a case of purulent pericarditis presented with cardiac tamponade. A 65-year-old man was admitted to our hospital with shock by cardiac tamponade and high grade fever. Transthoracic echocardiography revealed a large amount of pericardial effusion. Surgical drainage via median sternotomy was carried out in an emergent manner. Because culture of pericardial effusion showed positive for methicillin sensitive Staphylococcus aureus and thick white purulence covering over all pericardium, purulent pericarditis was diagnosed. Postoperative course was uneventful and discharged in a good condition. Special care should be taken of purulent pericaditis in differential diagnosis of cardiac tamponade.

Objective: The left superior vena cava (LSVC) is often complicated with congenital heart defect. Although we simply clamp LSVC during cardio-pulmonary bypass (CPB), appropriateness of this technique has not been clarified. We noninvasively evaluate cerebral tissue oxygenation while the clamping of LSVC under CPB by near-infrared spectroscopy (NIRS)., Methods: Six children (3 male and 3 female; aged 1.0 +/- 0.6 year) undergoing open heart surgery were studied. The NIRO 300 was incorporated into an established multimodal monitoring system. Tissue oxygenation index (TOI), oxyhemoglobin (O2Hb), and deoxyhemoglobin (HHb) changes were assessed and compared with LSVC pressure., Results: There were no significant changes in cerebral oxygen delivery after LSVC clamp. LSVC pressure increased from 7.3 +/- 1.8 mmHg to 20.1 +/- 2.6 just after LSVC clamp, but gradually decreased without any maneuver., Conclusion: These data demonstrated that LSVC could be safely clamped when LSVC pressure was under 30 mmHg.

Background: The choice of appropriate treatment in children with aortic valvular lesions remains controversial. The purpose of this study is to assess early and late outcomes of aortic valve replacement with annular enlargement in children., Methods: A retrospective study was conducted in 16 consecutive patients aged 0.26 to 15.9 years operated on between 1993 and 2008. Thirteen children underwent aortic valve replacement with Konno procedure (mechanical valve: 12, homograft: 1), 2 children underwent Ross procedure, and the last child underwent Nicks procedure with mechanical valve replacement. All patients undergoing mechanical valve replacement were given warfarin with a monthly international normalized ratio (INR) control., Results: Overall early mortality was 12.5% (2 cases). Emergency operation was performed in these cases because of infectious endocarditis and acute cardiac failure. However, cardiopulmonary bypass (CPB) weaning could not be obtained. There was 1 late death 5 months after mechanical valve replacement. The patient developed methicillin-resistant Staphylococcus aureus (MRSA) sepsis after cleft palate repair. Reoperation was needed in 1 case., Conclusion: We conclude that mechanical valve replacement with aortic annular enlargement is an acceptable treatment in children. It is associated with acceptable mortality and low incidence of late events, and provides long-term survival.

Background: Interrupted aortic arch (IAA) is associated with a multitude of lesions ranging from isolated ventricular septal defect to hypoplastic left ventricle or severe subaortic stenosis (SAS). Left ventricular outflow obstruction such as SAS continues to be an important factor for deciding the surgical procedure between univentricular and biventricular repairs., Methods: A retrospective study was conducted in 8 consecutive infants aged 14 to 117 days and operated on between 2004 and 2009. Seven patients had undergone bilateral pulmonary artery banding for pulmonary high flow regulation. All patients underwent Norwood-type operation (4 with systemic to pulmonary artery shunt, 3 with right ventricle to pulmonary artery shunt, and 1 with bidirectional Glenn shunt)., Results: One patient died 2 months after surgery due to respiratory failure. The others were discharged in a good condition. One patient underwent Rastelli-type operation and biventricular circulation was achieved. The other 6 patients were all Fontan candidates., Conclusion: Satisfactory initial palliation can be achieved by Norwood-type operation for IAA with severe SAS or hypoplastic left ventricular-aortic complex.

Background: In a pediatric setting, the need for lifetime oral anticoagulation is increasing because of currency of extracardiac total cavo-pulmonary connection (TCPC) and pediatric valve surgery. We evaluated a new compact device "CoaguChek XS" for measuring prothrombin time-internatinal normalized ratio (PT-INR)., Methods: The international normalized ratio (INR) values obtained from 71 patients (223 samples) by a CoaguChek XS were compared with those obtained by a laboratory-based coagulation analyzer., Results: The values from the CoaguChek XS had a significant correlation with the laboratory based results. (r2 = 0.92, p < 0.01, regression line y = 1.05 x -0.02)., Conclusion: The CoaguChek XS will be useful in pediatric management.

Operative correction of tetralogy of Fallot has been performed for more than 50 years and well established. To date, the literature on reoperation has focused and indications and the long-term outcome have not been well defined. We herein report a case of pulmonary valve replacement due to ectopic ossification on expanded polytetrafluoroethylene (ePTFE) cusp in a female child who had undergone right ventricular outflow tract reconstruction for tetralogy of Fallot with pulmonary atresia. Because of severe right ventricular dilatation with end diastolic volume of 186 ml/m2 on cardiac catheterization, redo operation was planned for functional recovery of the right ventricle. Previous transannlar patch was removed and pulmonary valve replacement was performed using CEP 23 mm. A hard calcification was observed around the cusp of transannular patch. Pathological findings revealed ectopic ossification with osteoblast colonization around the ePTFE cusp. This is the 1st report of ectopic ossification on ePTFE.

We report a female infant with acute coronary syndrome after Aubert-Imai modification for transposition of the great arteries. Two months postoperatively, she visited the emergency room because of perspiration and tachypnea. She was diagnosed with acute coronary syndrome by blood chemistry examination. Cardiac catheterization revealed Aubert route stenosis, and emergency operation was performed. The lumen around the aortopulmonary window was almost totally occluded by fibrous tissue extending from the equine pericardial patch. This fibrous tissue was completely resected and the aortopulmonary window was deepened to enlarge the coronary ostium. We recommend a close follow-up including angiography after these procedures.

A 81-year-old woman admitted with general fatigue was found to have a giant polyp in the gastric antrum by endoscopy. The polyp prolapsed into the duodenum through the pylorus. Angiographic examination of the abdomen revealed the polyp to be about 90×35 mm in size. Laparotomy was performed. It was finally diagnosed as heterotopic Brunner's gland adenoma which had a stalk on the antrum of the stomach. Heterotopic Brunner's gland adenoma is rare. Only 3 cases including the present case have been reported in Japan.

A male baby was delivered by emergency cesarean section due to fetal distress at 30 weeks of gestational age with a birth weight of 813 g. By fetal echocardiography, the patient had been diagnosed with transposition of great arteries (type 1). Early two-staged arterial switch operation was planned after 34 gestational age avoiding intracranial hemorrhage under cardiopulmonary bypass. At 19 days of life, vegetation was revealed on the pulmonary valve by echocardiography, so he was diagnosed as infectious endocarditis. Cefotaxime and gamma-globulin were given intravenously for 4 weeks. While waiting for the increase in the body weight, desaturation from chronic respiratory distress syndrome was exacerbated. At 8 months old, urgent Senning operation was performed to improve desaturation. The patient was discharged at 20 post operative day. We conclude that Senning operation can be feasible operation in such a complicated case.

A 27-year-old woman was hospitalized by emergency due to multiple blunt trauma by traffic accident. Computed tomography (CT) showed pericardial effusion. Because hematothorax and pericardial effusion appeared alternatively, thoracocentesis and pericardiocentesis were performed. Finally, the diagnosis of cardiac rupture was confirmed, and surgery was performed through median sternotomy. The left ventricular free wall was penetrated by the edge of a broken rib through the hole of the pericardium and the pleura. Broken ribs were considered to have penetrated the pericardium and caused cardiac rupture. The left ventricular rupture was repaired using direct suture technique under no cardiopulmonary bypass. The postoperative course was uneventful. The patient was discharged on the 52th day after the operation.

Aortic aneurysms and aortic regurgitation (AR) with aortitis syndrome are occasionally reported in young women. We report a case of aortic dissection with severe AR in an 8-year-old girl. The patient underwent aortic root replacement with a composite graft. Pathological report revealed aortitis syndrome and steroid therapy was continued to suppress further inflammatory vascular reaction.

A 78-year-old female was admitted to our hospital with a diagnosis of severe aortic valve regurgitation. She had had dyspnea on effort and syncope twice in 5 months. She had also suffered from right pneumonia 8 years before, and her respiratory function was severely constrictive. Chest X-ray showed her mediastinum significantly shifted toward the right side. Chest computed tomography (CT) revealed the main pulmonary artery, right atrium (RA) and right pulmonary veins also shifted toward the right. We planned right thoracotomy at 4th intercostals space to obtain a good surgical field. A cardiopulmonary bypass was established by RA appendage drainage and femoral artery perfusion. Aortic valve replacement(AVR) was performed successfully after aortic clamp. Though defibrillator pads were placed on her back and the anterior wall of the left chest during operation, no ventricular fibrillation occurred. AVR via right thoracotomy is considered to be a good option for such a mediastinum shifted case.

A 77-year-old female was admitted to our hospital with a diagnosis of severe mitral regurgitation. Cardiopulmonary revival was done by an emergent resuscitation for the ventricular fibrillation before admission. She had mild anoxic brain damage and brain magnetic resonance imaging (MRI) revealed severe brain atrophy. Chest X-ray showed severe cardiomegaly and congestion. Beating heart mitral valve replacement was planned for the prevention of reperfusion injury. A cardiopulmonary bypass was established by bicaval drainage and aortic return. The prolapse of anterior leaflet was recognized through transeptal approach after aortic clamp. We selected continuous infusion of antegrade cardioplegia for intraoperative coronary perfusion. Mitral valve replacement was done successfully. During intraoperation and postoperation, ventricular fibrillation did not occur. On-pump beating mitral valve replacement is a good procedure to prevent perioperative ventricular arrhythmia especially such the case with a decompressed myocardial function and with a preoperative episode of lethal ventricular arrhythmia necessary for cardiopulmonary resuscitation.

One of the most common chemotherapy-related adverse reactions has been nausea and vomiting. With the recent advances in supportive care, however, it has become possible to prevent most of the chemotherapy-induced nausea and vomiting. This article summarizes the ASCO guideline published in 1999 and the 2004 NCCN guideline together with the recent advances in this field.

The availability of G-CSF increases the safety margin of chemotherapy use, especially in the management of infection. This in turn makes administration of a more intense regimen of chemotherapy possible. However, this improvement in neutropenic management could lead to an undesirable concurrent rise in thrombocytopenia risk due to the higher dose of chemotherapy administered. Although mortality from thrombocytopenia is generally quite rare, transfusions of platelets are often expensive and can be associated with side effects such as fever, hypersensitivity reaction, and occasionally infection. Therefore, transfusion of platelets should be performed when it is truly indicated. In general, the threshold for platelet transfusion is accepted as being when the platelet count drops below 10,000/microliter, unless there is an obvious bleeding lesion or other coagulation abnormality, such as DIC being identified in the patients. On the other hand, thrombotic microangiopathy (TMA) can also occur as a rare complication of the malignancy itself or from the associated cancer chemotherapy. The major features of TMA are thrombocytopenia and marked increases of destroyed erythrocytes and LDH in peripheral blood. Despite a low incidence, its high mortality rate makes it important for all physicians caring for cancer patients to be aware of it, especially in view of the ready availability of successful treatments (e.g., plasma exchanges). Early diagnosis of TMA in patients receiving chemotherapy requires special attention because some characteristics of TMA are often masked by common side-effects of chemotherapy such as bone marrow suppression. Since delay in initiation of plasma exchange could result in higher mortality, urgent hematology consultation should be obtained if TMA is ever suspected.

We introduced video-assisted thoracoscopic surgery (VATS) for chest disorders in our institution in March, 1992. At first, many of the subjects' disorders were non-malignant diseases such as spontaneous pneumothorax, but later we started to perform this procedure for lung cancer and mediastinum neoplasm, with improved result over thoracoscopic surgical procedures. Now most of the chest disorders at our institution are treated with VATS. However, many kinds of complications due to manual techniques and instrument troubles surfaced during this period. Therefore, in this article we would like to describe the complications that we have experienced in our institution using VATS and discuss how we have attempted to deal with these complications.

A 71-year-old woman receiving both angiotensin II receptor antagonist and calcium antagonist suffered severe systemic edema. She had been treated for essential hypertension with amlodipine for 2 years and candesartan for 3 months, and systemic lupus erythematodes (SLE) with steroids. During treatment, severe systemic edema appeared, mainly on her face, arms, and legs. At first, we suspected drug-induced edema by candesartan, so it was halted, but the edema still continued. We then considered amlodipine to be the culprit, and finally, the severe systemic edema disappeared after cessation of amlodipine. To control her blood pressure, we recommended candesartan, but 3 months late she suffered severe systemic edema again, thus the causative we drugs of her edema were thought to be both amlodipine and candesartan. Edema is a common symptom in elderly patients and we frequently observe drug-induced edema. In this case, there was underlying acceleration of blood vessel permeability induced by SLE and steroids and moreover, vasodilatation by candesartan and/or amlodipine further accelerated blood vessel permeability, and thus might have caused severe edema. It is very difficult to determine the cause of edema, especially in elderly patients, but we should consider not only one but also two or more drugs as being involved in drug-induced edema.

We studied the adhesive and sealing effects of sheet style fibrin adhesive, TO-193 (TachoComb), on some tissues and organs, comparing them with those of sheet style collagen agent, collagen sponge, Novacol, and Avitene and liquid fibrin adhesive agent, Beriplast P. TO-193 showed more a potent adhesive effect on liver than the sheet style collagen agents and was more potent on bone and skin than the liquid fibrin adhesive agent. Furthermore, TO-193 had a potent sealing effect at the site of incomplete suture immediately after application on a motile organ such as lung and stomach. These effects may be partly attributable to rapid expression of the effect due to the presence of a high concentration of fibrinogen on coverage. Enhancement of fibrin penetrability to the tissues by compression and inhibition of cleavage of coverage by the collagen sponge also may be participating in the effects of TO-193. These results suggest that TO-193 will be a valuable adhesive and sealing agent.

TO-193 (TachoComb) is a new fibrin adhesive agent that consists of a collagen sheet coated with fibrin glue. We compared the hemostatic effect of TO-193 in several experimental models with Beriplast P as a fibrin adhesive agent, Avitene and Novacol as a microfibrillar collagen hemostat, and a sponge-like collagen sheet as the constitutional parts of TO-193. In the in vitro bleeding model in which blood leaks out through cotton cloth, the pressure of TO-193 when blood leakage was observed was higher than those of Beriplast P, Avitene, Novacol and the collagen sheet, indicating that TO-193 possessed a strong adhesive effect on the bleeding surface. On the kidney resection surface, TO-193 showed a more potent adhesive effect than those of Beriplast P and the collagen sheet, suggesting that TO-193 has a potent hemostatic effect. In the liver resection, TO-193 significantly reduced the bleeding volume compared with that of Novacol in normal rats. Furthermore, the bleeding volume of TO-193 was about half that of Beriplast P and equivalent to that of Novacol even in anticoagulant-treated rats. From these data, it is expected that TO-193 would be a valuable hemostatic agent for clinical use since TO-193 possesses a potent adhesive ability on the bleeding resection surface and would certainly stop bleeding in both patients with normal coagulation and those with a low blood coagulation condition.