Neonatal Marfan syndrome is the most severe form of Marfan syndrome usually showing critical cardio-respiratory symptoms from the neonatal period or early infancy. We report a boy with this syndrome who presented with heart failure at 3 months of age and was referred to our department at 6 months old after intense medical treatment. He had enophthalmos, funnel chest, arachnodactyly, and Steinberg's thumb sign, but had no family history of Marfan syndrome or other cardiac diseases. Left ventricular dilatation, severe mitral regurgitation and moderate tricuspid regurgitation were noted on echocardiography. Mitral valvuloplasty and tricuspid annuloplasty were performed, and the regurgitation improved to trivial and mild level, respectively. However, rapid exacerbation of mitral regurgitation occurred, and the patient fell into circulatory collapse which needed circulatory support with extracorporeal membrane oxygenator (ECMO) on 18th postoperative day. In the emergency operation, the previous surgical procedures on the mitral valve were intact and we thought that rapid progression of the mitral annular dilatation and valve expansion to be the cause of exacerbation. Mitral valve replacement (Regent® 21 mm aortic) was performed, and the cardiac function improved, but ECMO was still needed because of the depressed respiratory function. Furthermore, tricuspid regurgitation due to annular dilatation and valve expansion was aggravated rapidly which needed tricuspid valve replacement (ATS® 20 mm mitral) 9 days after the mitral valve replacement. ECMO was ceased on the 37th day and the patient was extubated on 71st day. He was discharged from the hospital 5 months after the first operation. One year has passed after discharge, and he is doing well with anticoagulation. In the treatment of neonatal Marfan syndrome, surgical procedure for valve repair is still controversial and it should be remembered that rapid exacerbation of the atrioventricular valve can occur even after satisfactory valve repair and there should be no hesitation regarding surgical intervention when needed.

We sometimes encounter the case that we have to make an anastomosis between a prosthetic graft and an autologous vein graft in revascularization of a lower extremity. However, it is said that the intimal hyperplasia in the anastomosis site of a prosthetic graft and autologous vein graft has a tendency to become severe in the long term postoperatively. We herein report a case in which a vein cuff (St. Mary's boot) technique was very useful to prevent recurrent stenosis due to intimal hyperplasia. No recurrence of stenosis in repair lesion has been detected for 7.5 years after operation.

We have previously reported on a predictive model for deficiency-excess pattern diagnosis that was unable to predict the medium pattern. In this study, we aimed to develop predictive models for deficiency, medium,and excess pattern diagnosis, and to confirm whether cutoff values for diagnosis differed between the clinics. We collected data from patients' first visit to one of six Kampo clinics in Japan from January 2012 to February 2015. Exclusion criteria included unwillingness to participate in the study, missing data, duplicate data, under 20 years old, 20 or less subjective symptoms, and irrelevant patterns. In total, 1,068 participants were included. Participants were surveyed using a 153-item questionnaire. We constructed a predictive model for deficiency, medium, and excess pattern diagnosis using a random forest algorithm from training data, and extracted the most important items. We calculated predictive values for each participant by applying their data to the predictive model, and created receiver operating characteristic (ROC) curves with excess-medium and medium-deficiency patterns. Furthermore, we calculated the cutoff value for these patterns in each clinic using ROC curves, and compared them. Body mass index and blood pressure were the most important items. In all clinics, the cutoff values for diagnosis of excess-medium and medium-deficiency patterns was > 0.5 and < 0.5, respectively. We created a predictive model for deficiency, medium, and excess pattern diagnosis from the data of six Kampo clinics in Japan. The cutoff values for these patterns fell within a narrow range in the six clinics.

We present a successful case of a patient with multiple metal allergy—cobalt, nickel, chromium, and zinc—who had a past history of systemic metal dermatitis. He was a 58-year-old man who complained of exertional chest discomfort. After admission, he had a fever and his blood culture was positive with methicillin-sensitive Staphylococcus aureus. Three days later, multiple micro cerebral infraction was detected in magnetic resonance imaging. After an improvement of inflammatory reaction, he was transferred to our facility for cardiac examination. Moderate mitral regurgitation due to valve perforation and multiple coronary vessel stenosis were detected. Mitral valve replacement and coronary artery bypass grafting were planned to perform. We chose surgical materials based on a preoperative epicutaneous (patch) test and his clinical course was uneventful without any allergic reaction. Metal contact allergy is an important issue in cardiovascular surgery. A collaboration with dermatologists is essential for the preparation of surgical materials.

We describe a rare complication and treatment progression that occurred in a 64-year-old man with an aortic abdominal aneurysm (AAA) that had been treated by endovascular aneurysm repair (EVAR). He had undergone EVAR to treat an infra-renal type AAA 21 months previously and returned to the emergency department with back pain. Contrast-enhanced computed tomography (CT) revealed acute type B aortic dissection, so he was admitted and conservative medical management was started. Acute stomachache and limb pain appeared on hospital day 7, which prevented him from moving his lower limbs. The main body of the stent graft had collapsed, blocking blood flow, and contrast was not found in arteries from the collapsed stent graft portion to the knee level on emergency contrast CT images of the leg. His legs were revascularized by an extra-anatomical right axial-bilateral external iliac bypass. His symptoms disappeared and reperfusion injury was avoided. The collapsed stent graft had retained its original shape at 11 and 18 days after surgery. Furthermore, follow-up CT 4.5 years later showed that the stent graft retained its original form.

A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.

Cardiac leiomyosarcoma and atrial intramural hematoma are extremely rare. Here, we present the case of a 52-year-old woman with dyspnea. Echocardiography showed a large mass in the left atrium. Tumor resection was performed. A pathological diagnosis of intramural hematoma was made. Ten months later, she presented to our hospital again with dyspnea. Echocardiography and computed tomography showed recurrence of the left atrial tumor. Another tumor resection operation was performed. The tumor originated from the inferior wall of the left atrium, and it was surrounded by atrial wall thickening. Complete resection was not possible. Although postoperative chemotherapy was prescribed, the patient died of heart failure nine months after the second operation.

A 67-year-old man with dilated cardiomyopathy was admitted to our hospital for treatment of cardiac failure. After using heparin because cerebral infarction developed during hospitalization, in acknowledgment of thrombocytopenia, we reach the diagnosis of HIT. We judged surgery to be necessary because heart failure had difficulty with catecholamine secession and the left ventricular dilation progressed rapidly, and performed left ventriculoplasty, mitral valve plasty. There were no complications such as the thrombosis during cardiopulmonary bypass, and the postoperative course was good without leading to re-thoracotomy due to bleeding. He passes without a heart failure symptom by the follow of one year 6 months after surgery at home.

Due to the increasing number of patients with repaired tetralogy of Fallot (TOF), the amount of subsequent sequelae has also increased along with the need for further surgical interventions. This case report described a patient who underwent multiple reoperations for late sequelae over a period of 48 years after repair of TOF. The patient was a 58-year-old man who underwent a reparative operation for TOF at the age of 9 years. At the age of 30, he underwent patch closure of residual VSD and reconstruction of right ventricular outflow tract (RVOT) with a mono-cusped trans-annular patch combined with aortic valve replacement. At the age of 47, he underwent aortic root replacement for aneurysmal dilatation of the Valsalva sinus, and he was implanted with a pacemaker for bradycardia atrial fibrillation at the age of 51. By the age of 58, he required pulmonary valve replacement and tricuspid valve annuloplasty for right ventricular dysfunction resulting from regurgitation of the pulmonary and tricuspid valves as 4th midline sternotomy surgery. His status improved to NYHA functional class III to II after the most recent procedures, although he still needed strict medical control. A proactive approach during long-term follow-up after TOF repair is important for timely and appropriate surgical intervention in the event of late sequelae.

When mitral valve dysfunction occurs in infants and mitral valve repair is difficult, mitral valve replacement (MVR) is required. However, commercially available prosthetic heart valves can be too large to implant in infants with a small annulus. In these children, the technique of supra-annular MVR is useful. Here we report two cases of supra-annular MVR, which were performed using an expanded polytetrafluoroethylene (ePTFE) graft as a skirt for a prosthetic valve. This method has been previously reported by Sung et al. The first case was a 16-month-old, 6.7-kg male infant who suffered from Shone' syndrome, mitral stenosis (MS) with a parachute mitral valve, coarctation of the aorta (CoA), and ventricular septal defect (VSD). MS progressed after CoA repair and VSD closure and a supra-annular MVR was performed. The second case was a 5-month-old, 4.9-kg female infant who suffered from polysplenia, intermediate atrioventricular septal defect (AVSD), and severe left atrioventricular valve regurgitation. AVSD repair was performed at the age of 3 months. However, valve stenosis and regurgitation gradually progressed postoperatively and consequently, a supra-annular MVR was performed. Postoperative prosthetic valve function was good in both cases. We believe that this method of performing supra-annular MVR is useful for infants with a small annulus.