Primary angiosarcoma arising in the sinus of Valsalva: A case report.

INTRODUCTION: Primary angiosarcoma of the aorta, particularly within the sinus of Valsalva, is uncommon, with no documented instances of primary angiosarcoma. The absence of apparent clinical manifestations in this severe condition makes it challenging to diagnose, often resulting in a poor prognosis. CASE PRESENTATION: A 60-year-old patient underwent procedures for fistula closure and coronary artery bypass grafting, which resulted in the rupture of an aneurysm within the sinus of Valsalva. Computed tomography examination 5 years after the procedure suggested no pathological abnormalities. Nevertheless, the patient required repeat surgery at 67 years due to the observed expansion of the sinus of Valsalva aneurysm noted during a clinical evaluation, prompted by elevated levels of inflammatory markers. Exploration of the residual aneurysmal locus within the sinus of Valsalva revealed an intraluminal thrombus devoid of any demonstrable hemodynamic access into the aneurysmal sac. Histopathological assessment of the aneurysmal wall confirmed a definitive diagnosis of primary angiosarcoma within the sinus of Valsalva. After surgery, the patient exhibited pyrexia. Magnetic resonance imaging substantiated multifocal osseous metastases, corroborated by histological analysis following a bone biopsy, confirming a diagnosis of angiosarcoma. Therefore, adjuvant chemotherapy with paclitaxel was initiated. After 1 year, a sustained state of disease stability was noted. DISCUSSION: In this case, the need for surgical intervention, based on an expanded sinus of Valsalva aneurysm, culminated in the unanticipated detection of primary angiosarcoma. CONCLUSION: Neoplastic etiologies may plausibly underlie the pathogenesis of aneurysm formation in cases where the etiology remains obscure in the early stages of therapeutic intervention.

[Minimally Invasive Cardiac Surgery-Coronary Artery Bypass Grafting by Left Small Intercostal Thoracotomy for Very Elderly Patients:Report of Two Cases].

The increase in the age of patients undergoing operations has become more prominent in recent years. We report our experience of minimally invasive cardiac surgery-coronary artery bypass grafting (MICS-CABG) by left small intercostal thoracotomy for very elderly patients. Case 1:The patient was a 91-year-old woman with a history of percutaneous coronary intervention (PCI) for left anterior descending artery (LAD) and right coronary arteries at different timings. Due to her contrast media allergy, we performed MICS-CABG to treat 75% stenosis of LAD. Blood transfusion was not required. She was discharged on the 13th day. Case 2:The patient was a 92-year-old man. We performed MICS-CABG for 75% stenosis of the left main trunk and 90~99% stenosis of the LAD with severe calcification. Blood transfusion was not required. He was discharged on the 21th day. MICS-CABG can be performed with less invasiveness in comparison to the conventional method and therefore seems to be an effective method of surgery for very elderly patients.

A Case of Heparin-Induced Thrombocytopenia Type 2 after Repair of Juxtarenal Abdominal Aortic Aneurysm.

Heparin-induced thrombocytopenia (HIT) is still a relatively uncommon condition and it is not well known how to administer argatroban during continuous hemodiafiltration (CHDF). A 72-year-old man required CHDF with heparin because of the oliguria and hyperpotassemia directly after the open repair of a juxtarenal abdominal aortic aneurysm. As the postoperative blood platelet count dropped and there was a thrombus in the CHDF circuit, HIT was suspected and nafamostat mesilate, but not heparin, was immediately administered for CHDF. As heparin-platelet factor 4 complex was positive, we diagnosed him with HIT and started argatroban while monitoring the activated clotting time (ACT), resulting in no further obstruction of the CHDF and an increase in the platelets. There was no disadvantage for administering nafamostat mesilate which we have commonly used instead of heparin, we should have used argatroban once we suspected HIT. It may be important to consider the history of heparin especially in administering heparin and it may be useful to monitor the ACT when initially starting argatroban for patients with HIT.

[Aortic regurgitation with osteogenesis imperfecta].

A 49-year-old male was referred to our hospital for cardiomegaly and severe aortic regurgitation. He had been diagnosed with osteogenesis imperfecta (OI) due to his history of multiple fractures in childhood and blue sclera. Aortic valve replacement(AVR) was performed via femoral cannulation and full sternotomy, with the opening of the sternum limited to a width of 6-7 cm in order to protect the brittle sternum and thorax. After weaning the patient off cardiopulmonary bypass, the sternum was closed using titanium plates. He subsequently recovered without excessive bleeding or other complications, and the healing of the sternum 3 months after the surgery was satisfactory. Cardiovascular surgery has been reported to be associated with high morbidity and mortality in patients with OI due to their friability of tissue and bleeding tendency. In the present case, AVR was performed successfully in a patient with OI using various surgical techniques.

[W-shaped Resection and Suture of the Posterior Mitral Leaflet in a Patient with Barlow's Disease].

A 72-year-old female presented with congestive heart failure. Ultrasound cardiogram revealed billowing mitral leaflets and severe mitral regurgitation. We diagnosed her with Barlow's disease and performed mitral valve repair. The posterior leaflet was large, and the leaflet height was more than 26 mm. Leaflet height reduction was necessary in order to prevent systolic anterior movement (SAM). The middle scallop was resected in a" W" shape, and its center vertex was designed to have a height of 18 mm, which was the final height of the posterior leaflet. SAM and major leaks were not seen by transesophageal echocardiography after weaning the patient from cardiopulmonary bypass. The use of a W-shaped resection is a simple technique that can be used to reduce the volume and height of the posterior mitral leaflet.

[Circulatory disturbance of middle lobe by bending of lobar pedicle after right upper lobectomy].

A 66-year-old man was diagnosed as lung cancer. We performed right upper lobectomy and lymphnode dissection. On the 1st postoperative day, a chest radiograph showed an opacification in the right upper lung field. Computed tomography (CT) showed a stenosis of the middle lobe bronchus and infiltrative shadow of the middle lobe on the 3rd postoperative day. Fiber optic bronchoscopic examination also revealed a bend and stenosis of the middle lobe bronchus, but tortion was not demonstrated. On the 6th postoperative day, chest radiographic findings was worsened. Torsion of the middle lobe was suspected, and rethoracotomy was performed on the 7th postoperative day. The right middle lobe was not rotated, but the lobar pedicle bend toward cranial. The middle lobe was highly congested necessitating lobectomy.

The effect of sivelestat sodium hydrate on severe respiratory failure after thoracic aortic surgery with deep hypothermia.

Patients who undergo thoracic aortic surgery with deep hypothermia frequently have postoperative respiratory failure as a complication. Severe lung injury in these patients results in a fatal outcome. A specific neutrophil elastase inhibitor, sivelestat sodium hydrate, is an innovative therapeutic drug for acute lung injury. We evaluated the protective effects of sivelestat sodium hydrate on severe lung injury after thoracic aortic surgery with deep hypothermia. From January 2002 to July 2007, 71 consecutive patients underwent thoracic aortic surgery with deep hypothermia. Of these patients, 22 had postoperative respiratory failure with PaO2/FiO2 ratios of less than 150. They were randomly assigned to one of two groups. The first group (Group S, n = 10) was administered sivelestat sodium hydrate continuously at 0.2 mg/kg/h until weaning from mechanical ventilation; the second group (Group C, n = 12) was not administered sivelestat sodium hydrate. The groups were comparable with respect to clinical data. There were no significant differences between the two groups in age, operation duration, total cardiopulmonary bypass time, circulatory ischemia time, cardiac arrest time, intraoperative blood loss, and total transfusion volume. The improvement of pulmonary function was observed in the both groups, but more marked in Group S by statistical analysis using analysis of variance for repeated measurements. Especially, in the early phase, pulmonary function improvement was more marked in Group S. The duration of mechanical ventilation, the length of stay in the intensive care unit, and the length of hospital stay were shorter in Group S, but not significantly. Sivelestat sodium hydrate is a specific neutrophil elastase inhibitor that improves pulmonary function in patients with severe postoperative respiratory failure following thoracic aortic surgery with deep hypothermia. The drug may shorten the duration of postoperative ventilation, intensive care unit stay, and hospital stay.

Aortic valve replacement combined with the endoventricular patch technique for aortic valve stenosis complicated by ischemic heart disease.

The indication for aortic valve replacement (AVR) combined left ventricular (LV) plasty in the patient with aortic valve stenosis (AS) complicated by ischemic heart disease is controversial. We describe a case of AS with ischemic heart disease of a patient who underwent a successful surgical treatment, AVR combined with the endoventricular patch technique. The patient was an 82-year-old woman who suffered from heart failure, New York Heart Association (NYHA) class III. The heart failure derived from AS and ischemic heart disease with severely compromised LV function. She underwent AVR combined with the endoventricular patch technique and the postoperative course was uneventful. She has been well with NYHA class I for about 5 years after the operation without heart failure.

[Successful repair of penetrating cardiac injury with chopstick; report of a case].

We used percutaneous cardiopulmonary support (PCPS) to resuscitate a 54-year-old man who had stabbed himself in the left anterior chest with a chopstick. Chest computed tomography showed that the chopstick had penetrated the heart. As he was in shock due to the development of tamponade while waiting for emergency surgery, we immediately decided to initiate PCPS. After cardiopulmonary bypass was established through a median sternotomy replacing PCPS, the chopstick was removed and the stab wounds were closed by mattress sutures. The postoperative course was uneventful.

Delayed postoperative paraplegia and graft infection after a thoracoabdominal dissection.

WE REPORT THE SUCCESSFUL TREATMENT OF THORACOABDOMINAL DISSECTION, WHICH EXTENDED INTO THE LEFT ILIAC ARTERY, DESPITE TWO INDEPENDENT COMPLICATIONS: graft infection and a relatively rare, delayed postoperative paraplegia. The paraplegia suddenly occurred on postoperative day 10, and after an intravenous infusion of heparin and methylprednisolone, it gradually subsided. Moreover, graft infection was diagnosed on postoperative day 27, and with continuous irrigation of antibiotic treatment it was cured without recurrence of infection. Although anticoagulation therapy is not indicated for paraplegia, we suppose that it might be used as an adjunct therapeutic.

Successful surgical treatment of acute type A aortic dissection complicated with distal arch aneurysm.

We report successful surgical treatment in a case of acute type A aortic dissection complicated with distal arch aneurysm. A 74-year-old man presenting with sudden posterior headache was found by enhanced computed tomography to have an ascending aortic dissection (type A) and a distal arch aneurysm of 69 mm in maximal minor axis diameter. We performed total arch replacement, employing a four-branched graft and elephant trunk anastomosis through a median sternotomy. Because the aneurysm was not effectively thrombo-excluded, we performed descending aorta replacement using the elephant trunk through the left fifth intercostal space on the 44th postoperative day. The postoperative course was uneventful.

Advanced cancer with situs inversus totalis associated with KIF3 complex deficiency: report of two cases.

Situs inversus totalis (SIT) is a relatively rare congenital anomaly, occurring at an incidence of 1 in 10 000-50 000 live births. Although there are some case reports of SIT with the presence of cancer, there are few reports on the relationship between SIT and cancer. However, the recent phylogenetic investigations of this condition suggest that this may be linked to the development and progression of cancer on the molecular level. The key elements are one of the intracellular motor proteins, the KIF3 complex, and the cell-adhesion factors N-cadherin and beta-catenin. We herein present the cases of advanced gastric cancer and lung cancer with SIT, and review the relationship between SIT and the development and progression of cancer.

A case of acute arterial thrombosis caused by nephrotic syndrome.

Venous thromboembolic complications are frequently caused by nephrotic syndrome, while arterial thrombosis has rarely been reported. We report the successful treatment of a 53-year-old man who suffered from sudden severe pain of the left lower limb and facial edema. Abdominal computed tomography showed that the left common iliac artery was occluded from its origin. Although he had left peroneal nerve paralysis, thrombectomy and fasciotomy were performed for limb salvage. Renal biopsy revealed minimal change nephrotic syndrome after the operation. No recurrence has been observed. Nephrotic syndrome might be considered as a cause of acute arterial thrombosis.

Biphasic metaplastic sarcomatoid carcinoma of the breast: report of a case.

A 69-year-old woman was admitted to our hospital with the complaint of a right breast mass. As a result of thorough examinations, she was diagnosed with breast cancer and underwent breast-conserving surgery. The pathological findings of the resected specimen showed that the tumor consisted of intermingled carcinomatous and sarcomatous components with a transition zone. On immunohistochemical study, the sarcomatous cells in this transition zone showed partial positive staining for CD10, the myoepithelial marker, suggesting that the myoepithelial cells had transformed into sarcoma, and then this biphasic tumor was formed. Finally, she was diagnosed with biphasic metaplastic sarcomatoid carcinoma of the breast. Biphasic metaplastic sarcomatoid carcinoma of the breast is a relatively rare but aggressive disease. The pathological diagnosis is often controversial, requiring detailed immunohistochemical analysis. We report our experience with a case of biphasic metaplastic sarcomatoid carcinoma of the breast.

Vasculo-Behçet's Disease that Began with Femoral Arterial Pseudoaneurysm Followed by Deep Venous Thrombosis: Report of a Case.

Vasculo-Behçet's disease mainly affects the venous system and central or peripheral arteries. It is often difficult to diagnosis this due to the complexity of symptoms and the rarity. A 35-year-old man with refractory inguinal lymphadenitis was admitted to our hospital. He was diagnosed with left femoral arterial pseudoaneurysm by enhanced CT scan of the lower legs, and we performed an emergency operation. Seven months postoperatively, he suddenly developed deep venous thrombosis, and then, symptoms such as aphthous stomatitis and pudendal ulcer started to develop progressively, complicating his uveitis. Finally, he was diagnosed with vasculo-Behçet's disease.

Hemodynamic and humoral effects of atrial natriuretic peptide on pulmonary circulation after cardiac surgery.

PURPOSE: Human atrial natriuretic peptide (h-ANP) elicits biological effects such as natriuresis, diuresis, and vasodilation, and plays a role in regulating pulmonary circulation. We conducted this clinical study to define its role and elucidate its mechanisms. METHODS: Twelve consecutive adult patients scheduled to undergo cardiac surgery with cardiopulmonary bypass (CPB) were prospectively selected for this study. After the completion of surgery, h-ANP was infused from the right atrium through a Swan-Ganz (S-G) catheter. Blood samples for measurement of ANP and cyclic guanosine monophosphate (cGMP), the second messenger of ANP, were drawn from the pulmonary artery (PA) through the S-G catheter and from the left atrium (LA) through the left atrial pressure line, before and after the infusion of h-ANP. Hemodynamic values were measured at the same time. RESULTS: After the h-ANP infusion, the plasma levels of ANP were significantly lower in the LA than in the PA, whereas the plasma levels of cGMP were significantly higher in the LA than in the PA. The infusion of h-ANP decreased the mean PA pressure significantly, and the systolic PA pressure remarkably. CONCLUSION: The infusion of h-ANP after cardiac surgery stimulates the secretion of cGMP from the pulmonary vascular bed and dilates the PA, thereby decreasing the PA pressure.

Penetrating atherosclerotic ulcer in the juxtarenal abdominal aorta and coronary artery disease: emergency one-stage repair with off-pump coronary surgery.

An 82 year-old woman suddenly developed severe back pain. Enhanced computed tomography and aortography revealed penetrating atherosclerotic ulcer (PAU), that was a localized contrast-filled outpouching in the juxtarenal abdominal aorta and intramural hematoma within the aortic wall. Coronary angiography revealed significant stenosis in the left anterior descending artery and right coronary artery. Urgent aortic repair was required; therefore we performed the combined operations of coronary artery bypass grafting and aortic repair. PAUs typically occur in elderly patients with a history of hypertension, and are frequently complicated by coronary artery disease. However, few cases have been reported in the literature, such as cases involving combined operations. In this study, we report on a successful case of emergency repair involving concomitant juxtarenal abdominal aortic replacement for PAU and off-pump coronary artery bypass grafting.

Bicuspid aortic valve stenosis with single coronary artery.

A 56-year-old female with congestive heart failure was transferred to our institution. Aortography demonstrated aortic valve stenosis (AS) with a congenitally bicuspid valve and dilatation of the ascending aorta. Preoperative coronary angiography showed a left single coronary artery. Replacement of the aortic valve and ascending aorta was performed. She had an uneventful postoperative course. We report the case of aortic bicuspid valve stenosis with single coronary artery as an extremely rare congenital cardiac anomaly combination.

Papillary fibroelastoma of the mitral valve associated with rheumatic mitral valve stenosis.

With the advent of echocardiography, diagnosis of papillary fibroelastoma in living patients has been made possible, yet papillary fibroelastoma found in the living remains a very rare cardiac tumor. We report a case of papillary fibroelastoma of the mitral valve with rheumatic mitral valve stenosis. A 68-year-old woman was referred to our hospital with a mitral valve tumor and rheumatic mitral valve stenosis. She underwent anticoagulation therapy with Warfarin for 8 years since having a cerebral embolization. Echocardiography revealed a mass attached to the mitral valve, with severe mitral valve stenosis. Electrocardiography demonstrated a chronic atrial fibrillation. Tumor excision with mitral valve replacement and maze procedure were performed. Both the surgical and histological findings depicted papillary fibroelastoma. The postoperative course was uneventful and the patient has remained symptom-free one year after surgery.

Cardiac chondrosarcoma producing parathyroid hormone-related protein.

Chondrosarcoma is a malignant tumor characterized by the formation of cartilage. A case of primary cardiac chondrosarcoma of the left atrium developed in a middle-aged male. The preoperative serum concentrations of C-parathyroid hormone-related protein (PTHrP) and calcium were high (413.2 pmol/L and 12.2 mg/dl, respectively), but normalized after resection of the tumor, which measured 7 x 5 x 3.5 cm. The tumor was histopathologically diagnosed as chondrosarcoma, composed of outer atypical chondroid cells and inner pleomorphic and spindle mesenchymal cells mimicking malignant fibrous histiocytoma. Half of the cartilaginous tumor cells and a few pleomorphic cells showed cytoplasmic immunoreactivity for PTHrP. The tumor is a possible example of the functional pleiotropy of chondrosarcoma.