A 72-year-old woman, who had been treated for autoimmune hemolytic anemia with prednisolone and azathioprine since 2002, was found to have mild aortic stenosis in 1994. In December 2003, she suffered congestive heart failure, and was on temporary mechanical ventilation. In February 2004, the maximum pressure gradient between left ventricle and aorta increased to 115.8mmHg on echocardiographic examination. On April 6, aortic valve replacement was carried out with a 19mm bioprosthesis (Carpentier-Edwards PERIMOUNT^®, Edwards Lifesciences, Irvine, California). Preoperative prednisolone administration was continued until the day of the operation. Four packs of washed red blood cells were transfused intraoperatively and four packs of red blood cells were transfused postoperatively. Before transfusion, haptoglobin and water-soluble prednisolone were administrated to prevent hemolysis. Oral prednisolone and azathioprine were reestablished on the third postoperative day. Her postoperative course was uneventful and she did not suffer either infection or hemolysis. She was discharged on the 30th postoperative day.

We report a case of a 24-year-old woman who presented with orthopnea, in whom an echocardiographic exam showed a very large mass in the left atrium. We diagnosed this as cardiac failure due to the tumor occupying it. Although the tumor malignancy remained unclear, we had to perform emergency surgery to excise the tumor. The tumor was excised in its entirety, including the interatrial septum and a large segment of the left atrial wall. We reconstructed them with the autologous pericardium. The pathological diagnosis was undifferentiated pleomorphic sarcoma. Conventional adjuvant chemotherapy and radiotherapy was performed. Primary cardiac malignant tumor prognosis is very poor, but she has survived over 1 year without recurrent symptoms after complete excision and adjuvant therapy. In addition to reporting this case, we discussed the diagnosis and treatment of undifferentiated pleomorphic sarcoma.

We describe successful surgical treatment of a right coronary artery aneurysm associated with a fistula to the right atrium (RA). The patient was a 50-year-old man who complained of palpitations. ECG showed supraventricular extrasystole, and coronary CT revealed a remarkably dilated and undulating fistulous tract originating from the region corresponding to the orifice of the normal right coronary artery (RCA). The fistulous tract detoured to the posterior wall of the RA. An RCA of normal size originating from the midway of the fistulous tract was observed. The patient was operated on under cardio-pulmonary bypass. An aortocoronary bypass was performed, using a radial artery graft to section of the RCA that had a normal diameter. The RCA was subsequently ligated at the proximal side of the anastomosis. The orifice of the fistulous tract from the aorta was closed with a patch, and the entrance to the RA was also closed with mattress sutures. The postoperative recovery was uneventful, and he was discharged on the 19th postoperative day. Currently, the patient has been doing well without any complaints at 2 years postoperatively.

This study compared the hemodynamic performance of the Carpentier-Edwards PERIMOUNT Magna bioprosthesis (Magna) with the Carpentier-Edwards PERIMOUNT bioprosthesis (CEP) for aortic valve stenosis (AS). Between January 2005 and May 2010, 164 patients underwent aortic valve replacement for AS with either the Magna (n=68) or the CEP (n=96) at our institute. Patients undergoing a concomitant mitral valve procedure were excluded from this study. The 21-mm Magna and CEP prostheses were the most frequently used during this period. Transthoracic echocardiography was postoperatively performed within 2 weeks. The peak velocity (PV) of the Magna was significantly lower than that of the CEP (2.59±0.36 vs. 2.75±0.47 m/s ; p=0.022). The mean pressure gradient (PG) was not significantly different. For the 19-mm prostheses, the mean PG and PV of the Magna were significantly lower than those of the CEP [16.4±4.5 vs. 19.7±6.4 mmHg ; p=0.034 (PG) and 2.70±0.36 vs. 3.03±0.49 m/s ; p=0.008 (PV)]. The effective orifice area (EOA) of the Magna was larger than that of the CEP [19 mm : 1.29±0.18 vs. 1.11±0.24 cm² (p=0.007) ; 21 mm : 1.46±0.23 vs. 1.42±0.18 cm² (p=0.370) ; and 23 mm : 1.70±0.34 vs. 1.52±0.25 cm² (p=0.134)]. In this study, the EOA of the Magna was approximately 80% of that described in the manufacture's description. Patient-prosthesis mismatch (PPM ; EOA index≤0.85 cm²/m²) was seen in 26.8% of patients with the Magna and in 47.2% of patients with the CEP (p=0.018). Severe PPM (EOA index≤0.65 cm²/m²) was not seen in any patients with the Magna. The EOA of the 19-mm Magna was significantly larger and the mean PG was lower than those of the 19-mm CEP. Compared with the CEP, the Magna significantly reduced the incidence of PPM, and had superior hemodynamic performance.

Acute aortopulmonary artery fistula is a rare but potentially fatal disorder. We encountered a case in which this disorder was successfully treated by urgent total arch graft replacement and repair of the left pulmonary artery. A 74-year-old man was referred to Shizuoka City Hospital with a 2-day history of worsening dyspnea and thoracic aortic aneurysm. The patient had a history of hypertension and dyslipidemia. Physical examination showed diastolic hypotension, marked peripheral coldness, and systolic murmur. Arterial blood gas analysis showed severe metabolic acidosis with base excess of −16 mmol/l. Contrast-enhanced computed tomography (CT) revealed an aortic arch aneurysm on the lesser curvature, almost obstructing the left pulmonary artery. A Swan-Ganz catheter study confirmed severe low-output syndrome and uncompensated congestive heart failure. After amelioration of critically ill conditions with dopamine, milrinone, and carperitide, oxymetry revealed significant left-to-right shunt with Qp/Qs＝3.2 at the pulmonary artery level. Acute aortopulmonary artery fistula was diagnosed and urgent surgery was planned. Transesophageal echocardiography showed systolic shunt flow from the aneurysm into the left pulmonary artery. Surgery was performed through a median sternotomy. Aortic arch graft replacement with a 24-mm Dacron graft and repair of the left pulmonary artery with an equine pericardial patch were accomplished under hypothermic circulatory arrest and selective antegrade cerebral perfusion. Flooding of pulmonary circulation until circulatory arrest was prevented by manual control through the main pulmonary artery incision. Postoperative recovery was uneventful, and the patient is doing well at one year postoperatively.

A 51-year-old man was referred to our hospital with pain and coldness of the upper left extremity. Contrasted computed tomography revealed a silhouette protruding into the aortic arch. Peripheral embolism in upper left extremity by tumor or thrombosis was suspected. Magnetic resonance imaging revealed a mobile mass in the aortic arch. To prevent recurrent embolization, the mass and the aortic arch to which the mass was attached were excised and partial arch replacement was performed under cardiopulmonary bypass. Histologically, the mass was a fibrin thrombus with no malignancy. The aortic wall showed only mild atherosclerosis of the intima. No thrombotic predisposition such as protein S or C deficiency or antiphospholipid antibody syndrome was observed. Anticoagulant therapy was started and the patient was discharged on postoperative day 10 without recurrent thromboembolism. Three years have passed since the operation and there is no recurrence of thromboembolism.

The patient was a 34-year-old woman who had been routinely monitored after receiving a childhood diagnosis of partial anomalous pulmonary venous connection, but unilaterally discontinued follow-up examinations after the age of 18. At 33 years of age, she was admitted to our hospital after a physical examination revealed an abnormal shadow on a chest X-ray. Transthoracic echocardiography detected an atrial septal defect (ASD), and contrast-enhanced computed tomography showed that the right lower pulmonary vein drained to the inferior vena cava. The patient was diagnosed with scimitar syndrome with ASD. Cardiac catheterization showed a pulmonary/systemic flow ratio (Qp/Qs) of 2.48 and a left-to-right shunt rate of 59.7%. Surgical treatment was deemed to be indicated. The right lower pulmonary vein was anastomosed to the anterolateral wall of the right atrium, and an intra-atrial baffle repair was performed from the orifice within the right atrium to the left atrium through the existing ASD using untreated fresh autologous pericardium. Two years after the operation, good blood flow was maintained within the baffle with no stenosis at the anastomotic site. This report describes a rare case of scimitar syndrome with ASD in an adult woman, and provides a review of the existing literature.

An 87-year-old man underwent a transcatheter aortic valve implantation (TAVI) for severe aortic stenosis. Approximately 8 months later, he was readmitted to our institution because of a cerebral infarction. Viridans Streptococcus was identified from the blood culture, and transesophageal echocardiography revealed a mobile mass on the leaflet. Prosthetic valve endocarditis (PVE) was diagnosed and we initially administered intravenous antibiotic therapy for 4 weeks, after which the patient underwent surgical aortic valve replacement. Herein, we report on the surgical AVR in the patient using a pericardial valve after successful removal of the infected prosthetic valve, and discuss some issues related to this rare complication after TAVI.

A 32-year-old woman was diagnosed with Takayasu's arteritis 5 years ago and underwent aortic valve replacement for aortic regurgitation 1 year ago. She had been taking Prednisolone and Azathioprine for Takayasu's arteritis, but these drugs were switched to subcutaneous Tocilizumab 4 months ago. One month ago, she had dyspnea on exertion, and 2 days ago, chest discomfort appeared, and she came to our hospital. Blood tests showed CRP 0.02 mg/dl, and echocardiography and CT showed perivalvular leakage and aortic root pseudoaneurysm, which led us to suspect aortic root pseudoaneurysm due to Takayasu's arteritis and to perform emergency surgery. Although a circumferential pseudoaneurysm was observed at the aortic root, no destruction of the prosthetic valve was observed. The suture from the previous surgery was attached to the sawing cuff of the prosthetic valve, and the prosthetic valve was not fixed to the aortic annulus and could be easily removed. The Bentall operation was performed using a bioprosthetic valve. The histopathological diagnosis was subacute infective endocarditis, and the patient was diagnosed with a pseudoaneurysm of the aortic root due to infection. The patient had a good postoperative course and was discharged home on the 19th day. We report a case of Takayasu's arteritis with valve annular abscess after AVR, which was treated surgically during biologic drug administration.