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.

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.

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.

OBJECTIVE: Diagnosis of sporadic cerebellar ataxia is a challenge for neurologists. A wide range of potential causes exist, including chronic alcohol use, multiple system atrophy of cerebellar type (MSA-C), and sporadic late cortical cerebellar atrophy. Recently, an autosomal-dominant spinocerebellar ataxia (SCA) mutation was identified in a cohort of patients with non-MSA-C sporadic cerebellar ataxia. The aim of this study is to genetically screen genes involved in SCA in a Japanese single-hospital cohort. METHODS: Over an 8-year period, 140 patients with cerebellar ataxia were observed. There were 109 patients with sporadic cerebellar ataxia (no family history for at least four generations, 73 patients with MSA-C, and 36 patients with non-MSA-C sporadic cerebellar ataxia) and 31 patients with familial cerebellar ataxia. We performed gene analysis comprising SCA1, 2, 3, 6, 7, 8, 12, 17, 31, and dentatorubro-pallidoluysian atrophy (DRPLA) in 28 of 31 non-MSA-C sporadic patients who requested the test. Familial patients served as a control. RESULTS: Gene abnormalities were found in 57% of non-MSA-C sporadic cerebellar ataxia cases. Among patients with sporadic cerebellar ataxia, abnormalities in SCA6 were the most common (36%), followed by abnormalities in SCA1 (7.1%), SCA2 (3.6%), SCA3 (3.6%), SCA8 (3.6%), and DRPLA (3.6%). In contrast, gene abnormalities were found in 75% of familial cerebellar ataxia cases, with abnormalities in SCA6 being the most common (29%). For sporadic versus familial cases for those with SCA6 abnormalities, the age of onset was older (69 years vs. 59 years, respectively), and CAG repeat length was shorter (23 vs. 25, respectively) in the former than in the latter (not statistically significant). CONCLUSION: Autosomal-dominant mutations in SCA genes, particularly in SCA6, are not rare in sporadic cerebellar ataxia. The reason for the frequency of mutations in SCA6 remains unclear; however, the reason may reflect a higher age at onset and variable penetrance of SCA6 mutations.
Age of Onset ; Asian Continental Ancestry Group* ; Atrophy ; Cerebellar Ataxia ; Cohort Studies* ; Diagnosis ; Family Characteristics ; Genetic Testing* ; Heredity ; Humans ; Multiple System Atrophy ; Penetrance ; Spinocerebellar Ataxias*

Androgens play a central role in prostate cancer pathogenesis, and hence most of the patients respond to androgen deprivation therapies. However, patients tend to relapse with aggressive prostate cancer, which has been termed as hormone refractory. To identify the proteins that mediate progression to the hormone-refractory state, we used protein-chip technology for mass profiling of patients' sera. This study included 16 patients with metastatic hormone-refractory prostate cancer who were initially treated with androgen deprivation therapy. Serum samples were collected from each patient at five time points: point A, pre-treatment; point B, at the nadir of the prostate-specific antigen (PSA) level; point C, PSA failure; point D, the early hormone-refractory phase; and point E, the late hormone-refractory phase. Using surface-enhanced laser desorption/ionization time-of-flight mass spectrometry, we performed protein mass profiling of the patients' sera and identified a 6 640-Da peak that increased with disease progression. Target proteins were partially purified, and by amino acid sequencing the peak was identified as a fragment of apolipoprotein C-I (ApoC-I). Serum ApoC-I protein levels increased with disease progression. On immunohistochemical analysis, the ApoC-I protein was found localized to the cytoplasm of the hormone-refractory cancer cells. In this study, we showed an increase in serum ApoC-I protein levels in prostate cancer patients during their progression to the hormone-refractory state, which suggests that ApoC-I protein is related to progression of prostate cancer. However, as the exact role of ApoC-I in prostate cancer pathogenesis is unclear, further research is required.
Aged ; Amino Acid Sequence ; Antineoplastic Agents, Hormonal ; therapeutic use ; Apolipoprotein C-I ; analysis ; blood ; isolation & purification ; Blotting, Western ; Cell Line ; Disease Progression ; Drug Resistance, Neoplasm ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Molecular Sequence Data ; Prognosis ; Prostatic Neoplasms ; drug therapy ; metabolism ; Protein Array Analysis ; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

OBJECTIVE: Reports on the repeated administration of medroxyprogesterone acetate (MPA) for intrauterine recurrence after fertility-preserving therapy for atypical endometrial hyperplasia (AEH) and early grade 1 endometrioid carcinoma (G1) are lacking. We aimed to clarify the outcomes of repeated MPA therapy in cases of intrauterine recurrence after fertility-preserving therapy with MPA against AEH/early G1. METHODS: Patients with AEH or stage IA well-differentiated endometrioid carcinoma without myometrial invasion who underwent first-line MPA therapy for primary lesions or intrauterine recurrence were divided into initial treatment and repeated treatment groups (162 and 82 patients, respectively). Oral MPA administration (400−600 mg/day) was continued until pathological tumor disappearance. Data regarding clinicopathological factors, adverse events, and outcomes following the initial and repeated hormonal treatments were extracted from medical records and analyzed. RESULTS: Complete response rates in the initial and repeated treatment groups were 98.5% and 96.4%, respectively, among patients with AEH, and were 90.7% and 98.1%, respectively, among patients with G1. In the initial treatment group, 5-year recurrence-free survival (RFS) rates were 53.7% and 33.2% among patients with AEH and G1, respectively. In the repeated treatment group, RFS rates were 14.0% and 11.2% among patients with AEH and G1, respectively. Among patients with AEH, the pregnancy rate tended to be lower in the repeated treatment group than in the initial treatment group (11.1% vs. 29.2%; p=0.107), while no significant group difference was observed among patients with G1 (20.8% vs. 22.7%). CONCLUSION: Repeated treatment is sufficiently effective for intrauterine recurrence after hormonal therapy for AEH/early G1.
Carcinoma, Endometrioid ; Endometrial Hyperplasia* ; Endometrial Neoplasms* ; Female ; Fertility Preservation ; Fertility* ; Hormone Replacement Therapy ; Humans ; Medical Records ; Medroxyprogesterone Acetate* ; Medroxyprogesterone* ; Pregnancy Rate ; Recurrence

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.

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 79-year-old woman presented to our hospital with high energy trauma. Enhanced CT revealed injury to the aortic arch. The left carotid artery was pulled out due to extension force and a drawing out lesion formed. Cardiopulmonary bypass was established with cannulation of the right femoral artery and the right atrium, and systemic cooling was started. We opened the aortic arch with deep hypothermic circulatory arrest, and detected a 10 mm drawing out lesion at the bottom of the left carotid artery. Aortic arch was transected at the distal of the left carotid artery to exclude the drawing out lesion, and partial arch replacement was performed. The patient's postoperative course was uneventful, and she was discharged from our hospital without any complication.