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The proband was a male in his seventies who came to our facility because of shortness of breath. He was not anemic but presented dissociation between oxygen saturation (SpO2) and partial pressure of oxygen (PaO2) by blood gas analysis, and also demonstrated hemoglobinopathy after measurement of Hb A1c using high performance liquid chromatography (HPLC). Twenty-three percent of unknown hemoglobin (Hb) bands were detected. After sequencing the β-globin gene, we noted a missense mutation at codon 74 (GGC>CGC) (Gly→Arg) of the β-globin chain and he was diagnosed with Hb Aalborg (HBB: c.223G>C). One of the proband's siblings was diagnosed to have a low SpO2 level and also diagnosed to carry Hb Aalborg; she was also mildly anemic. This is the first known familial case of Hb Aalborg in Japan. In addition to Hb Aalborg, our case had underlying chronic obstructive pulmonary disease (COPD). Herein we present this case as a rare addition to the hematological literature.

The proband was a male in his seventies who came to our facility because of shortness of breath. He was not anemic but presented dissociation between oxygen saturation (SpO

Aortoesophageal fistula (AEF) is relatively rare and usually life-threatening. Lots of strategies have so far been discussed for this entity including the role of endovascular repair. The aim of this study is to review our experiences and reconsider the surgical strategy for aortoesophageal fistula in the endovascular era.This is a retrospective multicenter study. From 1995 to 2011, 10 aortoesophageal fistula cases were identified in four institutions. For all of these cases surgical procedures and results were retrieved from medical records.Six patients underwent open aortic repair and four patients underwent thoracic endovascular aortic repair (TEVAR) as a primary intervention. Three patients who underwent open aortic repair with esophagectomy and omental coverage in early phase, either as a primary intervention or performed after bridging TEVAR, showed 100 % 1-year survival. On the other hand, three patients with TEVAR alone did not survive more than 1 year without recurrence. One patient with bridging TEVAR underwent concomitant esophageal resection and conventional aortic graft replacement 2 days later, and simultaneous gastric tube reconstruction was performed with intact whole omentum covering the aortic prosthesis. This patient is doing well with no sign of infection at 1-year follow-up.For AEF, TEVAR as a primary approach is quite useful to stabilize the patients' condition. However, definitive aortic repair with omental coverage should be performed as early as possible as a next step. It may be one of the strategies for the treatment of AEF that concomitant esophageal resection and aortic graft replacement is performed with simultaneous gastric tube reconstruction with intact whole omentum after removing the stent graft, so far as the patient's physical condition permits.

症例は64歳女性．2005年より完全右脚ブロックにて他院にてフォローされていたが，2007年7月より心不全症状が出現し，精査にて心サルコイドーシスの診断となる．UCGにて心室中隔基部の菲薄化と右室側への瘤化の進行を認めたため当科を紹介された．UCG上EF40%と壁運動の低下を認めたが，特に中隔基部の右室側へのdyskinesisが心不全の原因と考え手術施行した．右房，右室，大動脈切開にて菲薄化部分を特定し，これを切除したのち，4×3 cmのダクロンパッチにて修復した．完全房室ブロックに対しては両室ペーシングができるように右室と左室後面にリードを置いたDDDペースメーカーを移植した．術後経過は良好であった．病理所見では正常心筋と菲薄化した部分の境界部に非乾酪性類上皮肉芽腫を認め，心サルコイドーシスの診断であった．心サルコイドーシスの外科治療はまれであり，特に中隔切除を必要とする場合外科手技上種々の問題を有するため報告する．

A 24-year-old man with Ebstein anomaly underwent a bidirectional Glenn shunt and closure of an atrial septal defect. Postsurgical prulent mediastinitis was treated by irrigation and drainage, but was followed by rupture of the ascending aorta. During emergency surgery, hypothermic circulatory arrest became necessary due to massive bleeding. Since he had under-gone a bidirectional Glenn shunt, left heart venting was essential to obtain deep hypothermic circulatory arrest without cardiac distention and was successfully performed via an anterior thoracotmy approach. The perforated site of the ascending aorta was repaired with a Xeromedica® patch. The anterior mediastinum was wrapped with the omentum. Transthoracic left heart venting via an anterior thoracotomy is an useful approach when hypothermic circulatory arrest is required to perform a median sternotomy and to approach the heart.

To compare the surgical results of patients with ruptured (rAAA) and nonruptured abdominal aortic aneurysms (NrAAA), 267 consecutive patients surgically treated for abdominal aortic aneurysms (AAA) were reviewed. The patients' characteristics, preexistent risk factors, perioperative factors, and postoperative early and long-term survival were compared between the rAAA group (n = 27) and the NrAAA group (n = 240). A multivariate analysis to predict postoperative survival was also conducted in the rAAA group. The hospital mortality rate was 3.3% (8/232) for the NrAAA group and 22.2% (6/27) for the rAAA group (P0.001). The maximum size of aneurysms, period of preoperative hypotension, and intraoperative bleeding volume were significantly higher in the rAAA group than in the NrAAA group. The 5- and 10-year cumulative survival rates in the rAAA group were 88.1% and 42.0%, which were comparable to those in the NrAAA group. The incremental risk factors for hospital death in the rAAA group included advanced age, preoperative hypotension (80 mmHg), and postoperative renal failure requiring dialysis. These findings showed that the interval from rupture to cross-clamping must be shortened, maintaining hemodynamic stability to avoid prolonged hypotension. Reducing risk factors and minimizing deterioration of organ functions postoperatively would be essential to improve the prognosis of patients with rAAA.

A total of 274 patients with abdominal aortic aneurysms due to atherosclerosis (AAA) and 16 patients with inflammatory abdominal aortic aneurysms (IAAA) were reviewed to compare and contrast the clinical characteristics of the 2 groups. The AAA group comprised 243 men and 31 women with a mean age of 69.2 +/- 0.4 (range 51-86) years. The IAAA group comprised 15 men and 1 woman with a mean age of 67.4 +/- 2.0 (range 53-81) years. Most patients with IAAA (12/16; 75.0%) had pain at presentation, whereas only 37 out of 274 patients (13.5%) with AAA had pain (p < 0.001). Fifty out of 274 patients (18.2%) with AAA were asymptomatic, the most common principal complaint being a pulsatile tumor, which was found in 150 out of 274 patients (54.7%; p < 0.005 vs IAAA). Regarding laboratory findings of inflammation, preoperative erythrocyte sedimentation rate values were elevated in 15 out of 16 (93.8%) patients, and C-reactive protein values were elevated in 13 out of 16 (81.3%) patients with IAAA. The incidence of perioperative complications was similar in the 2 groups. The 30-day postoperative mortality among AAA patients was 6.2% (17/274 cases), including 12 cases of non-ruptured and 5 cases of ruptured AAA; in contrast, no early deaths occurred among patients with IAAA. The cumulative 5-year survival rate was 80.2% for IAAA patients and 74.6% for AAA patients (NS). The results of our review suggest that careful diagnosis and intra- and postoperative management could lead to patients with IAAA having a similar survival rate to those with AAA.

Immunoglobulin (Ig) G4-related disease is a novel disease entity characterized by diffuse lymphoplasmacytic infiltrates and the presence of abundant IgG4-positive plasma cells in extensive fibrosis, frequently associated with tumorous swelling lesion and elevated serum IgG4 concentrations. Immunoglobulin G4-related disease has been described in almost every organ system but rarely affects the heart. We describe a rare case of IgG4-related disease of the heart causing aortic regurgitation and heart block in a 59-year-old woman. The excised lesion revealed a high ratio of IgG4-positive to IgG-positive plasma cells, providing a definite diagnosis of IgG4-related disease. The aortic valve was replaced using Manouguian technique, resulting in a favorable outcome.

J Thorac Cardiovasc Surg 2002;124:395-7

Tumor necrosis factor-alpha (TNF-alpha) release has been implicated in a sepsis-like syndrome following cardiopulmonary bypass (CPB). This also may be important in patients who have had a left ventricular assist device (LVAD) implanted. This report investigates the effect of reducing systemic blood flow on hemodynamic response, mixed venous oxygen saturation (SvO(2)), and the release of TNF-alpha. LVADs were implanted in 9 pigs. The aorta was clamped, and thus the LVAD flow represented the entire systemic blood flow. Plasma TNF-alpha in the femoral artery (FA) and superior mesenteric vein (SMV) was measured at baseline and following systemic blood flow changes. Simultaneously, hemodynamic parameters and oxygen saturation in the pulmonary artery (SvO(2)) were measured. Following reductions in systemic blood flow, plasma TNF-alpha increased gradually to a maximum level at a systemic blood flow of 20%. There was no significant difference between TNF-alpha levels in the SMV and the FA. There was a significant (p < 0.05) correlation between cardiac index, stroke volume index, and TNF-alpha. The SvO(2) decreased significantly (p < 0.05) at a systemic blood flow of 30 and 20%. A rise in TNF-alpha occurred when the SvO(2) was less than 75%. The data demonstrate that a reduction in systemic blood flow causes an increase in plasma TNF-alpha. This can lead to the development of a sepsis-like syndrome in a group of patients who already are hemodynamically compromised. While weaning short-term LVAD support, rapid diminution of the cardiac output and the pump flow must be avoided.

We previously demonstrated that tumor necrosis factor-alpha (TNF-alpha) increased following a reduction in systemic blood flow to 60% or less of the original cardiac output using a left ventricular assist device (LVAD). The aim of this study was to investigate the effect of reducing systemic blood flow on tissue oxygenation in the gastrointestinal tract (GIT) and the consequences of this on TNF-alpha release. LVADs were implanted in 9 pigs. The aorta was clamped, and thus the LVAD flow represented the entire systemic blood flow. Plasma TNF-alpha of the superior mesenteric vein was measured at baseline and during systemic blood flow changes. Simultaneously, pH, lactate, oxygen delivery index (DO(2)I), oxygen consumption index (VO(2)I), and oxygen extraction (O(2)ER) in the GIT were measured. The pH decreased and the lactate level increased significantly (p < 0.05) at a systemic blood flow of 50% or less. The VO(2)I was positively correlated with DO(2)I. The O(2)ER increased significantly (p < 0.05) with reductions in systemic blood flow to 30% or less. There was a significant (p < 0.01) correlation between TNF-alpha and O(2)ER at levels higher than 55%. These data demonstrate that the GIT oxygenation is inadequate with a reduction in systemic blood flow to 50% and that GIT oxygenation becomes critical at a reduction of 30%. During LVAD weaning, careful attention must be given to the GIT. The pH and lactate may be good markers of the adequacy of tissue oxygenation in the GIT.

Local delivery of angiogenic growth factors for the treatment of myocardial ischemia has been well documented in various animal models, and clinical trials are now in progress. Our strategy was radically different, based on selective protection of some of the growth factors naturally present within the injured tissue. This protection was obtained by applying a chemically defined substitute for Dextran called RGTA11 (for ReGeneraTing Agent). RGTA is a family of agents, which has properties mimicking those of heparan sulfates toward heparin-binding growth factors (HBGF) and which stimulate tissue repair and protection. Indeed, we have previously shown that RGTA prevents most of the damage resulting from acute skeletal muscle ischemia [FASEB J. (1999) 13, 761-766]. We now show that the same agent can be used for the treatment of myocardial infarction. Acute myocardial infarction was induced in pigs by ligation of the left circumflex artery. One hour later, a single injection of 10 mg of RGTA11 was made in the center of the infarcted area. Three weeks later we observed 1) recovery of 84% of the initial left ventricular ejection fraction (only 55% in saline-treated controls), 2) an almost 50% reduction in the infarct size, 3) a reduction in fibrotic tissue formation, 4) significant preservation of myocytes, and 5) an increase in the number of blood vessels. The treatment of ischemic heart disease with RGTA would have clear advantages over other therapies such as growth factor, gene, or cell transplants, based on a stable, simple, and easy-to-develop chemical product.

In their experimental approach to dynamic cardiomyoplasty (DCMP), the authors hypothesized that a wrapping method using the proximal part of a free latissimus dorsi muscle (LDM) graft might augment ventricular contractility more than the classic Carpentier's wrapping method. The authors divided 12 mongrel dogs into a pedicled LDM graft group (Group 1, n = 6) and a free LDM graft group (Group 2, n = 6) to evaluate the properties of the different wrapping methods. To evaluate the effect of DCMP on left ventricular systolic and diastolic function, the authors used precise indicators that minimize the influences of load conditions. The slope of the linear preload recruitable stroke work relationship (Mw) and an X-intercept (Vo) were utilized as the indicator of left ventricular systolic function. The constants of pressure decay (tau) and peak filling rate (PFR) were measured to determine diastolic function. All experimental animals were evaluated with the acute, non preconditioning model. Mw was significantly increased with LDM stimulation in both groups (postwrap non stimulation 59.1 +/- 6.3; postwrap stimulation 98.6 +/- 9.7 erg.cm-3. 10(3); p0.01 in Group 1, postwrap non stimulation 66 +/- 6.7; postwrap stimulation 155 +/- 15.7 erg.cm-3.10(3), p0.001 in Group 2). Stimulated free LDM grafts significantly increased the Mw in comparison to pedicled grafts (p = 0.011). Vo was unchanged in both groups and there was no significant difference between the two groups. Tau increased after LDM wrap in both groups (p0.05), but there was no difference between the two groups (tau; prewrap 45.8 +/- 6.0; postwrap non stimulation 69.3 +/- 10.3; postwrap stimulation 72.3 +/- 13.9 msec in Group 1, prewrap 50.0 +/- 6.0; post wrap non stimulation 61.8 +/- 5.0; post wrap stimulation in 64.3 +/- 4.7 msec in Group 2). Peak filling rate was unchanged after LDM wrap in both groups. Free LDM grafts significantly increased left ventricular systolic function compared to the pedicled LDM. Although myocardial relaxation was impaired after LDM wrap in both groups, there was no difference between the two groups. The authors' results emphasize the explicit benefit of utilization of a free LDM graft in respect to left ventricular systolic function; the free LDM grafts have no gross detrimental effect on diastolic function when compared with a pedicle graft in the acute model.

Study on the growth of CaWO4 (Scheelite) needle crystals by chemically reacted flux method was made by means of slow cooling of CaWO4 from KCl fluxed melt.A mixture of CaClex H2O, WON, K2CO3, and KCl was heated in a platinum crucible in an electric furnace at a temperature of 1000 or 1150°C for 10 hours and then gradually cooled to 700°C at a cooling rate of 5°C/hr. CaWO4 could be produced as follows: CaCl2, H2O + WO8 + K2C08 + y KCl → CaWO4 + (2+y) KCl +xH2O↑ + CO2↑During cooling CaWO4 crystals have been formed.Results obtained are as follows: (1) On cooling from a temperature of the liquid consisting of CaWO4-KCl, whose region being shown in the phase diagram, CaWO4 needle crystals were formed. (2) The needle crystal, having maximum length of about 2 mm, could be formed. The needle crystals had a silky luster and their surfaces were smooth. The growth direction of the needle crystal was considered to be or . (3) The maximum growth rate of needle crystal was about 16 × 10-3 p/sec on the basis of suitable assumptions.