Paget-Schroetter syndrome accompanied by pulmonary thromboembolism: A case report.

A 17-year-old male, who was involved in a baseball club, presented to our emergency department with the complaint of gradual onset of swelling of his right arm. Contrast-enhanced computed tomography showed obstruction of the proximal portion of the right subclavian vein and pulmonary thromboembolism. Venography confirmed an occluded right subclavian vein. The patient was diagnosed with right subclavian vein thrombosis, which is referred to as Paget-Schroetter syndrome (PSS). An ultrasonography for the affected subclavian vein was helpful not only for making an accurate diagnosis of PSS, but also for verifying dynamic venous flow changes depending on the forearm position. .

Accelerated Atherogenicity in Tangier Disease.

We report a case of Tangier disease with Leriche syndrome and bleeding tendency. In this male patient, nasal hemorrhage had been observed frequently throughout childhood. At 46 years old, he experienced effort angina, and coronary angiography demonstrated 75% stenosis in the right coronary artery. Orange-colored tonsils, mild hepatosplenomegaly and very low levels of serum high-density lipoprotein cholesterol (HDL-C) were observed, and the patient was diagnosed with Tangier disease. At 52 years old, effort angina recurred. Coronary angiography revealed 75% stenosis of the left main trunk, left anterior descending, and right coronary arteries. Stenosis of the brachiocephalic and right common iliac arteries was also recorded. Stents were implanted, and coronary artery bypass surgery was performed. At 53 years old, 15 months after surgery, the patient reported intermittent claudication, coldness of feet, and impotence. Aortic angiography showed progression of the stenosis at the bifurcation of the common iliac artery. The patient was diagnosed with Leriche syndrome, and aorta-left external iliac artery graft bypass surgery was performed. After surgery, oozing from subcutaneous tissue and leaking from the anastomotic region were observed. Additional analysis revealed two single-nucleotide polymorphisms (V825I and N935T) in the ATP-binding cassette transporter A1 (ABCA1) gene, and accumulation of small dense low-density lipoprotein together with low levels of HDL-C. In Tangier disease, HDL-C is markedly decreased because of ABCA1 deficiency. However, this is the first reported case to exhibit extensive atherosclerosis and bleeding tendency. This patient had atypical extensive and multiple atherosclerotic lesions, accompanied by Leriche syndrome and uncontrollable bleeding.

Relation of estimated glomerular filtration rate to the presence of coronary plaque and obstructive coronary artery disease in a zero or low coronary artery calcium score.

OBJECTIVES: Although renal dysfunction is associated with the presence of atherosclerosis, little is known about the relationship between reduced estimated glomerular filtration rate (eGFR) and the presence of atherosclerosis detected by coronary computed tomographic angiography (CCTA). This study evaluated the relation of eGFR to the presence of coronary plaque and obstructive coronary artery disease (CAD) in patients with a zero or low coronary artery calcium score (CACS). METHODS: Coronary artery calcium scoring and CCTA were performed with CT scanners. Serum creatinine was measured before CCTA, and GFR was estimated. A total of 720 patients with a CACS ≤ 10 were enrolled. RESULTS: Coronary plaque was detected in 118 patients. Of the 118 patients, 36 had a diagnosis of obstructive CAD. The multiple-adjusted odds ratios of presenting with coronary plaque and obstructive CAD were 1.82 (95% CI 1.06-3.12, p = 0.030) and 1.79 (95% CI 0.71-4.49, p = 0.217) for the lowest tertile of eGFR compared with the highest tertile, respectively. CONCLUSIONS: Lower eGFR levels were associated with the presence of coronary plaque in patients with a zero or low CACS. However, the association between eGFR and the presence of obstructive CAD was not statistically significant.

Novel combined GPIHBP1 mutations in a patient with hypertriglyceridemia associated with CAD.

AIM: Lipoprotein lipase (LPL) deficiency is a rare autosomal recessive disorder characterized by severe hypertriglyceridemia. Similar clinical phenotypes have been reported with respect to defects in several LPL-associated proteins. However, it remains controversial whether severe hypertriglyceridemia itself is atherogenic. We herein present a case of LPL deficiency due to novel combined mutations of glycosylphosphatidylinositol (GPI)-anchored high-density lipoprotein (HDL)-binding protein 1 (GPIHBP1) in a patient with coronary artery disease (CAD). PATIENT: We evaluated a 54-year-old woman with severe hypertriglyceridemia and double vessel CAD. Although the LPL mass and activity in the postheparin plasma were extremely low, no mutations were detected in the LPL gene itself. RESULTS: Genetic analyses revealed that the patient had double homozygous mutations at 41 bp (c.41 G > T) and 202 bp (c.202 T > C) in the GPIHBP1 gene, resulting in C14F and C68R, respectively. Although the C14F/C68R GPIHBP1 exhibited a normal LPL-binding activity, the levels of mutant proteins were extremely reduced compared to those of the wild-type proteins in vitro. CONCLUSION: We found novel combined mutations of GPIHBP1 in a patient with hypertriglyceridemia and severe CAD. The present case provides important insight into the pathogenesis of severe hypertriglyceridemia associated with atherosclerosis.

Takotsubo Cardiomyopathy in Two Patients without Any Cardiac Symptom on Maintenance Hemodialysis.

Takotsubo cardiomyopathy is a disorder characterized by left ventricular apical ballooning and electrocardiographic changes in the absence of coronary artery disease. While reversible in many cases, the mechanism of this disorder remains unclear. The most frequent clinical symptoms of takotsubo cardiomyopathy on admission are chest pain and dyspnea, resembling acute myocardial infarction. Here, we describe two cases of takotsubo cardiomyopathy without chest pain or dyspnea in patients on maintenance hemodialysis. The asymptomatic nature of these two cases may be due to the patients being on hemodialysis. Periodic electrocardiograms (ECG) may be helpful in screening this population for asymptomatic takotsubo cardiomyopathy and in evaluating its incidence.

Relation of cardiovascular risk factors and angina status to obstructive coronary artery disease according to categorical coronary artery calcium score.

Coronary artery calcium (CAC) is associated with the presence of coronary artery disease (CAD) and cardiovascular risk factors. However, the relation between cardiovascular risk factors and CAD has not yet been fully elucidated in patients with a zero or low coronary artery calcium score (CACS). The purpose of this study was to evaluate the relation of cardiovascular risk factors and angina status to obstructive CAD according to categorical CACS. A total of 753 patients were enrolled in this study. CAC scoring and coronary computed tomographic angiography (CCTA) were performed with dual-source 64-slice CT scanners. The number of patients with a CACS ≤10 and ≤100 were 358 and 528, respectively. Patients with a higher CACS were older and more frequently male, and had a greater frequency of hypertension, diabetes, and hypercholesterolemia. The prevalence of obstructive CAD increased with the CACS. Among patients with a CACS ≤100, age, male gender, diabetes, hypercholesterolemia, and typical angina pectoris were related to obstructive CAD. The presence of hypercholesterolemia was relatively strongly associated with obstructive CAD (OR 6.67, 95% CI 2.91-15.3, p < 0.001) on multivariate analysis. Among patients with a CACS ≤10, men, hypercholesterolemia, and typical angina pectoris were significantly more frequent in patients with than in those without obstructive CAD (p < 0.01). Our data suggest that neither the absence nor low of coronary calcium burden may reliably exclude obstructive CAD in typical symptomatic male patients with hypercholesterolemia. This result may be useful to interpret the relation of CACS to obstructive CAD.

An autopsy case of left ventricular apical ballooning probably caused by pheochromocytoma with persistent ST-segment elevation.

We describe the autopsy case of an 86-year-old man who experienced left ventricular (LV) apical ballooning with pheochromocytoma. During the follow-up period, his electrocardiogram (ECG) showed persistent ST-segment elevation in leads V3 to V6, and an echocardiogram revealed persistent LV dysfunction in the apical region. He died 64 days after admission. Pathological findings suggested catecholamine-induced cardiomyopathy and pheochromocytoma. This is the report of a rare autopsy case of LV apical ballooning.

Glycosylated hemoglobin is a predictor of major adverse cardiac events after drug-eluting stent implantation in patients with diabetes mellitus.

OBJECTIVES: Diabetes mellitus is associated with the risk of restenosis and mortality after coronary stenting, but the relation between glycosylated hemoglobin (hemoglobin A1c) and prognosis has not yet been fully elucidated in patients with diabetes mellitus. The purpose of this study was to evaluate whether hemoglobin A1c is associated with a risk of major adverse cardiac events (MACE) after successful drug-eluting stent (DES) implantation in patients with diabetes mellitus. METHODS: In a retrospective study with a prospective follow-up, 206 patients with diabetes mellitus undergoing successful DES implantation were enrolled in this study. Hemoglobin A1c levels were measured within 1 month before coronary stenting. RESULTS: During a period of 4,811 person-months, we confirmed 40 cases of MACE. Higher hemoglobin A1c levels increased the incidence of MACE. Based on multivariate analysis, hemoglobin A1c was a significant predictor of MACE. The multiple-adjusted hazard ratio for a 1% increase in hemoglobin A1c levels was 1.40 (95% CI: 1.13-1.74, p = 0.002) for MACE after adjustment for age, gender, ejection fraction, chronic renal failure on hemodialysis, and statins. CONCLUSIONS: Hemoglobin A1c is associated with an increased risk of MACE after successful DES implantation in patients with diabetes mellitus.

Comparison of blood glucose values on admission for acute myocardial infarction in patients with versus without diabetes mellitus.

Previous studies have reported that acute hyperglycemia is associated with high mortality after acute myocardial infarction (AMI). However, optimal plasma glucose level may be different between diabetic and nondiabetic patients. The purpose of this study was to assess the relation between admission glucose and in-hospital mortality after AMI in patients with and without diabetes. This study consisted of 3,750 patients who were admitted to the 35 hospitals participating to the Japanese Acute Coronary Syndrome Study (JACSS) group within 48 hours after the onset of AMI. Plasma glucose was measured at the time of hospital admission. In patients without a history of diabetes, there was a linear relation between admission glucose and in-hospital mortality. Nondiabetic patients with a glucose level <6 mmol/L had the lowest mortality (2.5%). As admission glucose increased by 1 mmol/L, mortality increased by 17% (13% to 21%, p <0.001). In patients with a history of diabetes, however, there was a U-shape relation between glucose and mortality. Diabetic patients with glucose 9 to 10 mmol/L had the lowest mortality (1.9%); not only severe hyperglycemia (glucose > or =11 mmol/L, 9.1%, p <0.001) but also euglycemia (glucose <7 mmol/L, 9.4%, p = 0.009) were associated with higher mortality compared to moderate hyperglycemia (glucose 9 to 11 mmol/L, 3.2%). Diabetic patients with admission glucose 9 to 10 mmol/L had the lowest mortality, whereas lower glucose was better in nondiabetic patients. In conclusion, optimal glucose level on admission may be different between diabetic and nondiabetic patients with AMI.

Combined prognostic utility of white blood cell count, plasma glucose, and glomerular filtration rate in patients undergoing primary stent placement for acute myocardial infarction.

Although high white blood cell (WBC) count and plasma glucose (PG) and low glomerular filtration rate (GFR) on admission have been associated with poor outcomes after acute myocardial infarction (AMI), the combined prognostic utility of these 3 variables was unclear. The association of WBC count, PG, and GFR on admission to in-hospital outcomes was examined in 2,633 patients who underwent primary stent placement for ST-segment elevation AMI within 48 hours after symptom onset. In-hospital mortality progressively increased as the number of the variables of high WBC count (> or =11,120/microl; upper tertile), high PG (> or =10.4 mmol/L; upper tertile), and low GFR (< or =60 ml/min/1.73 m(2); lower tertile) increased. Patients with all 3 variables had a strikingly higher in-hospital mortality rate (25.9%). After adjusting for baseline characteristics, multivariate analysis showed that compared with patients who had none of these variables, odds ratios for in-hospital mortality were 1.63 (95% confidence interval [CI] 0.88 to 3.03, p = 0.12) in patients with only 1 variable, 2.33 (95% CI 1.28 to 3.96, p = 0.047) in those with 2 variables, and 6.16 (95% CI 2.98 to 12.6, p <0.001) in those with all 3 variables. In conclusion, combined evaluation of WBC count, PG, and GFR on admission was a simple and useful method for the early prediction of risk of in-hospital death in patients undergoing primary stent placement for ST-segment elevation AMI.

Impact of body mass index on in-hospital outcomes after percutaneous coronary intervention for ST segment elevation acute myocardial infarction.

BACKGROUND: The impact of body mass index (BMI) on outcomes after primary percutaneous coronary intervention (PCI) for acute myocardial infarction (AMI) remains unclear. METHODS AND RESULTS: A total of 3,076 patients undergoing PCI for AMI within 48 h after symptom onset were studied. Patients were divided into 4 groups according to baseline BMI: lean (<20 kg/m(2)), normal weight (20.0-24.9 kg/m(2)), overweight (25.0-29.9 kg/m(2)) and obese (>or=30.0 kg/m(2)). Obese patients were younger and had a higher frequency of diabetes mellitus, hyperlipidemia, hypertension and smoking. Lean patients were older, usually women and had a lower frequency of the aforementioned risk factors. Killip class on admission, renal insufficiency, and final Thrombolysis In Myocardial Infarction (TIMI) flow grade did not differ among the 4 groups. In lean, normal weight, overweight and obese patients, in-hospital mortality was 9.2%, 4.4%, 2.5% and 1.8%, respectively (p<0.01). Multivariate analysis showed that compared with normal weight patients, odds ratios for in-hospital death in lean, overweight and obese patients were 1.92, 0.79 and 0.40, respectively (p=NS). Independent predictors were age, Killip class on admission, renal insufficiency and final TIMI flow grade. CONCLUSION: BMI itself had no impact on in-hospital mortality in patients undergoing primary PCI for AMI. The phenomenon ;obesity paradox' may be explained by the fact that obese patients were younger at presentation.

A Japanese family of typical Loeys-Dietz syndrome with a TGFBR2 mutation.

This report describes a Japanese family with vessel and craniofacial abnormalities. Although the clinical findings of the patient's father fulfilled the diagnostic criteria for Marfan syndrome, arterial tortuosity, aneurysms, hypertelorism and a bifid uvula were noted in both the patient and his father. These findings were compatible with the clinical manifestations that were previously reported in Loeys-Dietz syndrome. A molecular genetic analysis demonstrated a heterozygous missense mutation of the transforming growth factor-beta receptor II gene in both the patient and his father, which thus caused Loeys-Dietz syndrome. This is the first Japanese family case report of typical Loeys-Dietz syndrome.

Weekend onset of acute myocardial infarction does not have a negative impact on outcome in Japan.

BACKGROUND: Studies from North America indicate that patients admitted during the weekend with acute myocardial infarction (AMI) have a worse outcome than weekday-admitted patients, probably reflecting a lower rate of invasive procedures. However, it is unclear whether the same is true in Japan, which has a different healthcare system. METHODS AND RESULTS: Using the Japanese Acute Coronary Syndrome Study (JACSS) database, this study included 4,805 consecutive patients who were admitted within 48 h of onset of AMI (3,526 [73.4%] patients with weekday onset [Monday through Friday] and 1,279 [26.6%] with weekend onset [Saturday and Sunday]). There were no significant differences between the 2 groups in patient background and clinical features. The proportions of patients who underwent emergency catheterization (88.4% vs 88.0%) and reperfusion therapy (81.5% vs 81.4%) were also similar. There were no differences between the 2 groups in the in-hospital, 30-day, and 1-year mortality rates. Even after various adjustments, there was no difference in the risk of death associated with weekend versus weekday onset of AMI. CONCLUSION: There were no obvious differences in outcome for Japanese AMI patients in the weekday- or weekend-onset group, suggesting the quality of the Japanese healthcare system is similar for the entire week.

Long-term nitrate therapy after acute myocardial infarction does not improve or aggravate prognosis.

BACKGROUND: There is conflicting information about whether nitrate treatment aggravates long-term prognosis, so the present retrospective study was designed to determine the effects of long-term nitrate therapy on major adverse events after acute myocardial infarction (AMI) in the coronary interventional era. METHODS AND RESULTS: Using the Japanese Acute Coronary Syndrome Study database, 1,236 consecutive patients who were hospitalized within 48 h of onset of symptoms of AMI from January to December 2003 were evaluated. All-cause mortality, cardiac events and cardiovascular events were lower in patients treated with nitrates than in the untreated controls. However, these crude comparisons included several confounding factors on nitrate prescription. To minimize the effect of selection bias on outcomes, the technique of propensity score matching for clinical characteristics was used and distortion of effective nitrate treatment was excluded as much as possible. The results of propensity score matching showed that nitrate therapy had no impact on all-cause mortality, cardiac events and cardiovascular events at 30, 60 or 90 days, 6 months, 1 year, and 2 years follow-up. CONCLUSIONS: Long-term nitrate therapy after AMI neither improves nor aggravates prognosis. Prospective randomized clinical trials are warranted to determine the effects of long-term nitrate therapy for secondary prevention of AMI.

Usefulness of combined white blood cell count and plasma glucose for predicting in-hospital outcomes after acute myocardial infarction.

Admission white blood cell (WBC) count and plasma glucose (PG) have been associated with adverse outcomes after acute myocardial infarction (AMI). This study investigated the joint effect of WBC count and PG on predicting in-hospital outcomes in patients with AMI. WBC count and PG were measured at the time of hospital admission in 3,665 patients with AMI. Patients were stratified into tertiles (low, medium, and high) based on WBC count and PG. Patients with a high WBC count had a 2.0-fold increase in in-hospital mortality compared with those with a low WBC count. Patients with a high PG level had a 2.7-fold increase in mortality compared with those with a low PG level. When a combination of different strata for each variable was analyzed, a stepwise increase in mortality was seen. There was a considerable number of patients with a high WBC count and low PG level or with a low WBC count and high PG level. These patients had an intermediate risk, whereas those with a high WBC count and high PG level had the highest risk, i.e., 4.8-fold increase in mortality, compared with those with a low WBC count and low PG level. Multivariate analysis was performed to assess the predictor for in-hospital mortality using WBC count and PG level as continuous variables and showed that WBC count and PG level were independently associated with in-hospital mortality. These findings suggested that a simple combination of WBC count and PG level might provide further information for predicting outcomes in patients with AMI.

Sex differences in early mortality of patients undergoing primary stenting for acute myocardial infarction.

BACKGROUND: Limited information exists regarding the impact of gender on in-hospital outcome after primary stenting for acute myocardial infarction (AMI). METHODS AND RESULTS: A total of 2,981 patients (790 women and 2,191 men) participated in the study who were admitted within 24 h after symptom onset and underwent emergency primary stenting for AMI. Compared with men, women were significantly older; had higher incidences of hypertension, diabetes mellitus, hyperlipidemia, Killip class > or =2, and cardiogenic shock; had a higher blood glucose level and a lower serum creatinine level on admission. Other baseline characteristics, including the incidences of ST-segment elevation AMI, anterior infarction, 3-vessel disease, initial or final Thrombolysis in Myocardial Infarction (TIMI) flow grade did not significantly differ between the sexes. The in-hospital mortality rate was significantly higher in women than in men (9.4% vs 5.2%, p<0.001). On multivariate analysis, age, Killip class, blood glucose level, serum creatinine level, and final TIMI grade were independent predictors of in-hospital death, but female gender was not (odds ratio 1.01, p=0.69). CONCLUSIONS: Our findings suggest that in patients undergoing primary stenting for AMI, women have higher in-hospital mortality than men, but female gender itself is not independently associated with increased in-hospital mortality after adjustment for baseline differences.

[Paraganglioma of the urinary bladder: a case report].

A patient with paraganglioma of the urinary bladder is reported. A 65-year-old woman was referred to our hospital with the chief complaint of postvoiding headache, palpitation, and cold sweat. She had has no episodes of hematuria. On laboratory data, mild elevation was found in plasma neuron specific enolase (NSE), urinary noradrenaline and urinary metanephrine. The patient showed hypertension after urination with the elevation of plasma noradrenaline. 131I-MIBG scintigraphy showed abnormal accumulation in the bladder, and no abnormal accumulation in the other lesion. Pelvic magnetic resonance imaging (MRI) revealed a solid tumor of the urinary bladder, measuring 2 cm in diameter. Paraganglioma of the urinary bladder was diagnosed and the tumor was extirpated. The tumor measured 2 x 2 x 1.5 cm, and histopathologically diagnosed as paraganglioma. After extirpation, the patient became free of the postvoiding symptoms, and showed normal catecholamine levels. She has been followed up for 4 months after operation without any evidence of recurrence.

Cell surface-expressed moesin-like HDL/apoA-I binding protein promotes cholesterol efflux from human macrophages.

HDL and its major component, apolipoprotein A-I (apoA-I), play a central role in reverse cholesterol transport. We recently reported the involvement of a glycosylphosphatidylinositol anchor (GPI anchor) in the binding of HDL and apoA-I on human macrophages, and purified an 80 kDa HDL/apoA-I binding protein. In the present study, we characterized the GPI-anchored HDL/apoA-I binding protein from macrophages. The HDL/apoA-I binding protein was purified from macrophages and digested with endopeptidase, and the resultant fragments were sequenced. Cholesterol efflux, flow cytometry, immunoblotting, and immunohistochemical analyses were performed to characterize the HDL/apoA-I binding protein. Two parts of seven amino acid sequences completely matched those of moesin. Flow cytometry, immunoblotting, and immunohistochemistry using anti-moesin antibody showed that the HDL/apoA-I binding protein was N-glycosylated and expressed on the cell surface. It was termed moesin-like protein. Treatment of macrophages with anti-moesin antibody blocked the binding of HDL/apoA-I and suppressed cholesterol efflux. The moesin-like protein was exclusively expressed on macrophages and was upregulated by cholesterol loading and cell differentiation. Our results indicate that the moesin-like HDL/apoA-I binding protein is specifically expressed on the surface of human macrophages and promotes cholesterol efflux from macrophages.-Matsuyama, A, N. Sakai, H. Hiraoka, K-i. Hirano, and S. Yamashita. Cell surface-expressed moesin-like HDL/apoA-I binding protein promotes cholesterol efflux from human macrophages.

Acute hyperglycemia is associated with adverse outcome after acute myocardial infarction in the coronary intervention era.

PURPOSE: This study was undertaken to assess the association between acute hyperglycemia and inhospital outcome after acute myocardial infarction (AMI) in the percutaneous coronary intervention (PCI) era. We also assessed outcome of patients with a history of diabetes mellitus in the PCI era. METHODS: Between January 2001 and December 2001, 1253 patients were admitted to the hospitals within 48 hours after the onset of AMI. Plasma glucose was measured at hospital admission. Acute hyperglycemia was defined as plasma glucose of > 11 mmol/L (198 mg/dL), regardless of the diabetic status. Primary PCI was performed in 898 (72%) patients. RESULTS: The inhospital mortality rate was significantly higher in patients with acute hyperglycemia than in patients without (16% vs 6%, P < .001). However, there was no significant difference in mortality between diabetic and nondiabetic patients (8% vs 9%, P = .54). Acute hyperglycemia was associated with a higher inhospital mortality rate both in nondiabetic patients (24% vs 6%, P < .001) and in diabetic patients (10% vs 5%, P = .039). Acute hyperglycemia was associated with a higher incidence of no reflow during PCI (21% vs 12%, P < .001), but diabetes was not (14% vs 15%, P = .71). CONCLUSION: Acute hyperglycemia, but not diabetes, was a predictor for inhospital mortality after AMI in the PCI era. No reflow occurred more frequently during PCI in patients with acute hyperglycemia, suggesting that microvascular dysfunction might have contributed to adverse outcome of these patients.