Miction-induced Hypertension Disclosed by Home Blood Pressure Measurement in a Patient with Small Paraganglioma.

A 46-year-old woman complained of a 10-year history of headache, nausea, a precordial oppressive feeling and shortness of breath on miction. She had noted a marked elevation in her blood pressure after miction using home blood pressure measurement. Her catecholamine levels were less than twice the value of the normal upper limit. Several imaging modalities detected a urinary bladder tumor, and 123I-metaiodobenzylguanidine scintigraphy showed positive accumulation. The diagnosis of urinary bladder paraganglioma was confirmed by partial cystectomy. We must keep in mind that paroxysms and hypertension associated with miction are important diagnostic clues of pheochromocytoma/paraganglioma. Home blood pressure measurement was very useful for detecting hypertension in this case.

A case of warfarin-induced eosinophilia.

Warfarin is widely used in clinical practice all over the world. We report a man in whom prominent eosinophilia appeared after the initiation of warfarin administration following aortic valve replacement. Laboratory data following the administration and discontinuation of warfarin suggested that this drug was responsible for the eosinophilia. It is important to recognize the possibility of warfarin-induced hypereosinophilia as a latent adverse effect even when there are no clinical signs or symptoms.

Admission with metabolic disorder is a useful predictor of the 1-year prognosis for patients with unstable angina, but not for patients with acute myocardial infarction: east-Osaka acute coronary syndrome registry.

BACKGROUND: For patients with acute myocardial infarction (AMI), several studies have examined the relationship between the metabolic syndrome and prognostic outcome. However, few studies have revealed an association between the metabolic syndrome and clinical outcomes in patients with unstable angina (UA). This study compared the differences in the usefulness of recognizing metabolic disorders for the prediction of a 1-year prognosis in patients with UA and AMI. METHODS: The study cohort consisted of 1173 patients with a mean age of 67 years. The focus was on general prognostic factors and five metabolic disorders (body mass index; hypertension; blood glucose/diabetes mellitus; and, serum concentrations of triglycerides and high-density lipoprotein cholesterol) at the time of admission. RESULTS: According to multivariate logistic regression analysis, metabolic scores on admission positively related to 1-year mortality or major adverse cardiovascular events (MACE) for patients with UA, but not for those with AMI, with an increase in either all-causes mortality or MACE being associated with the degree of metabolic dysfunction. No other general prognostic factors were related to either 1-year mortality or MACE in patients with UA. By contrast, general prognostic factors such as age and the Killip classification had a positive effect on 1-year mortality or on MACE for the patients with AMI. CONCLUSION: Accumulation of the effects of each metabolic disorder may affect mortality and MACE for patients with UA.

Measuring visceral fat with water-selective suppression methods (SPIR, SPAIR) in patients with metabolic syndrome.

We attempted to measure the area and volume of visceral fat using magnetic resonance (MR) imaging to avoid radiation exposure. We used water suppression-spectral attenuation with inversion recovery (WS-SPAIR) as prepulses and conducted T(1) high-resolution isotropic volume examination (THRIVE). Image processing software can be used to estimate the area and volume of fat and separate the fat and water signals at a visually optimal threshold in the MR image, which requires contrast enhancement between intestinal contents and visceral fat. In 14 volunteers, we evaluated WS-SPAIR and water suppression-spectral presaturation with inversion recovery (WS-SPIR) with respect to the relationship between the flip angle of THRIVE and signal contrast. We used flip angles of 5 degrees, 10 degrees, and 20 degrees. The minimum threshold that allowed exclusion of intestinal contents from the masked region was determined for each technique. The volume and area of the masked region, which included subcutaneous fat, were measured at the umbilicus level. Both volume and area increased with a smaller flip angle. The masked region was larger with WS-SPIR-THRIVE (flip angle 5 degrees ). The size of the masked region was determined according to the minimum threshold that allowed exclusion of the intestinal contents from the masked region, expressing the contrast between the intestinal contents and fat in a relative manner. It was speculated that by separating the signals at the threshold, WS-SPIR-THRIVE (flip angle 5 degrees) was a more suitable technique for measuring the area and volume of visceral fat.

Core protein of hepatitis C virus induces cardiomyopathy.

Hepatitis C virus (HCV) has been reported to be associated with cardiomyopathy. However, the mechanism of cardiomyopathy in chronic HCV infection is still unclear. Therefore, we investigate the development of cardiomyopathy in mice transgenic for the HCV-core gene. After the age of 12 months, mice developed cardiomyopathy that appeared as left ventricular dilatation, and systolic and diastolic dysfunction assessed by Doppler echocardiography. Histologically, hypertrophy of cardiomyocytes, cardiac fibrosis, disarray and scarcity of myofibrils, vacuolization and deformity of nuclei, myofibrillar lysis, streaming of Z-bands, and an increased number of bizarre-shaped mitochondria were found in HCV-core transgenic mice. These histological changes are just consistent with cardiomyopathy. In conclusion, the HCV-core protein directly plays an important role in the development of cardiomyopathy.

Successful endovascular repair of an aneurysm of the ductus diverticulum with a branched stent graft: case report and review of literature.

Aneurysm of the ductus diverticulum rarely has been reported in adults, and the reported aneurysms were treated with conventional open surgery or were diagnosed at autopsy. We report a successful endovascular repair of an aneurysm of the ductus diverticulum with the Inoue branched stent graft. In a 78-year-old woman, an abnormal shadow was noted at the aortopulmonary window on a chest x-ray film. A computed tomography scan demonstrated a 3.8-cm saccular aneurysm, which protruded inferiorly from the distal end of the aortic arch. The aneurysm was considered an aneurysm of the ductus diverticulum, and surgery was required. However, the patient was considered at high risk for respiratory dysfunction with conventional open surgery. Endovascular repair with an Inoue single-branched stent graft was performed with the patient under local anesthesia, successfully and without complication or endoleak. To our knowledge, this is the first report of endovascular treatment of an aneurysm of the ductus diverticulum.

Quadricuspid aortic valve: report of three cases.

Quadricuspid aortic valve (QAV) is a very rare congenital malformation. We have encountered three patients with QAV, of whom one patient may be the eldest reported patient with this particular anatomical abnormality. In another of our patients, there was aortic regurgitation, aortic stenosis, and healed infective endocarditis, with adhesion of the tips of the cusps. In all three patients, the cusps were all of equal size. Until now, there has been very little documented evidence about the anatomical variations in QAV or its relationship with infective endocarditis. From the available literature, we conclude that the anatomical variations in patients with QAV are similar to those in patients with quadricuspid pulmonary valve, and infective endocarditis may not be an uncommon complication.

Detecting viable myocardium and predicting functional improvement: comparisons of positron emission tomography, rest-redistribution thallium-201 single-photon emission computed tomography (SPECT), exercise thallium-201 reinjection SPECT, I-123 BMIPP SPECT and dobutamine stress echocardiography.

BACKGROUND: Low-dose dobutamine stress echocardiography (LDDE) has become a useful and safe method for identifying hibernating or stunned myocardium and for predicting improvement in wall motion after coronary revascularization. METHODS AND RESULTS: In the present study, fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET), rest-redistribution thallium-201 ((201)Tl) single-photon emission computed tomography (RR-Tl SPECT), (123)I-15-(p-iodophenyl)-3-(R,S)-methyl pentadecanoic acid (BMIPP) and LDDE were performed in 30 patients with acute myocardial infarction (AMI) at 10+/-3 days after the onset of AMI. Also, exercise (201)Tl reinjection SPECT (RI-Tl SPECT) was performed at 14+/-2 days. Follow-up echocardiography was performed 5+/-3 months later in all patients after interventional therapy for the assessment of functional recovery. Of the 390 segments analyzed by echocardiography, 110 (28%) had abnormal wall motion. There were no significant differences between RR-Tl SPECT and LDDE in sensitivity, specificity, positive predictive value and negative predictive value using the chi(2)-test; however, in akinetic segments, there was a significant difference in sensitivity. Among FDG-PET, RI-Tl SPECT, BMIPP and LDDE, there were significant differences in 3 variables. In akinetic segments, LDDE is superior to RR-Tl SPECT in sensitivity and to FDG-PET in specificity. In hypokinetic segments, LDDE is superior to RI-Tl SPECT and BMIPP in sensitivity, and to FDG-PET and BMIPP in specificity. CONCLUSIONS: LDDE could detect functional recovery of viable myocardium in the early period of AMI and can be performed easily and safely.

Fabry disease female proband with clinical manifestations similar to hypertrophic cardiomyopathy.

Fabry's disease is an X-linked inborn error of glycosphingolipid catabolism, resulting from a deficiency in alpha-galactosidase A (alpha-Gal A). A 56-year-old Japanese woman was at first suspected of having hypertrophic cardiomyopathy. The patient and her son had alpha-Gal A activity in leukocytes that was remarkably below the limit of controls. DNA analysis of the alpha-Gal A gene revealed a novel missense mutation at codon 19 in exon 1, resulting in leucine-to-proline substitution. As a result she was confirmed as a classic Fabry heterozygote. Recent advances in enzyme replacement therapy can reverse the storage of glycosphingolipids in Fabry's disease. Thus, in patients with cardiac hypertrophy, it is important to differentiate Fabry's disease from other causes of hypertrophy. Therefore, it is necessary to measure alpha-Gal A activity in all suspected cases and to analyze genetic abnormalities in heterozygotes.

Prevalence of hepatitis C virus infection among patients with hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is defined as inappropriate ventricular hypertrophy without a cardiac or systemic cause. On the other hand, hepatitis C virus (HCV) causes extrahepatic manifestations as well as chronic persistent infection in hepatocytes. We studied the association of HCV infection with HCM, comparing the prevalence of HCV antibodies between HCM patients and age- and gender-matched controls with other cardiovascular diseases at a single institution, for reasons of exclusion of bias. We then described the clinical features and genotype analysis of HCV RNA in HCM. The diagnosis of HCM was established by echocardiographic demonstration of a hypertrophied (> or =15 mm), nondilated left ventricle in the absence of another systemic or cardiovascular disease capable of producing the magnitude of hypertrophy observed. The study population consisted of 80 patients with HCM, in whom HCV antibody was examined (55 men and 25 women; mean age 56.6 +/- 12.4 years; ranging from 19 to 80 years), compared with a total of 80 age- and gender-matched controls without HCM. The prevalence of HCV infection in patients with HCM (18/80) was significantly higher than in control subjects (5/80) (Chi(2) = 7.312, P = 0.007). Of the 12 patients in whom the genotype of HCV was analyzed, 7 had type 1b and 5 had type 2a. The prevalence of HCV infection was higher in patients with HCM than in age- and gender-matched control subjects with other cardiovascular diseases. The result suggests that HCV may play an important role in these HCV-positive HCM patients.

Coronary flow reserve correlates left ventricular diastolic dysfunction in patients with dilated cardiomyopathy.

The relationship between altered coronary circulation and left ventricular (LV) function in dilated cardiomyopathy (DCM) remains unclear. We used the Doppler guidewire and transthoracic echo Doppler in 24 DCM patients to investigate the relationship between coronary flow reserve (CFR) and LV systolic/diastolic function, trying to predict diastolic dysfunction and evaluate DCM severity with CFR. CFR correlated better with the deceleration time (DT) of the E-wave and the ratio of E-wave peak value to that of the A-wave (E/A) than with LV ejection fraction (EF). The optimal CFR cutoff value for predicting the restrictive pattern of transmitral flow velocity (DT = 120 msec) was 2.6 (sensitivity 91%, specificity 100%). Dividing patients into two groups around the CFR = 2.6 cutoff, differences in DT and E/A between groups were more prominent than those for EF. CFR correlates better with LV diastolic than systolic function and may be useful for predicting diastolic dysfunction in DCM patients.

Pitfalls of echocardiographic measurement in tissue harmonic imaging: in vitro and in vivo study.

BACKGROUND: Tissue harmonic imaging (THI) is a useful method for endocardial border detection by transthoracic echocardiography, especially in technically difficult patients, even though accuracy of this method in the echocardiographic measurement is unclear. The purpose of this study is to evaluate the accuracy of echocardiographic measurement by THI in vivo and in vitro. METHODS: In vitro, we measured wall thickness, dimension, and volume of the excised hearts by THI. In 11 patients, we assessed the comparative accuracy of THI and fundamental imaging (FI) in determination of left ventricular (LV) wall thickness, dimension, volume, and ejection fraction. RESULTS: In vitro, thickness measurements by THI overestimated true length, and both volume and dimension measurements by THI underestimated true values. In vivo, LV ejection fraction measurements obtained by THI exhibited excellent correlation and agreement with those obtained by FI. However, LV wall thickness determined by THI was significantly larger than that determined by FI, and the dimensions and volume of LV measured by THI were significantly smaller than those measured by FI. CONCLUSION: Although THI is an excellent imaging technique for determination of LV ejection fraction, echocardiographic measurement by THI underestimates LV dimensions and volume, and overestimates LV wall thickness.

Unruptured aneurysm of the sinus of valsalva with Behçet's disease.

A 52-year-old Japanese man who had suffered from Behçet's disease since the age of 45 years was admitted to hospital for evaluation of syncope and heart murmur. Echocardiography and aortography revealed severe aortic regurgitation and cystic masses under the right coronary cusp and the left ventricular outflow tract, but no shunt jet. He was diagnosed with unruptured aneurysm of the sinus of Valsalva, and surgical closure of the orifice of the aneurysm was performed. The diameter of the orifice was 11 mm and the aneurysm was 15 mm in depth, and consisted of 2 chambers. Because the aortic regurgitation was reduced after patch closure of the orifice, aortic valve replacement was not performed. Unruptured aneurysm of the sinus of Valsalva is a rare clinical lesion, but patients with active inflammatory disease of the aorta, such as in Behçet's disease, should have periodic echocardiography for early detection of an aneurysm or valvular involvement, even if there are not any symptoms.

Incremental value of assessment of regional wall motion for detection of multivessel coronary artery disease in exercise (201)Tl gated myocardial perfusion imaging.

UNLABELLED: Assessment of reversible perfusion defects in exercise (201)Tl perfusion SPECT has low sensitivity and high specificity for detection of multivessel coronary artery disease (CAD). The goal of this study was to evaluate whether worsening of left ventricular regional wall motion assessed by an automated algorithm in exercise (201)Tl electrocardiography-gated SPECT had incremental diagnostic value over perfusion data for detection of multivessel CAD. METHODS: Two hundred one patients underwent exercise (201)Tl gated SPECT. Software that automatically analyzes left ventricular function was used to assess exercise and rest regional wall motion. Regional wall motion on initial images was compared with that on rest images, that is, delayed images for patients without reinjection images and reinjection images for patients with reinjection images. The left ventricle was divided into 9 segments, with individual segments assigned to 3 coronary territories. Worsening of wall motion was defined as worsening in any segment on initial images compared with rest images. RESULTS: Of 73 patients with multivessel CAD, 20 (27.4%) had reversible perfusion defects in multiple coronary territories, 26 (35.6%) exhibited worsening of regional wall motion in multiple territories, and 37 (50.7%) had reversible perfusion defects or worsening of regional wall motion in multiple territories. The sensitivity of the combination of reversible perfusion defect and worsening of regional wall motion was significantly higher than that of reversible perfusion defect alone for detection of multivessel CAD (50.7% vs. 27.4%, P < 0.05). The specificity of the combination of reversible perfusion defect and worsening of regional wall motion for detecting multivessel CAD did not differ from that of reversible perfusion defect alone and that of worsening of regional wall motion alone (94.5% vs. 99.2% and 97.7%, respectively, P = not statistically significant). CONCLUSION: Combined assessment of worsening of left ventricular regional wall motion by exercise and perfusion data in exercise (201)Tl gated myocardial SPECT was more sensitive, with acceptable specificity, than was assessment with perfusion data alone for detection of multivessel CAD.

Change in coronary flow reserve on transthoracic Doppler echocardiography after a single high-fat meal in young healthy men.

BACKGROUND: High-fat meals and elevated triglyceride levels are associated with cardiovascular disease. In recent studies of brachial artery vasoactivity, a single high-fat meal reduced endothelial function in young healthy men. It is unknown whether coronary microcirculation is affected after high-fat meals. OBJECTIVE: To evaluate change in coronary flow reserve after a single high-fat meal. DESIGN: Controlled interventional study. SETTING: University hospitals. PATIENTS: 15 young healthy men (mean age [+/-SD], 29 +/- 4 years). INTERVENTION: Coronary flow reserve was determined by using transthoracic Doppler echocardiography before and after consumption of a high-fat meal in all 15 men and before and after consumption of a low-fat meal in 5 of 15 men. MEASUREMENTS: Coronary flow reserve, lipid levels, and hemodynamic characteristics. RESULTS: In all men, triglyceride levels increased significantly from baseline 5 hours after the high-fat meal, from 1.1 mmol/L to 2.8 mmol/L (100 mg/dL to 250 mg/dL) (P < 0.001). Average coronary flow reserve was 4.02 before and 3.30 5 hours after the high-fat meal (decrease, 18% [95% CI, 13% to 23%]). In the 5 men who received both meals, mean coronary flow reserve decreased by 0.79 after the high-fat meal and increased by 0.07 after the low-fat meal (difference, -0.86 [CI, -1.36 to -0.37]; P = 0.03). Mean triglyceride levels increased by 1.6 mmol/L (140 mg/dL) after the high-fat meal and 0.1 mmol/L (10 mg/dL) after the low-fat meal (difference, 1.5 mmol/L [CI, 0.3 to 2.7 mmol/L], 130 mg/dL [CI, 23 to 236 mg/dL]; P = 0.03). CONCLUSIONS: Coronary flow reserve decreased after a single high-fat meal in young healthy men. High-fat meals may be detrimental to coronary microcirculation.

Incremental value of left ventricular ejection fraction for detection of multivessel coronary artery disease in exercise (201)Tl gated myocardial perfusion imaging.

UNLABELLED: Assessment of reversible defects in exercise (201)Tl perfusion SPECT has low sensitivity and high specificity for detection of multivessel coronary artery disease (CAD). The goal of this study was to evaluate whether the left ventricular ejection fraction (LVEF) in exercise (201)Tl gated SPECT had incremental diagnostic value over perfusion data for detection of multivessel CAD. METHODS: One hundred eighty-two patients underwent exercise (201)Tl gated SPECT. Automated LV function analysis software was used for calculation of the postexercise and the rest LVEF. The best threshold between 0- to 1-vessel CAD and 2- to 3-vessel CAD was determined as the cutoff that on receiver-operating-characteristic analysis resulted in the best sensitivity for detection of multivessel CAD with an associated specificity of >90%. RESULTS: Only 18 (26.9%) of 67 patients with multivessel CAD had reversible defects in multiple territories. Sensitivities of the postexercise and the rest LVEF and the worsening of the LVEF by exercise did not differ from those of perfusion data alone. Sensitivities of the combination of perfusion data and the postexercise and rest LVEF did not differ from those of perfusion data alone, whereas the sensitivity of the combination of perfusion data and worsening of the LVEF (i.e., reversible defects in multiple territories or worsening of the LVEF >5.6% [or both]) was significantly greater than that of perfusion data alone (43.3% vs. 26.9%; P < 0.05), with an acceptable level of specificity (90.4%). CONCLUSION: The worsening of the LVEF by exercise has the potential to detect patients with multivessel CAD among those without multivessel patterns of reversible defects.