Systemic immunoglobulin light-chain amyloidosis presenting hematochezia as the initial symptom.

Immunoglobulin light-chain (AL) amyloidosis is characterized by the deposition of insoluble fibrils composed of immunoglobulin light chains secreted by monoclonal plasma cells. Given the recent advances in the therapy of AL amyloidosis, it is important to diagnose this disease as early as possible. Herein, we describe the case of a 62-year-old man with hepatitis C virus (HCV)-related cirrhosis presenting with hematochezia. Colonoscopy showed multiple submucosal hematomas within the region ranging from the transverse colon to the sigmoid colon. Kappa immunoglobulin light-chain amyloid deposition was also detected. Bone marrow examination revealed a monoclonal abnormal plasma cell population. Thus, the patient was diagnosed with systemic immunoglobulin light-chain amyloidosis. The hematochezia was conservatively managed. However, because of liver failure caused by liver cirrhosis, the patient developed massive pleural effusion and died of respiratory failure. Postmortem examination revealed amyloid deposition in the esophagus, stomach, duodenum, ileum, descending colon, pancreas, heart, and lung. In these organs, amyloid deposition was limited to the vascular wall. We concluded that AL amyloidosis can present hematochezia arising from submucosal hematoma in the large colon before other systemic symptoms appear.

Critical in-stent restenosis following fracture of biolimus-eluting stent: a report of 2 cases.

The development of drug-eluting stents (DES) has dramatically reduced the incidence of in-stent restenosis. Stent fracture (SF) of DESs, however, has recently emerged as a rare but serious complication, which may lead to acute coronary syndrome or sudden cardiac death. DES fracture results from metal fatigue and vessel hemodynamic stress on the stent strut, due to markedly reduced neointimal formation. Although actual incidence of SF is not known, previous reports have demonstrated that SF rates are specific to each DES type. In this report, 2 cases of fracture of a Nobori stent are described, with insights into the mechanisms of SF and strategies for its successful management.

Hemodialysis-induced P-wave signal-averaged electrocardiogram alterations are indicative of vulnerability to atrial arrhythmias.

BACKGROUND: Atrial fibrillation (AF) is the most common supraventricular arrhythmia, often occurring during hemodialysis (HD). Prolongation of the total filtered P-wave duration (PWD) and reduction of the root mean square voltages for the last 20 ms of the P wave (RMS20) on a P-wave signal-averaged electrocardiogram (P-SAECG) are predictors of AF. We investigated whether HD induces alterations of P-SAECG, and determined the influential factors. METHODS AND RESULTS: Thirty-three end-stage kidney disease patients (66.7 ± 12.6 years, 23 males) undergoing maintenance HD were enrolled in this study. Digital ambulatory P-SAECG monitoring and laboratory examination of serum proteins and ions were carried out before, during, and after the HD sessions. Data were analyzed by multiple regression analysis. PWD was significantly prolonged, and RMS20 significantly reduced, during HD. These values recovered after completion of HD. Multiple regression analysis showed that prolongation of PWD significantly correlated with HD duration and the rate of removal of body fluid. On the other hand, RMS20 significantly correlated with HD duration and blood urea nitrogen variation. CONCLUSIONS: HD resulted in prolongation of PWD and reduction of RMS20, indicating the vulnerability of HD patients to AF. These P-SAECG changes correlated with HD duration and the rate of removal of the body fluid. These findings underline the importance of the control of dialysis variables in the prevention of atrial arrhythmias following HD.

Torsion analysis in the early detection of anthracycline-mediated cardiomyopathy.

AIMS: Anthracyclines have profound consequences on the structure and function of the heart, which over time cause a cardiomyopathy that leads to congestive heart failure. Early detection of subclinical left ventricular (LV) dysfunction following a low dose of anthracyclines may be a preventive strategy. The aim of this study was to determine torsion analysis using two-dimensional speckle-tracking imaging (STI), useful for detecting early anthracycline-mediated cardiotoxicity. METHODS AND RESULTS: Conventional and Doppler echocardiography images were obtained from 25 patients (mean age 58 ± 11 years) before chemotherapy and 1 and 3 months after treatment. The cumulative anthracycline doses were 98 ± 59 and 170 ± 87 g/m(2) at 1 and 3 months, respectively. After standard echocardiography, LV torsion and twisting velocity profiles from apical and basal short-axis images were analysed using STI. LV dimensions and ejection fraction did not change throughout follow-up. Although isovolumic relaxation time showed prolongation 3 months after chemotherapy, other Doppler indices did not show significant changes. However, significant deteriorations in torsion (P < 0.0001 by ANOVA), twisting rate (P < 0.0001 by ANOVA), and untwisting rate (P < 0.001 by ANOVA) were found 1 month after chemotherapy. A significant negative correlation was observed between cumulative anthracycline doses and torsion (r = -0.524, P < 0.0001). CONCLUSION: LV torsion analysis could be a useful non-invasive approach for early detection of subclinical anthracycline cardiotoxicity.

Catheter ablation of non-inducible atrial tachycardia after surgical repair of heart disease.

We present a patient with non-inducible atrial tachycardia (AT) after atriotomy for surgical repair of heart disease who underwent ablation successfully. Using a 3-D mapping system, we presumed the atriotomy site on the lateral right atrial wall by searching for linear double potentials (DP) during sinus/paced rhythm from the coronary sinus, but it was evaluated incompletely. We could verify the edges of the atriotomy scar precisely by pacing from close to the linear DP lesion and the opposite site. After ablation between the presumed atriotomy scar and the inferior vena cava and cavotricuspid isthmus, no AT recurred without anti-arrhythmic drugs.

Low-dose rosuvastatin improves arterial stiffness in high-risk Japanese patients with dyslipdemia in a primary prevention group.

BACKGROUND: The treatment effects of rosuvastatin on arterial stiffness were assessed and compared to those of fluvastatin in high-risk Japanese patients with dyslipidemia in a primary prevention group. METHODS AND RESULTS: Patients were randomly assigned to either 2.5-5 mg/day of rosuvastatin (Group A) or 20-40 mg/day of fluvastatin (Group B) and followed up for 12 months. In Group A (n=38), there was a progressive reduction in brachial-ankle pulse wave velocity (baPWV) along with a decrease in the low-density lipoprotein cholesterol/high-density lipoprotein cholesterol (L/H) ratio and high-sensitivity C-reactive protein (hsCRP), and the change in baPWV correlated significantly with that of the L/H ratio and that of hsCRP after rosuvastatin treatment. In Group B (n=37), although fluvastatin achieved a significant improvement in baPWV, L/H ratio, and hsCRP, baPWV was significantly greater than that in Group A and showed a significant correlation with that of hsCRP alone after fluvastatin treatment. In a subgroup of patients (n=26), switching from fluvastatin to rosuvastatin further improved baPWV and the L/H ratio without altering hsCRP after 12 months. CONCLUSIONS: Low-dose rosuvastatin would be more effective than fluvastatin in improving arterial stiffness in high-risk Japanese patients with dyslipidemia. The results suggest that improvement in arterial stiffness by rosuvastatin mainly depends on its strong lipid-lowering effects, whereas that by fluvastatin is strongly dependent on the pleiotropic effects, especially an anti-inflammatory action.

Early intervention with rosuvastatin decreases the lipid components of the plaque in acute coronary syndrome: analysis using integrated backscatter IVUS (ELAN study).

BACKGROUND: It has recently become possible to analyze coronary plaque characteristics by using integrated backscatter intravascular ultrasound (IB-IVUS). The aim of this study was to use this modality to evaluate the impact of early intervention with rosuvastatin on both the volume and tissue characteristics of non-culprit plaques in acute coronary syndrome (ACS). METHODS AND RESULTS: Patients with ACS underwent IB-IVUS after percutaneous coronary intervention procedure and were administered rosuvastatin. Follow-up IB-IVUS was recorded 6 months later. We analyzed the changes in plaque burden and tissue characteristics in these patients. Plaque components were classified as calcified, fibrous, and lipid according IB-IVUS. We comprehensively analyzed 20 ACS patients. The low-density lipoprotein-cholesterol levels decreased significantly from 117 ± 34 mg/dl to 73 ± 19 mg/dl (P<0.001) after statin therapy. Comparing the baseline images with the follow-up ones revealed a significant reduction in the plaque burden from 98.4 ± 42.1mm(3)/10mm to 80.2 ± 35.8 mm(3)/10mm (P<0.001) and in the lipid volume from 44.1 ± 29.6 mm(3)/10mm to 28.6 ± 17.8 mm(3)/10mm (P<0.001). With respect to the % lipid volume, the reduction rate at follow-up showed a significant correlation with its baseline value (r=-0.498, P=0.024). CONCLUSIONS: Early intervention with rosuvastatin in ACS patients enabled significant reduction of the non-culprit plaque during 6 months. This regression was mainly due to the decrease in the lipid component of the plaque.

Influence of circumferential pulmonary vein isolation for atrial fibrillation on ST elevation in patient with Brugada syndrome.

We present a patient with Brugada syndrome and paroxysmal atrial fibrillation who underwent circumferential pulmonary vein isolation. His electrocardiogram showed normal sinus rhythm and first-degree AV block (P-R 280 ms) with coved-type ST elevation in V1-2 (+2.0 mm) before ablation. During ablation around the left pulmonary vein ostium, atrial fibrillation, progression of ST elevation (+4.5 mm), and T wave alternans occurred. After right pulmonary vein encirclement was complete, ST elevation improved to +1.0 mm. The following day, the ST segments remained lower than baseline levels.

Transient pseudorestrictive pattern of transmitral flow velocity curve in patients with paroxysmal atrial fibrillation.

BACKGROUND: Early diastolic velocity of the mitral annulus and transmitral flow propagation velocity are reported as more reliable determinants of left ventricular diastolic function in patients with atrial fibrillation than are transmitral Doppler indices. This study aimed to test the hypothesis that transmitral flow curve shows pseudorestrictive pattern during rate-controlled atrial fibrillation. METHODS: Thirteen paroxysmal atrial fibrillation patients were monitored for three phases: before atrial fibrillation, during atrial fibrillation, and after the recovery of atrial fibrillation to sinus rhythm. Standard two-dimensional, color flow, and tissue Doppler echocardiography were performed. We compared the indices of left ventricular diastolic function among the three phases. RESULTS: The early diastolic velocity of transmitral flow increased significantly during atrial fibrillation (before, 0.76 ± 0.19 m/sec; during, 0.86 ± 0.20 m/sec; after recovery to sinus rhythm, 0.73 ± 0.16 m/sec; P < 0.01). The deceleration time of early transmitral diastolic wave decreased during atrial fibrillation (182.5 ± 39.6 ms; 149.1 ± 38.7 ms; 184.0 ± 44.5 ms, respectively, P < 0.01). The early diastolic velocity of the mitral annulus increased during atrial fibrillation (5.37 ± 1.31 cm/sec; 7.29 ± 1.25 cm/sec; 5.37 ± 1.32 cm/sec; respectively, P < 0.01). The transmitral propagation velocity did not change significantly during atrial fibrillation. CONCLUSION: Although conventional Doppler indices showed abnormal relaxation pattern, left ventricular diastolic function was preserved during rate-controlled atrial fibrillation, as determined from early diastolic velocity of the mitral annulus and transmitral flow propagation velocity.

Effect of graft adaptation of the internal mammary artery on longitudinal phasic blood flow velocity characteristics after surgery.

Left internal mammary artery (LIMA) bypass conduits undergo gradual longitudinal flow transition from the proximal to distal segments, and the diastolic/systolic (D/S) ratios of the flow indices can diagnose graft patency. However, the influence of graft adaptation on this has not been studied. We examined 46 patients with LIMA graft to the left anterior descending artery using a Doppler-tipped guidewire in the proximal, middle, and distal segments; 34 had patent LIMAs (group A: new LIMAs; <1 month postoperatively; n = 22 and group B: old LIMAs; ≥1 month postoperatively; n = 12), and 12 had new LIMAs with distal stenosis (group C). In diastole, the time-averaged peak velocities, maximum peak velocities, and velocity-time integrals in each segment were significantly greater in group A than in groups B or C; however, in systole, they did not differ significantly among the three groups. The D/S ratios of the indices in all segments in group A were significantly greater than those in groups B or C; however, they did not differ between groups B and C in any of the segments. Graft adaptation of a patent LIMA, itself, affects the longitudinal flow transition pattern. The D/S ratio of the three indices in the patent old LIMAs did not differ from those in the LIMAs with distal stenosis early after surgery. The timing of LIMA flow assessment must be considered during assessment of the graft patency from the flow velocity patterns.

Dynamic left ventricular outflow tract obstruction due to anemia in a 71-year-old patient with sigmoid septum.

Sigmoid septum, although frequently observed in the elderly, has no pathophysiological significance in general. A 71-year-old woman with sigmoid septum developed acute heart failure because of a significant left ventricular outflow tract obstruction associated with the aggravation of anemia. This report provides the evidence that a hypercontractile cardiac state induced by anemia can be critical, especially in patients with sigmoid septum. Assessment of the left ventricular outflow tract pressure gradient is important in heart failure patients with sigmoid septum and dynamic obstruction.

A case of left atrial involvement of cardiac sarcoidosis manifesting as atrial flutter treated with corticosteroids.

The clinical diagnosis of cardiac sarcoidosis can be elusive, because the clinical manifestations are non-specific, and the sensitivity and specificity of diagnostic modalities are limited. A 60-year-old woman suffered atrial flutter and diffuse thickening of the left atrial wall accompanied with gallium-67 uptake, both of which were successfully treated with corticosteroid therapy. The diagnosis of cutaneous sarcoidosis had been made two years previously; therefore, we diagnosed the patient as having atrial involvement of cardiac sarcoidosis, and discuss here the importance of noninvasive evaluation of cardiac sarcoidosis using echocardiography.

Rationale and design of assessment of lipophilic vs. hydrophilic statin therapy in acute myocardial infarction (the ALPS-AMI) study.

BACKGROUND: Statins reduce the incidence of cardiovascular events in patients with acute myocardial infarction (AMI). Although all statins are equally effective in secondary prevention, there might be certain differences in the effects of lipophilic and hydrophilic statins. Therefore, our aim is to compare the effectiveness of lipophilic atorvastatin and hydrophilic pravastatin in secondary prevention after AMI. METHODS AND RESULTS: This study is a prospective, randomized, open-label, multicenter study of 500 patients with AMI. Patients that have undergone successful percutaneous coronary intervention will be randomly allocated to receive either atorvastatin or pravastatin with the treatment goal of lowering their low-density lipoprotein-cholesterol level below 100 mg/dl for 2 years. The primary endpoint will be death due to any cause, nonfatal MI, nonfatal stroke, unstable angina, or congestive heart failure requiring hospital admission, or any type of coronary revascularization. CONCLUSION: This is the first multicenter trial to compare the effects and safety of lipophilic and hydrophilic statin therapy in Japanese patients with AMI. It addresses an important issue and could influence the use of statin treatment in the secondary prevention of coronary artery disease.

Coagulation activity is increased in the left atria of patients with paroxysmal atrial fibrillation during the non-paroxysmal period. Comparison with chronic atrial fibrillation.

BACKGROUND: Evaluation of plasma markers of thrombin activity (thrombin-antithrombin III complex: TAT), active fibrinolysis (plasmin-alpha 2-plasmin inhibitor complex: PIC), and platelet activity (platelet factor 4: PF4) is useful for identifying patients with various cardiovascular disorders who are at high risk of thromboembolism. In this study, these markers were investigated in the left atria (LA) of patients with paroxysmal atrial fibrillation (pAF) in the non-paroxysmal period. METHODS AND RESULTS: Patients with pAF (n=10) and chronic AF (cAF) (n=10) were enrolled. TAT, PIC, and PF4 levels were determined in blood samples obtained from the LA of AF patients before radiofrequency catheter ablation. TAT levels were high in both pAF and cAF patients. PF4 levels were slightly elevated in both groups, but there was no significant difference between the groups. On the other hand, PIC levels in both groups were almost within normal limits, again with no significant difference between groups. CONCLUSIONS: Coagulation activity is elevated in the LA of patients with pAF, even in the non-paroxysmal period, so these patients are at high risk of thromboembolism and anticoagulant therapy is indicated.

Noninvasive evaluation of coronary endothelial function following sirolimus-eluting stent implantation by using positron emission tomography.

OBJECTIVE: Several invasive studies have reported delayed reendothelialization and endothelial dysfunction following sirolimus-eluting stent (SES) implantation. We evaluated the changes in coronary endothelial function following SES implantation by using a noninvasive method that involved positron emission tomography and cold pressor testing (CPT). METHODS: The study was conducted on 14 lesions on which percutaneous coronary intervention (PCI) was successively performed. The lesions were classified into 2 groups depending on the PCI performed: the conventional PCI group, in which 7 conventional PCIs (plain old balloon angioplasty or bare-metal stents) were performed, and the SES group, in which 7 SESs were implanted. Coronary endothelial function was defined as the percent increase in the myocardial blood flow (MBF) during CPT. RESULTS: The resting MBF in the segments distal to the PCI sites did not differ between the conventional PCI and SES groups; however, the MBF significantly decreased in the SES group during CPT. CONCLUSIONS: These data suggest that SES implantation induces coronary endothelial dysfunction in the segments distal to the PCI sites.

Elevated osteopontin levels in patients with peripheral arterial disease.

This study was carried out to compare concentrations of osteopontin (OPN) and osteoprotegerin (OPG) in peripheral arterial disease (PAD). The study population consisted of 200 consecutive subjects in whom both OPN/OPG and ankle-brachial index were measured. It was found that OPN levels, but not OPG levels, were significantly more increased in patients with PAD than those without PAD. Serum OPN levels were significantly lower in subjects with angiotensin converting enzyme inhibitors or angiotensin II receptor blockers than those without these agents. In this study, it has been demonstrated for the first time that serum OPN levels are related to PAD. Inhibition of renin- angiotensin system could decrease OPN levels and prevent the progression of PAD.

Fluvastatin improves arterial stiffness in patients with coronary artery disease and hyperlipidemia: a 5-year follow-up study.

BACKGROUND: The present study was designed to test the hypothesis that fluvastatin might improve arterial stiffness, as assessed with pulse wave velocity (PWV), in patients with coronary artery disease (CAD) and hyperlipidemia over the long term. METHODS AND RESULTS: Ninety-three patients were randomly assigned to either fluvastatin (group A, n=50) or bezafibrate (group B, n=43) and followed for 5 years. There was no difference in the clinical findings between the 2 groups. In group A, there was a progressive reduction in the brachial-ankle PWV along with a decrease in serum low-density lipoprotein-cholesterol (LDL-C) and C-reactive protein (CRP) by 12 months after fluvastatin, and the improvement was maintained until 5 years after treatment. In group B, despite identical lowering of the serum lipid, PWV was progressively increased. In group A, the percentage change in PWV correlated significantly with that of the serum CRP (r=0.49, p<0.001), but not with that of the serum LDL-C after treatment. CONCLUSIONS: The beneficial vascular effects of fluvastatin persisted for a long period in patients with CAD and hyperlipidemia. Its anti-inflammatory action might contribute to the favorable effects on arterial stiffness.

Gigantic bronchial artery aneurysm treated with transcatheter arterial embolization: a case report.

Bronchial artery aneurysm (BAA) is a rare condition. Rupture of BAA can cause critical hemorrhage, and intervention for BAA is thus recommended. A 69-year-old woman presented with BAA 70 mm in diameter in the right hilum of the lung. Transcatheter arterial embolization for afferent arteries of the BAA was performed and the BAA has subsequently been shrinking as observed by radiography. We present this case and a brief review of management of BAA based on the literature.

Successful treatment of primary cardiac angiosarcoma with docetaxel and radiotherapy.

A 49-year-old man was admitted for primary cardiac angiosarcoma with a cardiac tamponade. Transthoracic echocardiography and contrast-enhanced computed tomography scan demonstrated a large mass in the right atrium and thickening of the right ventricular wall. 18F-labeled deoxyglucose (FDG) positron emission tomography (PET) scan showed increased FDG uptake in the mediastinum and over the heart. The patient responded to combination therapy with docetaxel and radiotherapy and tolerated the treatment well, except for radiation esophagitis, which required a soft diet and resolved 1 month after treatment. This combination therapy resulted in a minimal response with slight regression in the tumor size, but FDG-PET initially showed an increase in FDG uptake by the tumor that was no longer seen after combination therapy. There is no evidence of progression or metastasis even at 12 months after diagnosis.

Relationship of plasma brain-type natriuretic peptide levels to left ventricular longitudinal function in patients with congestive heart failure assessed by strain Doppler imaging.

BACKGROUND: Plasma brain-type natriuretic peptide (BNP) levels have been used to diagnose congestive heart failure (CHF), but significant heterogeneity in BNP levels is not explained by standard echocardiographic indexes of cardiac function. Recent data indicate that longitudinal left ventricular (LV) dysfunction exists in the presence of normal standard echocardiographic indexes and BNP was predicted well by LV longitudinal systolic velocity. We examined whether longitudinal LV function assessed by strain or strain rate (SR) tissue Doppler imaging could be determinants of plasma BNP levels in patients with CHF. METHODS: We examined 137 consecutive patients with suspected CHF. Standard echocardiography and Doppler flow records were obtained. Strain, and SR at the basal and mid parts of the ventricle were investigated in 2 apical views. Tissue velocities of mitral annulus were also obtained. Plasma BNP levels were measured on the day of echocardiography. RESULTS: Multivariate analysis showed the mean longitudinal LV strain (r=0.75, P<0.0001) to be an independent powerful predictor of plasma log BNP levels. Subgroup analysis (ejection fraction <50%, n=30; ejection fraction >50%, n=107) also showed that the mean longitudinal LV strain (r=0.89, P<0.0001; r=0.68, P<0.0001, respectively) was only an independent predictor of log BNP levels. CONCLUSION: Longitudinal LV strain is closely related to log BNP levels in patients with CHF, both in patients with systolic and diastolic heart failure.