Effects of Switching from Treatment with Amlodipine and Atorvastatin Using Two Pills to an Equal Dose of Single-Pill Therapy in Japanese Outpatients.

This study examined whether switching from amlodipine and atorvastatin treatment using two pills to an equal dose of single-pill therapy is useful in Japanese outpatients. We retrospectively reviewed data obtained from 94 outpatients for whom treatment with two pills, namely amlodipine and atorvastatin, was switched to an equal dose of single-pill therapy in 11 hospitals. The criterion for enrollment in this study was that patients had switched their medication without changing other anti-hypertensive or anti-cholesterol drugs. Neither systolic nor diastolic blood pressure changed significantly after switching to an equal dose of single-pill therapy, whereas low-density lipoprotein (LDL) cholesterol levels significantly decreased after the medication was switched from 94±24 mg/dl to 89±17 mg/dl (p=0.015). A switch from medication with two separate pills of amlodipine and atorvastatin to an equal dose of single-pill therapy resulted in an overall decrease in LDL cholesterol. The results indicated that the switch to single-pill therapy might be a useful treatment.

Prognostic Significance of Non-Dilated Left Ventricular Size and Mitral Regurgitation in Patients With Dilated Phase of Hypertrophic Cardiomyopathy.

Although a subtype of hypertrophic cardiomyopathy (HCM), dilated phase of HCM (D-HCM) characterized by left ventricular (LV) systolic dysfunction, has been reported to have a poor prognosis, some patients with D-HCM survive for a relatively long period. The degree of LV dilatation and functional mitral regurgitation (MR) are generally thought to be important predictors of poor prognosis in patients with LV systolic dysfunction. However, there is little information available on the relations among LV size, presence of significant MR, and prognosis in D-HCM patients.We retrospectively studied 31 patients with D-HCM to determine whether echocardiographic assessment of LV size and MR provides incremental prognostic information.During a follow-up period of 5.6 ± 4.2 years, there were 13 cardiovascular deaths. When the patients were divided into two groups by LV size at diagnosis of D-HCM, a non-dilated LV group (LV end-diastolic diameter (LVEDD) < 50 mm, n = 9) and a dilated LV group (LVEDD ≥ 50 mm, n = 22), the clinical course in the non-dilated LV group was significantly worse. As for the clinical impact of MR, no patient in the non-dilated LV group showed significant MR and 7 of the patients with dilated LV size showed significant MR during follow-up. Once significant MR was reached, cardiovascular deaths were significantly more frequent in patients with MR.Patients with D-HCM, particularly those with less LV dilatation at diagnosis of dilated phase and with significant MR during follow-up, have a poor prognosis.

Prevalence and clinical features of Fabry disease in Japanese male patients with diagnosis of hypertrophic cardiomyopathy.

BACKGROUND: The prevalence of Fabry disease (FD) in Japanese patients presenting with unexplained left ventricular hypertrophy (LVH) has remained unclear. METHODS: We measured plasma α-galactosidase A activity in 177 men with a diagnosis of hypertrophic cardiomyopathy (HCM) (maximum LV wall thickness ≥15mm). RESULTS: Two patients (1.1%) showed very low α-galactosidase A activity [0.0 and 0.3nmol/hr/ml (normal range: 3.6-17.6nmol/hr/ml)], and a clinical diagnosis of cardiac variant of FD was finally made. One patient was a 55-year-old man who came to our hospital because of abnormal results of electrocardiography and showed concentric LVH in echocardiography. A missense mutation, R112L, was identified. The other was a 74-year-old man who had been diagnosed with HCM at the age of 60 years in another hospital and was referred for evaluation of repeated hospitalization for heart failure. Although echocardiography revealed asymmetric septal hypertrophy (ASH) with interventricular septal wall thickness of 16mm and posterior wall thickness of 11mm and reduced LV ejection fraction with hypokinetic posterior wall motion, his echocardiographic findings at the initial diagnosis of HCM were not ASH but concentric LVH with normal LV systolic function. A splicing mutation, IVS4+919G>A, was identified. CONCLUSIONS: The prevalence of FD in Japanese male patients with a clinical diagnosis of HCM was found to be 1.1%. These patients showed late onset and concentric LVH at initial presentation.

Erythrocyte creatine as a marker of intravascular hemolysis due to left ventricular outflow tract obstruction in hypertrophic cardiomyopathy.

BACKGROUND: Erythrocyte creatine, a marker of erythrocyte age that increases with shortening of erythrocyte survival, has been reported to be a quantitative and reliable marker for intravascular hemolysis. We hypothesized that hemolysis could also occur due to intraventricular obstruction in patients with hypertrophic cardiomyopathy (HCM). The purpose of this study was to examine the presence of subclinical hemolysis and the relation between intravascular hemolysis and intraventricular pressure gradient (IVPG). METHODS AND RESULTS: We measured erythrocyte creatine in 92 HCM patients. Twelve patients had left ventricular outflow tract obstruction (LVOTO), 4 had midventricular obstruction (MVO), and the remaining 76 were non-obstructive. Erythrocyte creatine levels ranged from 0.92 to 4.36µmol/g hemoglobin. Higher levels of erythrocyte creatine were associated with higher IVPG (r=0.437, p<0.001). If erythrocyte creatine levels are high (≥1.8µmol/g hemoglobin), subclinical hemolysis is considered to be present. Half of LVOTO patients and no MVO patients showed high erythrocyte creatine levels. Although non-obstructive patients did not show significant intraventricular obstruction at rest, some showed high erythrocyte creatine levels. When LVOT-PG was measured during the strain phase of the Valsalva maneuver in 20 non-obstructive patients, 7 of those 20 patients showed LVOTO. In the 20 patients, there was no relation between erythrocyte creatine levels and LVOT-PG before the Valsalva maneuver (r=0.125, p=0.600), whereas there was a significant correlation between erythrocyte creatine and LVOT-PG provoked by the Valsalva maneuver (r=0.695, p=0.001). CONCLUSIONS: There is biochemical evidence of subclinical hemolysis in patients with HCM, and this hemolysis seems to be associated with LVOTO provoked by daily physical activities.

Metastatic cardiac tumor from urothelial carcinoma detected by transthoracic echocardiography: a case report.

INTRODUCTION: Cardiac metastasis of urothelial carcinoma is a very rare but clinically important complication. Most cardiac metastases are asymptomatic; symptoms from cardiac metastasis were seen in advanced stage and many of these cases were reported to have a poor prognosis. So it is important to find asymptomatic cardiac metastasis and to start chemotherapy early in order to improve the patient's prognosis. CASE PRESENTATION: A 73-year-old Asian man was referred to our hospital because of a right ventricular tumor. He had a history of left ureteral cancer 9 years ago. In screening echocardiography for paroxysmal atrial fibrillation, a low echogenic tumor was detected in his right ventricular apex, and characteristic ST segment elevation was detected in electrocardiography. An (18)F-fluorodeoxyglucose positron emission tomography revealed abnormal uptake in his right ventricular apex tumor and prostate, and a biopsy of the prostatic tumor showed urothelial carcinoma cells. He received systemic gemcitabine, paclitaxel and cisplatin chemotherapy for the urothelial carcinoma, and the cardiac tumor size was reduced temporarily. Finally, he died of multiple organ failure 16 months after his first admission, but his survival period was relatively longer than previous reports. CONCLUSIONS: We experienced a case of a metastatic cardiac tumor from urothelial carcinoma. We found asymptomatic cardiac metastasis by screening echocardiography and electrocardiography. Our patient received systemic chemotherapy and his survival period was relatively longer than previous reports. Electrocardiography and echocardiography may be useful to find asymptomatic cardiac metastasis of neoplasms.

Differentiation of infiltrative cardiomyopathy from hypertrophic cardiomyopathy using high-sensitivity cardiac troponin T: a case-control study.

BACKGROUND: Because infiltrative cardiomyopathy and hypertrophic cardiomyopathy (HCM) share clinical and hemodynamic features of left ventricular (LV) hypertrophy and abnormal diastolic function, it is often difficult to distinguish these entities. METHODS: We investigated the potential role of high-sensitivity cardiac troponin T (hs-cTnT) for differentiation of infiltrative cardiomyopathy from HCM. RESULTS: The study group consisted of 46 consecutive patients with infiltrative cardiomyopathies or HCM in whom sarcomere protein gene mutations were identified at Kochi Medical School Hospital; of these, there were 11 patients with infiltrative cardiomyopathy (cardiac amyloidosis in 8 patients and Fabry disease in 3 patients) and 35 HCM patients. Serum hs-cTnT level was significantly higher in patients who had infiltrative cardiomyopathy than in those who had HCM (0.083 ± 0.057 ng/ml versus 0.027 ± 0.034 ng/ml, p < 0.001), whereas brain natriuretic peptide levels did not differ between the two groups. In two age-matched the 2 cohorts (patients evaluated at > 40 years at age), hs-cTnT level, maximum LV wall thickness, posterior wall thickness, peak early (E) transmitral filling velocity, peak early diastolic (Ea) velocity of tissue Doppler imaging at the lateral corner and E/Ea ratios at both the septal and lateral corners were significantly different between the two groups. As for diagnostic accuracy to differentiate the two groups by using receiver operating characteristic analysis, hs-cTnT was the highest value of area under the curve (0.939) and E/Ea (lateral) was second highest value (0.914). CONCLUSIONS: Serum hs-cTnT is a helpful diagnostic indicator for accurate differentiation between infiltrative cardiomyopathy and HCM.

Clinical significance of high-sensitivity cardiac troponin T in patients with dilated cardiomyopathy.

Although conventional cardiac troponin T (cTnT) and I (cTnI) markers have been reported to predict adverse outcome in dilated cardiomyopathy (DCM), the usefulness of a new-generation high-sensitivity assay of cardiac troponin T (hs-cTnT) compared with these conventional biomarkers is unclear.We performed clinical evaluation including measurements of troponin markers in 54 patients with DCM under a clinically stable condition. At baseline, the serum concentration of hs-cTnT was 0.014 ± 0.016 ng/mL and 17 (31%) of the patients showed abnormal hs-cTnT values (> 0.014 ng/mL). During a mean follow-up period of 5.1 ± 1.6 years, there were 16 cardiac events: heart failure death in 6 patients, sudden cardiac death in 2 patients, and hospitalization for heart failure in 8 patients. Patients with abnormal hs-cTnT or abnormal cTnT (> 0.01 ng/mL) values had significantly more frequent cardiac events than did those with normal hs-cTnT or cTnT values. On the other hand, abnormal cTnI (> 0.03 ng/mL) value did not reach statistical significance for these adverse events. Multivariate analysis showed that only an abnormal hs-cTnT value was an independent predictor of all cardiac events (HR: 5.68, P = 0.003). When the patients were divided into 4 groups according to the degree of hs-cTnT levels, the clinical course was significantly worse in patients with higher hs-cTnT values.These results suggest that the serum concentration of hs-cTnT provides better risk stratification in DCM patients.

A case of cardiac calcified amorphous tumor (cardiac CAT) causing acute embolism in right common iliac artery.

A 68-year-old man was admitted to our hospital for the further examination of intermittent claudication. He had been on continuous ambulatory peritoneal dialysis for 2 years. Screening transthoracic echocardiography (TTE) revealed a club-shaped tumor and a round-shaped tumor attached to mitral annulus calcification (MAC). The club-shaped tumor was swinging and plunged into the left ventricle at diastolic phase. Because of the risk of fatal embolism, we planned early surgical resection of the tumors. However, 13 days after admission, his intermittent claudication was getting worse and some part of the club-shaped tumor had vanished by TTE. Urgent iliac angiography showed that the tumor had embolized the right common iliac artery. Although we tried embolectomy using a Fogarty catheter, it was unsuccessful. We therefore treated the iliac artery stenosis by endovascular therapy and the procedure was successful. Three months later, he suffered from unstable angina and was treated by percutaneous coronary intervention. However, subacute stent thrombosis occurred after one month. After urgent treatment, we decided to treat him by coronary artery bypass graft and surgical resection of the residual tumor on MAC. The operation was performed successfully. Finally, the tumor was diagnosed as cardiac calcified amorphous tumor by its histologic features. .

Gender differences in the clinical features of hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations.

BACKGROUND: Although gender may be one of the important factors modifying phenotypic expression in hypertrophic cardiomyopathy (HCM), there has been little information on it. METHODS AND RESULTS: We investigated gender differences in the clinical features of HCM caused by cardiac myosin-binding protein C gene (MYBPC3) mutations. Sixty-one subjects (28 families) carrying MYBPC3 mutations were studied. Of the 61 subjects with MYBPC3 mutations, 50 patients including 23 female patients were phenotype-positive by echocardiography. Disease penetrance in subjects aged ≤40 years old was 92% in males and 67% in females. Females showed delayed onset of left ventricular hypertrophy compared with males in subjects who were genotype-positive. Female patients were more symptomatic at diagnosis than were males (mean New York Heart Association class: 1.7±0.8 versus 1.2±0.4, p=0.012). From a longitudinal point of view by age, no significant gender difference in cardiovascular deaths or cardiovascular events was found. During the follow-up period after diagnosis of HCM (13±8 years), female patients who were phenotype-positive had significantly more frequent heart failure events than did phenotypically affected male patients (p=0.028). CONCLUSIONS: Although females with MYBPC3 mutations showed later onset of the disease, female patients were more symptomatic at diagnosis and had more frequent heart failure events once they had developed hypertrophy.

[Successful eculizumab treatment for multiple coronary thrombosis complicated in paroxysmal nocturnal hemoglobinuria].

We herein report a rare case of paroxysmal nocturnal hemoglobinuria (PNH) who repeatedly developed coronary arterial thromboembolism. Anticoagulant therapies including heparin, aspirin as an antiplatelet agent and even drug-eluting stent placement in the coronary artery failed to prevent the recurrence of ischemic heart disease. Of note, initiating the administration of a humanized anti-C5 antibody, eculizumab, achieved prompt thrombolysis and maintenance treatment with eculizumab prevented the recurrence of thromboembolic disease in this patient. Taking these observations together, we suggest that the use of eculizumab be considered for treatment or prevention of arterial thrombosis complicated by PNH, although arterial thrombosis is an extremely rare event in the Japanese population.

A case of percutaneous transluminal renal angioplasty for partial coverage of a renal artery by a stent graft after endovascular aneurysm repair.

An elderly woman was admitted to our hospital for evaluation of an abdominal aortic aneurysm (AAA) and we decided to treat her AAA with endovascular aneurysm repair. Her renal function became worse after the operation and a renogram suggested flow disturbance of her right renal artery. We performed angiography and checked her right renal artery using intravascular ultrasound. The ultrasound revealed thrombus formation and severe stenosis caused by the stent graft. We performed percutaneous transluminal renal angioplasty at the ostium of her right renal artery. After this procedure, her renal function rapidly improved.

Cardiac calcified amorphous tumor stuck in the aortic valve that mimicked a chameleon's tongue: report of a case.

A calcified amorphous tumor (CAT) is a rare intracardiac mass that carries a risk of embolism. We herein present the case of a club-shaped CAT that originated from the calcified mitral annulus. Echocardiography indicated a pendular motion of the mass and repeated entrapment by a stenotic aortic valve that was sustained for several beats, mimicking a chameleon's tongue. An emergency operation was performed because of the risk of embolism, as well as potential progression of cardiac failure due to worsening aortic valve stenosis. The histological findings were consistent with the diagnosis of a CAT. This report describes a case of an intracardiac tumor that showed unique motion like a chameleon's tongue.

Significance of high-sensitivity cardiac troponin T in hypertrophic cardiomyopathy.

OBJECTIVES: This study investigated the significance of the serum high-sensitivity cardiac troponin T (hs-cTnT) marker for prediction of adverse events in hypertrophic cardiomyopathy (HCM). BACKGROUND: Although serum cardiac troponins as sensitive and specific markers of myocardial injury have become well-established diagnostic and prognostic markers in acute coronary syndrome, the usefulness of hs-cTnT for prediction of cardiovascular events in patients with HCM is unclear. METHODS: We performed clinical evaluation, including measurements of hs-cTnT in 183 consecutive patients with HCM. RESULTS: Of 183 HCM patients, 99 (54%) showed abnormal hs-cTnT values (>0.014 ng/ml). During a mean follow-up of 4.1 ± 2.0 years, 32 (32%) of the 99 patients in the abnormal hs-cTnT group, but only 6 (7%) of 84 patients with normal hs-cTnT values, experienced cardiovascular events: cardiovascular deaths, unplanned heart failure admissions, sustained ventricular tachycardia, embolic events, and progression to New York Heart Association functional class III or IV status (hazard ratio [HR]: 5.05, p < 0.001). Abnormal hs-cTnT value remained an independent predictor of these cardiovascular events after multivariate analysis (HR: 3.23, p = 0.012). Furthermore, in the abnormal hs-cTnT group, overall risk increased with an increase in hs-cTnT value (HR: 1.89/hs-cTnT 1 SD increase in the logarithmic scale, 95% confidence interval: 1.13 to 3.15; p = 0.015 [SD: 0.59]). CONCLUSIONS: In patients with HCM, an abnormal serum concentration of hs-cTnT is an independent predictor of adverse outcome, and a higher degree of abnormality in hs-cTnT value is associated with a greater risk of cardiovascular events.

Inferior acute myocardial infarction due to acute cardiac herniation after right pneumonectomy.

A 69-year-old man underwent right intrapericardial pneumonectomy for lung cancer. After 24 h, he went into shock with inferior acute myocardial infarction. We performed urgent coronary angiography, which revealed total occlusion of the mid-right coronary artery. Intravascular ultrasound showed that the artery seemed to be compressed from the pericardial side. We implanted a coronary stent because the lesion was refractory to balloon dilatation. After the procedure, we performed computed tomography and cardiac herniation was diagnosed. Emergency thoracotomy was performed to return the herniated heart to its normal position. This patient was discharged 38 days after initial surgery.

Turbulent left-to-right shunt flow through the interatrial septum suggesting high left atrial pressure in patients with heart failure with preserved ejection fraction.

Lutembacher's syndrome is a combination of interatrial septal (IAS) defect or patent foramen ovale (PFO), associated with mitral stenosis. High left atrial (LA) pressure in mitral stenosis exaggerates left-to-right shunt in patients with interatrial communication. We present a case of heart failure with preserved ejection fraction. Echocardiography revealed normal left ventricular systolic function without mitral stenosis and turbulent left-to-right shunt through the IAS. The peak velocity of the shunt flow was 2.3 m/s and the estimated pressure gradient was 22 mmHg, indicating high LA pressure. The presence of turbulent left-to-right shunt through the IAS is helpful for detecting high LA pressure.

Abdominal visceral fat thickness measured by ultrasonography predicts the presence and severity of coronary artery disease.

Abdominal visceral fat plays a critical role in the pathogenesis of metabolic syndrome, which is a risk factor for coronary artery disease (CAD). Ultrasonography (US) distinctively quantifies visceral fat and subcutaneous fat. We measured the maximum preperitoneal visceral fat thickness (Vmax) and the minimum subcutaneous fat thickness (Smin) by US in 185 patients who underwent coronary angiography. Although the 144 patients with CAD had larger Vmax (8.8 ± 3.6 vs. 6.4 ± 2.8 mm; p < 0.001) than those without, there was no difference in Smin. Vmax of 6.9 mm or higher was an independent predictor of CAD (odds ratio, 3.710, p = 0.008) by multiple logistic regression analysis. Vmax significantly correlated with the number of diseased vessels. Assessment of abdominal visceral fat by US gives us incremental information beyond conventional risk factors for predicting CAD in routine clinical practice.

Smoking cessation after discharge among Japanese patients with established ischemic heart disease: a prospective cohort study.

In this prospective cohort study for Japanese patients with established ischemic heart disease (IHD), the authors investigated the rate of success of smoking cessation 3 months after hospital discharge and its related factors. The subjects included 90 current smokers admitted for IHD. A total of 58 subjects (64%) had quit smoking for 3 months after being discharged. In comparison with subjects with acute myocardial infarction, those with stable angina (SA) showed a significantly lower frequency of smoking cessation (relative risk of resuming smoking (95% confidence interval):2.06 (1.09, 3.92), p=0.036). This relationship remained significant even after controlling for sex, age, and scores of the Fagerstrom Test for Nicotine Dependence (adjusted odds ratio:3.39 (1.01, 11.37), p=0.048). However, it became insignificant when hospital admission followed by emergency medical service (EMS) care was additionally adjusted (adjusted odds ratio:2.48 (0.36, 16.97), p=0.356). The smoking cessation rate in this study was identical to that observed in studies conducted in Japan prior to the recent social changes with regard to tobacco use. SA still appears to be a risk factor for smoking resumption after discharge. Experiencing EMS care would be an intermediate variable in this relationship.