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BACKGROUND A strategy to predict mortality in elderly heart failure (HF) patients has not been established.Methods and Results:We retrospectively enrolled 413 HF patients aged ≥65 years (mean age 78 years) who had received comprehensive cardiac rehabilitation (CR) during hospitalization. Basic activities of daily life were assessed before discharge using the Barthel index (BI). Of 413 HF patients, 116 (28%) died during a median follow-up period of 1.90 years (interquartile range 1.20-3.23 years). An adjusted dose-dependent association analysis showed that the hazard ratio (HR) of mortality increased in an almost linear manner as the BI score decreased, and that a BI score of 85 corresponded to an HR of 1.0. Kaplan-Meier survival curves showed that the survival rate was lower for patients with a low BI (

Reportedly, approximately 9.9%-12.4% of patients with Takotsubo syndrome (TTS) are complicated with cardiogenic shock (CS) and its prognosis remains poor even with the support of conventional mechanical circulatory assist devices including intra-aortic balloon pumping and venoarterial extracorporeal membrane oxygenation. Impella, a novel percutaneous left ventricular assist device, provides strong circulatory support together with the unloading of the left ventricle, and it is theoretically a promising mechanical circulatory assist device for TTS. In this case study, we report four consecutive patients with TTS complicated with CS who were successfully resuscitated using the Impella support.

Aims The clinical outcome of heart failure (HF) is complicated by the presence of multiple comorbidities including malnutrition and cachexia, and prediction of the outcome is still difficult in each patient. Metabolomics including amino acid profiling enables detection of alterations in whole body metabolism. The aim of this study was to determine whether plasma amino acid profiling improves prediction of clinical outcomes in patients with HF. Methods and results We retrospectively examined 301 HF patients (70 ± 15 years old; 59% male). Blood samples for measurements of amino acid concentrations were collected in a fasting state after stabilization of HF. Plasma amino acid concentrations were measured using ultraperformance liquid chromatography. Clinical endpoint of this study was adverse event defined as all‐cause death and unscheduled readmission due to worsening HF or lethal arrhythmia. During a mean follow‐up period of 380 ± 214 days, 40 patients (13%) had adverse events. Results of analyses of variable importance in projection score, a measure of a variable's importance in partial least squares–discriminant analysis (PLS‐DA) showed that the top five amino acids being associated with adverse events were 3‐methylhistidine (3‐Me‐His), β‐alanine, valine, hydroxyproline, and tryptophan. Multivariate Cox‐proportional hazard analyses indicated that a high 3‐Me‐His concentration and low β‐alanine and valine concentrations were independently associated with adverse events. When HF patients were divided according to the cut‐off values of amino acids calculated from receiver operating characteristic curves, Kaplan–Meier survival curves showed that event‐free survival rates were lower in HF patients with high 3‐Me‐His than in HF patients with low 3‐Me‐His (68% vs. 91%, P

Multiple factors regulate glucagon-like peptide-1 (GLP-1) secretion, but a group of apparently healthy subjects showed blunted responses of GLP-1 secretion in our previous study. In this study, we examined whether the reduction in GLP-1 secretory capacity is associated with increased extent of coronary artery stenosis in non-diabetic patients. Non-diabetic patients who were admitted for coronary angiography without a history of coronary interventions were enrolled. Coronary artery stenosis was quantified by Gensini score (GS), and GS ≥ 10 was used as an outcome variable based on its predictive value for cardiovascular events. The patients (mean age, 66.5 ± 8.8 years; 71% males, n = 173) underwent oral 75 g-glucose tolerant tests for determination of glucose, insulin and active GLP-1 levels. The area under the curve of plasma active GLP-1 (AUC-GLP-1) was determined as an index of GLP-1 secretory capacity. AUC-GLP-1 was not correlated with fasting glucose, AUC-glucose, serum lipids or indices of insulin sensitivity. In multivariate logistic regression analysis for GS ≥ 10, AUC-GLP-1

Cardiac sarcoidosis (CS) is frequently complicated by fatal ventricular arrhythmias. T-peak to T-end interval to QT interval ratio (TpTe/QT) on electrocardiograms (ECG) was proposed as a marker of ventricular repolarization dispersion. Although this ratio could be associated with the incidence of ventricular arrhythmias in cardiovascular diseases, its prognostic implication in patients with CS is unclear. We sought to investigate whether TpTe/QT was associated with long-term clinical outcomes in patients with CS. Ninety consecutive patients with CS in 2 tertiary hospitals who had ECG data before initiation of immunosuppressive therapy between November 1995 and March 2019 were examined. The primary outcome was a composite of advanced atrioventricular block, ventricular tachycardia or ventricular fibrillation (VT/VF), heart failure hospitalization, and all-cause death. During a median follow-up period of 4.70 (interquartile range 2.06-7.23) years, the primary outcome occurred in 21 patients (23.3%). Survival analyses revealed that the primary outcome (p0.001), especially VT/VF or sudden cardiac death (p = 0.002), occurred more frequently in patients with higher TpTe/QT (≥ 0.242, the median) than in those with lower TpTe/QT. Multivariable Cox regression analysis showed that a higher TpTe/QT was independently associated with increased subsequent risk of adverse events (hazard ratio1.11, 95% confidence interval 1.03-1.20, p = 0.008) even after adjustment for the significant covariates. In conclusion, a higher TpTe/QT was associated with worse long-term clinical outcomes, especially fatal ventricular arrhythmic events, in patients with cardiac sarcoidosis, suggesting the importance of assessing TpTe/QT as a surrogate for risk stratification in these patients.

Aims A high prevalence of muscle wasting, that is, reduction in muscle mass, in patients with peripheral artery disease (PAD) and heart failure (HF) has been reported. However, whether the association between PAD and muscle wasting is independent of shared risk factors such as diabetes mellitus has not been examined. Methods and results We retrospectively enrolled 440 HF patients (mean age, 74 years; inter‐quartile range, 64–82 years; 52% male). Muscle wasting was defined as an appendicular skeletal muscle mass index (ASMI) of

Muscle wasting, that is, reduction in muscle mass, is frequently observed in patients with chronic heart failure (CHF) and diabetes mellitus (DM).We retrospectively examined 185 patients with CHF (median age of 71 years [interquartile range, 61-78 years]; 64% male) who received a dual-energy X-ray absorptiometry scan for assessment of appendicular skeletal muscle mass index (ASMI).Seventy patients with CHF (38%) had DM. Patients with DM had higher prevalences of ischemic heart disease and hypertension, lower levels of estimated glomerular filtration rate (eGFR) and ASMI, and higher levels of plasma renin activity (PRA) than did patients without DM. In simple regression analyses, ASMI was positively correlated with the Mini Nutritional Assessment Short Form (MNA-SF) score and levels of hemoglobin, eGFR, and fasting plasma insulin and was negatively correlated with levels of N-terminal pro B-type natriuretic peptide, PRA, and cortisol. In multiple linear regression analyses, age, MNA-SF score, DM, fasting plasma insulin level, and PRA were independently associated with ASMI. When multiple linear regression analyses were separately performed in a non-DM group and a DM group, MNA-SF score and fasting plasma insulin level were independent variables for ASMI in both groups. PRA was independently associated with ASMI in the DM group but not in the non-DM group, whereas cortisol concentration was independently associated with ASMI only in the non-DM group.In addition to malnutrition and reduction in plasma insulin, renin-angiotensin system activation may be responsible for the development of muscle wasting in CHF patients with DM.背景: 慢性心力衰竭(chronic heart failure, CHF)和糖尿病(diabetes mellitus, DM)患者常出现肌肉萎缩, 即肌肉质量减少。 方法: 我们回顾了185例CHF患者[中位年龄71岁(四分位数范围61~78岁), 其中64%为男性], 他们接受了双能X线骨密度仪扫描以评估全身四肢骨骼肌质量指数(appendicular skeletal muscle mass index, ASMI)。 结果: 70例CHF患者(38%)合并DM。与非糖尿病患者相比, 糖尿病患者缺血性心脏病和高血压的患病率更高, 肾小球滤过率(estimated glomerular filtration rate, eGFR)和ASMI水平更低, 血浆肾素活性(plasma renin activity, PRA)水平更高。在简单回归分析中, ASMI与MNA-SF(微型营养评估简表)评分、血红蛋白、eGFR和空腹血浆胰岛素水平呈正相关, 与N端B型利钠肽原、PRA和皮质醇水平呈负相关。多元线性回归分析显示, 年龄、MNA-SF评分、糖尿病、空腹血浆胰岛素水平和PRA与ASMI独立相关。在非糖尿病组和糖尿病组分别进行多元线性回归分析时, MNA-SF评分和空腹血浆胰岛素水平是两组ASMI的独立变量。在糖尿病组中, PRA与ASMI独立相关, 而在非糖尿病组中, 皮质醇浓度与ASMI无关, 而皮质醇浓度仅在非糖尿病组中与ASMI独立相关。 结论: 除了营养不良和血浆胰岛素降低外, 肾素-血管紧张素系统激活可能是心力衰竭合并糖尿病患者肌肉萎缩的原因。.

Background and aim Glucagon-like peptide-1 (GLP-1) regulates insulin secretion and also affords pleiotropic effects including protective effects on blood vessels. Multiple factors regulate GLP-1 secretion after meals, but a group of apparently healthy subjects showed blunted responses of GLP-1 secretion in our previous study. In this study, we examined the possibility that the reduced capacity of GLP-1 secretion is associated with increased extent of coronary artery stenosis in non-diabetic patients. Methods and results Non-diabetic patients who were admitted for coronary angiography without a history of coronary interventions were enrolled. Coronary artery stenosis was quantified by Gensini score (GS), and GS ≥10 was used as an outcome variable based on results of earlier studies indicating its predictive value for cardiovascular events. The patients (mean age, 66.5±8.8 years; 71% males, n=173) underwent oral 75 g-glucose tolerant tests for determination of glucose, insulin and active GLP-1 levels. The area under the curve of plasma active GLP-1 (AUC-GLP-1) was determined as an index of GLP-1 secretion capacity. AUC-GLP-1 was not correlated with fasting glucose, AUC-glucose, serum lipids, indices of insulin sensitivity or estimated glomerular filtration rate. In multivariate logistic regression analysis for GS ≥10, AUC-GLP-1 < median, age and hypertension were selected as explanatory variables, though fasting GLP-1 level was not selected. Conclusion The findings indicate significant association of reduced GLP-1 secretion capacity with increased extent of coronary artery stenosis in non-diabetic patients. A causal relationship between change in GLP-1 secretion capacity and coronary stenosis remains to be examined by a longitudinal study Funding Acknowledgement Type of funding sources: Private company. Main funding source(s): This study was supported in part by a research grant from Investigator-Initiated Studies Program of Merck Sharp & Dohme Corp./MSD K.K.

Background Sarcoidosis is a systemic granulomatous disease that affects multiple organs. Cardiac involvement is a key determinant of poor clinical outcomes in the patients with sarcoidosis, as it causes congestive heart failure, conduction abnormalities, ventricular tachycardia and fibrillation (VT/VF), and sudden cardiac death (SCD). Although implantable cardioverter defibrillators (ICDs) are used to prevent SCD from VT/VF in patients with cardiac sarcoidosis (CS), the generalizability of the AHA/ACC/HRS guidelines for the Japanese CS patients remains unclear. Purpose We aimed to assess, among Japanese patients with CS, the ICD recommendations from the 2017 AHA/ACC/HRS Guidelines for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death. Methods We examined 188 consecutive patients with CS in two tertiary hospitals between 1979 and 2020. The primary outcome was defined as a composite outcome involving SCD or ventricular arrhythmic events. Ventricular arrhythmic events were defined as either emergency treatment for VF or sustained VT, which included appropriate ICD therapy. Results During a median follow-up period of 5.68 (IQR: 4.87–6.70) years, the primary outcome occurred in 44 patients (23%), which included 6 cases of SCD and 38 cases of VT/VF. ICD implant was indicated based on left ventricular ejection fraction (LVEF) of ≤35% (class I recommendation) in 62 patients, with an annualized event rate of 3.93%. A LVEF of >35% with a need for a permanent pacemaker (class IIa recommendation) was observed for 53 patients, with an annualized event rate of 2.54%. A LVEF of >35% with late gadolinium enhancement (LGE) during cardiovascular magnetic resonance (class IIa recommendation) was observed for 62 patients, with an annualized event rate of 2.38% (Figure A). Kaplan-Meier analyses revealed that patients with a class I recommendation for ICD implantation had a significant higher incidence of the primary outcome, compared to patients with a class IIa recommendation and patients with no indication for ICD implantation (P=0.03). However, there were no significant differences in the incidence of the primary outcome between patients with a LVEF of >35% and a need for a permanent pacemaker and patients with a class I recommendation (P=0.08) or patients with a LVEF of ≤35% (P=0.31). Moreover, there was no significant difference in the incidence of the primary outcome between patients with a LVEF of >35% and LGE on cardiovascular magnetic resonance and patients with a class I recommendation (P=0.054) or patients with a LVEF of ≤35% (P=0.22) (Figure B). Conclusions The American guideline recommendations for ICD implantation might be applicable to Japanese patients with CS. Implantation of an ICD may need to be considered in these patients if they require a permanent pacemaker or have LGE, regardless of LVEF. Funding Acknowledgement Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Japan Society for the Promotion of Science

Background There are a lot of reports of the renal failure and heart failure due to coarctation of the aorta. However, there are no case reports in which revascularization dramatically improved left ventricular function in patients with progressive decline in left ventricular function. Herein, we present a rare case in which the left ventricular function was dramatically improved by surgical treatment for progressive left ventricular dysfunction due to atypical coarctation of the aorta. Case presentation A 58-year-old man underwent left axillary artery-bilateral femoral artery bypass at another hospital for atypical coarctation of the aorta due to Takayasu’s arteritis. Approximately 10 years later, he was re-hospitalized for heart failure, and the left ventricular ejection fraction gradually decreased to 28%. Computed tomography showed severe calcification and stenosis at the same site from the peripheral thoracic descending aorta to the lower abdominal aorta of the renal artery, and aortography showed delayed bilateral renal artery blood flow. An increase in plasma renin activity was also observed. Despite the administration of multiple antihypertensive drugs, blood pressure control was insufficient. We decided to perform surgical treatment to improve progressive cardiac dysfunction due to increased afterload and activated plasma renin activity. Descending thoracic aorta-abdominal aorta bypass and revascularization of the bilateral renal arteries via the great saphenous vein grafts were performed. Postoperative blood pressure control was improved, and the dose of antihypertensive drugs could be reduced. Plasma renin activity decreased, and transthoracic echocardiography 1.5 years later showed an improvement in contractility with a left ventricular ejection fraction of 58%. Conclusion In atypical coarctation of the aorta in patients with decreased bilateral renal blood flow, heart failure due to renal hypertension, and progressive decrease in left ventricular contractility, descending thoracic aorta-abdominal aortic bypass and bilateral renal artery recirculation can be extremely effective.

Takayasu arteritis (TA) is a large vessel vasculitis of unknown aetiology characterized by chronic inflammatory changes of the aorta and its major branches. We report the active TA case who had severe heart failure due to acute myocardial infarction and aortic regurgitation. Bentall procedure was successfully performed, but he had severely depressed left ventricular function and muscle wasting together with vascular inflammation. The treatment with tocilizumab, an interleukin‐6 receptor monoclonal antibody, in addition to prednisolone and standard heart failure therapy led to prompt remission of TA activity and improvement of left ventricular function and muscle wasting. Taken together with possible involvement of interleukin‐6 in the pathogenesis of heart failure and muscle wasting, inhibition of interleukin‐6 receptor signalling by tocilizumab may be a safe and reasonable approach in the treatment of active TA with heart failure and muscle wasting.

Objectives This study aimed to assess, among Japanese patients with cardiac sarcoidosis (CS), the implantable cardioverter-defibrillator (ICD) recommendations from the 2017 American Heart Association/American College of Cardiology/Heart Rhythm Society (AHA/ACC/HRS) guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death (SCD). Background Although ICDs are used to prevent SCD from ventricular tachycardia or ventricular fibrillation (VT/VF) in patients with CS, the generalizability of the AHA/ACC/HRS guidelines for Japanese patients with CS remains unclear. Methods This study examined 188 consecutive patients with CS in 2 tertiary hospitals between 1979 and 2020. Patients were followed for a primary outcome of VT/VF or SCD. Results During a median follow-up of 5.68 years, the primary outcome occurred in 44 patients (23%). Patients with a class I recommendation for ICD implantation showed the highest incidence of the primary outcome among patients in whom the guideline recommendations for ICD implantation were used (log-rank test; p = 0.03). However, compared with patients with left ventricular ejection fractions (LVEFs) ≤35%, there was no significant difference in the incidence of the primary outcome among patients with LVEFs >35% and those who required a permanent pacemaker (p = 0.31); similar results were observed in those with LVEFs >35% and late gadolinium enhancement during cardiovascular magnetic resonance imaging (p = 0.22). Conclusions The American guideline recommendations for ICD implantation might be applicable to Japanese patients with CS. Implantation of an ICD may need to be considered in these patients if they require a permanent pacemaker or have late gadolinium enhancement, regardless of LVEF.

BACKGROUND The clinical significance of osteoporosis in chronic heart failure (CHF) remains unclear.Methods and Results:A total of 303 CHF patients (75 years, [interquartile range (IQR) 66-82 years]; 41% female) were retrospectively examined. Bone mineral densities (BMDs) at the lumbar spine, femoral neck, and total femur were measured by using dual-energy X-ray absorptiometry (DEXA), and osteoporosis was diagnosed when the BMD at any of the 3 sites was

Background Although high body mass index (BMI) is a risk factor of heart failure (HF), HF patients with a higher BMI had a lower mortality rate than that in HF patients with normal or lower BMI, a phenomenon that has been termed the “obesity paradox”. However, the relationship between body composition, i.e., fat or muscle mass, and clinical outcome in HF remains unclear. Methods We retrospectively analyzed data for 198 consecutive HF patients (76 years of age; males, 49%). Patients who were admitted to our institute for diagnosis and management of HF and received a dual-energy X-ray absorptiometry scan were included regardless of left ventricular ejection fraction (LVEF) categories. Muscle wasting was defined as appendicular skeletal muscle mass index 2 in males and 2 in females. Increased percent body fat mass (increased FM) was defined as percent body fat > 25% in males and > 30% in females. Results The median age of the patients was 76 years (interquartile range [IQR], 67–82 years) and 49% of them were male. The median LVEF was 47% (IQR, 33–63%) and 33% of the patients had heart failure with reduced ejection fraction. Increased FM and muscle wasting were observed in 58 and 67% of the enrolled patients, respectively. During a 180-day follow-up period, 32 patients (16%) had cardiac events defined as cardiac death or readmission by worsening HF or arrhythmia. Kaplan-Meier survival curves showed that patients with increased FM had a lower cardiac event rate than did patients without increased FM (11.4% vs. 22.6%, p = 0.03). Kaplan-Meier curves of cardiac event rates did not differ between patients with and those without muscle wasting (16.5% vs. 15.4%, p = 0.93). In multivariate Cox regression analyses, increased FM was independently associated with lower cardiac event rates (hazard ratio: 0.45, 95% confidence interval: 0.22–0.93) after adjustment for age, sex, diabetes, muscle wasting, and renal function. Conclusions High percent body fat mass is associated with lower risk of short-term cardiac events in HF patients.

Objective Malnutrition is associated with an increased risk of mortality in heart failure (HF) patients. Here, we examined the hypothesis that assessment of energy intake in addition to nutritional status improves the stratification of mortality risk in elderly HF patients. Methods We retrospectively examined 419 HF patients aged ≥ 65 years (median 78 years, 49% female). Nutritional status was assessed by the Mini Nutritional Assessment Short Form (MNA-SF), and daily energy intake was calculated from intake during 3 consecutive days before discharge. Results During a median 1.52-year period (IQR 0.96–2.94 years), 110 patients (26%) died. Kaplan–Meier survival curves showed that patients with low tertile of daily energy intake had a higher mortality rate than did patients with high or middle tertile of daily energy intake. In multivariate Cox regression analyses, low daily energy intake was independently associated with higher mortality after adjustment for the model including age, sex, BNP, Charlson Comorbidity Index, history of HF hospitalization, and cachexia in addition to MNA-SF. Inclusion of both MNA-SF and energy intake into the adjustment model improved the accuracy of prediction of the mortality after discharge (continuous net reclassification improvement, 0.355, p = 0.003; integrated discrimination improvement, 0.029, p = 0.003). Results of a fully adjusted dose-dependent association analysis showed that risk of all-cause mortality was lowest among HF patients who consumed 31.5 kcal/kg/day of energy. Conclusions Energy intake during hospital stay is an independent predictor of the mortality in elderly HF patients, and its assessment together with established predictors improves the mortality risk stratification. Graphic abstract

The histopathological diagnosis of cardiac sarcoidosis (CS) is challenging because of sampling error in endomyocardial biopsy (EMB) and the determinants of positive EMB are unclear. Reduced left ventricular ejection fraction (LVEF) is a simple parameter of the extent of myocardial damage, and higher serum angiotensin-converting enzyme (ACE) activity would indicate the spread of disease activity in CS patients. Thus, we sought to examine whether these parameters are related to the histopathological diagnosis of CS by EMB.A total of 94 consecutive clinically diagnosed CS patients between August 1986 and March 2019 who were admitted to two academic hospitals were examined. We determined EMB as positive if non-caseating epithelioid granulomas were confirmed in the myocardial tissue. Patients were divided into two groups according to positive (n = 37) and negative (n = 57) EMB. We assessed the relationship between LVEF, serum ACE activity and positive EMB.Multivariable analysis revealed that both LVEF and serum ACE were independently associated with positive EMB (OR 0.83, 95% CI 0.70-0.99; OR 1.39, 95% CI 1.02-1.90, respectively). Moreover, patients with both lower LVEF (37%, median) and higher ACE activity (≥13.5 IU/L, median) had the highest frequency of positive EMB (p = .003). The combination of lower LVEF and higher serum ACE showed better specificity (91.2%) and positive predictive value (73.7%) than either LVEF or serum ACE alone for positive EMB.Lower LVEF and higher serum ACE activity were associated with positive EMB, suggesting that these parameters might be useful for predicting positive EMB in CS patients.

Background Obesity, defined as higher body mass index (BMI), is associated with better prognosis in patients with chronic heart failure (CHF), though the presence of obesity is a risk factor of development of CHF (Obesity paradox). On the other hand, muscle wasting, i.e. reduction in skeletal muscle mass, is frequently observed in CHF, leading to lower exercise capacity and poor cardiovascular outcome. Purpose The aim of this study was to examine whether analysis of body composition improves prediction of short-term readmission rates in patients with CHF. Methods We retrospectively analyzed data for 167 consecutive HF patients who were admitted to our institute for management of HF and received a Dual-energy X-ray absorptiometry (DEXA) scan. Muscle wasting was defined as DEXA-measured appendicular skeletal muscle mass index 25% in male, >30% in female. The primary endpoint was readmission due to cardiac events including worsening heart failure, arrhythmia, and cardiopulmonary arrest during a 180-days follow-up period after discharge. Results The mean age of the patients was 74±13 years and 46% of them were male. The mean BMI was 21.8±3.8 kg/m2. Forty-seven percent of the patients were classified as NYHA functional class III. The most frequent etiology of HF was cardiomyopathy (30%), followed by ischemic heart disease (27%) and valvular heart disease (27%). The prevalence of muscle wasting and that of obesity were 69% and 59%, respectively. Patients with muscle wasting had lower BMI level, higher prevalence of NYHA functional class III and diabetes mellitus compared with those without muscle wasting. On the other hand, patients with obesity had higher prevalence of hypertension and dyslipidemia, higher level of BMI, fasting plasma insulin and triglyceride, and lower level of HDL-cholesterol compared with those without obesity. During the follow-up period, 34 patients (19%) were re-hospitalized due to cardiac events. Kaplan-Meier survival curves showed that patients with obesity had a significantly lower readmission rate during a 180-days follow-up period than did the patients without obesity (14.3% vs. 29.0%, Log-Rank test, p Conclusion Body composition analysis by DEXA enables to find CHF patients with increased fat mass who have lower risk of short-term readmission.

Although the benefit of updated therapeutic regimens, including bortezomib, on the survival of immunoglobulin light chain (AL) amyloidosis patients with heart failure (HF) has been reported, predictors of mortality in the patients treated with the updated therapy remain unclear. We retrospectively enrolled AL amyloidosis patients who had severe HF at the time of diagnosis and received the updated therapy, including bortezomib (n = 19, 61 ± 6 years old, 68% male). Severe HF was defined as the presence of both NYHA functional class III or IV and BNP > 200 pg/ml or NT-pro-BNP > 900 pg/ml. One-year mortality rate during follow-up after commencement of the treatment was 37%. Left ventricular morphological parameters and indexes of left ventricular diastolic function on admission were similar in the non-survivors and survivors. However, non-survivors had higher incidences of atrial fibrillation and ventricular tachycardia, higher serum total bilirubin levels (1.34 ± 0.55 vs. 0.61 ± 0.29 mg/dl), higher right atrial volume index (RAVI 49.7 ± 29.9 vs. 27.3 ± 6.8 ml/m2), lower tricuspid annular peak velocities during systole (RVs’ 8.0 ± 1.8 vs. 11.6 ± 3.7 cm/sec) and late diastole (RVa’ 3.4 ± 0.9 vs. 11.4 ± 5.3 cm/sec), and larger inferior vena cava dimension (22.7 ± 6.4 vs. 16.3 ± 4.9 mm) than those in survivors. Kaplan–Meier curve analyses showed that larger RAVI and lower RVs’ and RVa’, but not left ventricular systolic/diastolic dysfunction, predicted higher mortality during 1-year follow-up. The present results suggest that the presence of right-sided heart abnormality on admission is associated with high 1-year mortality in AL amyloidosis patients with severe HF under the updated therapeutic regimens.

In a polar map display, the LAD originates from an area corresponding to 11 o’clock, and the relationship between localization of the culprit lesion and diagonal arteries can be inferred by the extent of decreased perfusion extending from 12 o’clock towards 1 o’clock. The location of a lesion in the LCX main trunk can be inferred by whether decreased perfusion is observed at any level within 2〜5 o’clock. Infarction volume depends on the anatomical size of the territories of the PD and AV but not on the location of a culprit lesion in the RCA trunk.

A 14-year-old girl with cardiopulmonary arrest was referred to our hospital. She had received an injection of inactivated influenza vaccine 7 days before the referral. Her cardiac rhythm was pulseless wide QRS tachycardia, and mechanical circulatory support was immediately begun. Results of endomyocardial biopsy showed that there was massive infiltration of CD3- and CD68-positive cells and various degrees of cardiomyocyte necrosis in all of 3 endomyocardial specimens, whereas infiltration of eosinophils or giant cells was not observed. A histologic diagnosis of lymphocytic myocarditis was made. Acute myocarditis is a rare but potentially fatal complication of the influenza vaccination.

Which combination of clinical parameters improves the prediction of prognosis in patients with pulmonary arterial hypertension (PAH) remains unclear. We examined whether combined assessment of pulmonary vascular resistance and right ventricular function by echocardiography is useful for classifying risks in PAH. In 41 consecutive patients with PAH (mean age of 48.9 ± 17.3 years, 31 females), a 6-min walk test, pulmonary function test, and echocardiography were performed at baseline and during PAH-specific therapies. The study endpoint was defined as a composite of cardiovascular death and hospitalization for PAH and/or right ventricular failure. During a follow-up period of 9.2 ± 8.7 months, 18 patients reached the endpoint. Multivariate regression analysis showed that the ratio of tricuspid regurgitation pressure gradient to the time-velocity integral of the right ventricular outflow tract (TRPG/TVI) and tricuspid annular plane systolic excursion (TAPSE) during PAH-specific treatment were independent prognostic predictors of the endpoint. Using cutoff values indicated by receiver operating characteristic analysis, the patients were divided into four subsets. Multivariate analyses by Cox's proportional hazards model adjusted for age, sex and body mass index indicated that subset 4 (TRPG/TVI ≥ 3.89 and TAPSE ≤ 18.9 mm) had a significantly higher event risk than did subset 1 (TRPG/TVI 3.89 and TAPSE 18.9 mm): HR = 25.49, 95% CI 4.70-476.97, p 0.0001. Combined assessment of TRPG/TVI and TAPSE during adequate PAH-specific therapies enables classification of risks for death and/or progressive right heart failure in PAH.

Fabry disease is an X-linked lysosomal storage disease characterized by globotriaosyceramide accumulation because of genetic loss/deficiency of α-galactosidase A (α-Gal A) activity. Clinical symptoms of classic Fabry disease, such as acroparethesia and neuropathic pain, typically become apparent in childhood and adolescence. Clinical manifestations in adulthood include cardiac and renal diseases, which are the main causes of death. Life expectancy of untreated Fabry males is ≈50 years. Myocardial involvement in Fabry disease is potentially misdiagnosed as hypertrophic cardiomyopathy unless careful workup of the patient, including pathological and genetic tests, is performed. The presence of chronic kidney disease with proteinuria in patients with ventricular hypertrophy leads to further analyses for diagnosis of Fabry disease. Although deterioration of renal function, leading to end-stage renal disease, is a typical manifestation of classical Fabry disease, a cardiac variant of Fabry disease, often accompanied by mild proteinuria, has also been reported. A 52-year-old man with ventricular hypertrophy and a history of acroparethesia during childhood was referred …

Objective Although risk factors of atrial fibrillation (AF) in the general population have been characterised, their impacts on patients with specific diseases are unclear. Our aim was to determine whether risk factors of AF are different in patients with and those without coronary artery disease (CAD). Methods We enrolled 1871 consecutive patients who underwent coronary angiography for evaluation of symptoms suggestive of CAD in the BOREAS-CAG Registry between August 2014 and January 2015. After exclusion of patients with valvular heart disease or a history of PCI/cardiac surgery, 1150 patients contributed to multivariate logistic regression analysis to identify risk factors of AF. We also retrieved data for 361 consecutive patients with CAD admitted to Sapporo Medical University Hospital between April 2013 and July 2014 and analysed data for 166 patients using the same inclusion and exclusion criteria as those in the BOREAS-CAG Registry. Results Unexpectedly, CAD was independently associated with the absence of AF. The patients were then divided into a non-CAD group (n=576) and a CAD group (n=574) for further analysis. The brain natriuretic peptide level showed a strong association with AF regardless of the presence or absence of CAD. In the non-CAD group, lack of statin use was independently associated with AF, whereas high serum uric acid level was an independent explanatory variable of AF in the CAD group. The association of AF with uric acid was confirmed in a separate group of patients (n=166) enrolled in the CAD cohort in Sapporo Medical University Hospital. Conclusions Major risk factors of AF are different in patients with CAD and those without CAD. Patients with CAD are more likely to develop AF when the serum uric acid level is high, whereas no statin administration predicts development of AF in patients without CAD.

Takayasu's arteritis (TA) is an inflammatory disease of unknown etiology involving the aorta and its branches, and also causes aortic regurgitation (AR). One of the most serious but rare complications after aortic valve replacement (AVR) in TA is aneurysm formation of the sinus of Valsalva. A 64-year-old woman had undergone AVR with a prosthetic valve for AR due to TA 4 years earlier and had received an implanted permanent pacemaker for complete atrioventricular block (AVB) 2 years later. Aortography 4 years postoperatively demonstrated aneurysm formation (47 mm in diameter) at the sinus of Valsalva although preoperative aortography showed severe AR without dilatation of the sinus of Valsalva. We recommended reoperation for the aneurysm but the patient refused. The perioperative histopathological examination revealed extensive destruction of the medial elastic fibers. Both the fragility of the sinus of Valsalva and the residual inflammation could have caused the patient's aneurysm formation. Moreover, extension of TA into the ventricular septum or mechanical compression of the aneurysm against the conduction system might have caused her progressive AVB. Close and lifelong follow-up for patients with TA regarding development of aneurysm after surgical treatment is indispensable when fragility of the aortic root had been confirmed.

The etiology of peripartum cardiomyopathy (PPCM) remains unestablished, but the involvement of abnormal autoimmunity has been suggested. We report a case of PPCM that was triggered by postpartum thyroiditis. Despite the presence of myocardial damage indicated by cardiac magnetic resonance imaging, the patient's cardiac function completely recovered with the addition of bromocriptine to standard therapies. We discuss the role of thyroid hormones in the development of PPCM through aggravation of a prolactin-dependent antiangiogenic effect, and we argue that more attention should be paid to postpartum thyroiditis as a novel risk factor for PPCM.

BACKGROUND Basal thinning of the interventricular septum (IVS) is an important diagnostic feature of cardiac sarcoidosis (CS), but its long-term prognostic significance remains unclear. METHODS AND RESULTS We examined 74 consecutive patients who were diagnosed with CS. Basal IVS thickness at a point located 10 mm from the aortic annulus was measured. IVS thickness at the left ventricular minor axis level (IVS) was also measured according to the recommended procedure of the American Society of Echocardiography. Patients were divided into 2 groups based on the presence or absence of basal IVS thinning, which was defined as basal IVS ≤4 mm and/or basal IVS/IVS ratio ≤0.6. Basal IVS thinning was observed in 21 patients and was associated with greater long-term adverse events during follow-up (5.1±2.5 years), although the baseline characteristics were comparable between groups (overall, P

BACKGROUND Cardiac involvement is the worst prognostic determinant in patients with sarcoidosis, but the long-term prognostic significance of corticosteroid therapy for cardiac sarcoidosis (CS) remains unclear. METHODS AND RESULTS We examined 83 consecutive patients diagnosed with CS. Patients were divided into 2 groups based on the presence or absence of corticosteroid therapy at diagnosis. Patients with corticosteroid therapy had lower age and higher rate of positive findings in the myocardium on gallium scintigraphy (Ga) at diagnosis than those without. LVEF, biomarkers, and use of cardiovascular medication were similar between the 2 groups. During the follow-up (7.6±4.4 years), corticosteroid therapy was associated with fewer long-term adverse events (overall, P=0.005; cardiac death, P=0.92; symptomatic arrhythmias, P=0.89; heart failure admission, P