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Background: Fulminant myocarditis presentation (FMP) is a rare and severe presentation of myocarditis. The natural history of FMP and its clinical features associated with poor outcomes are incompletely understood because there is a lack of generalizable evidence. Methods: This multicenter retrospective cohort study included patients hospitalized with histologically proven myocarditis who underwent catecholamine or mechanical support from 235 cardiovascular training hospitals across Japan between April 2012 and March 2017. Clinical features and the prognostic predictors of death or heart transplantation within 90 days on the basis of clinical and pathologic findings were determined using the Kaplan-Meier method, log-rank test, and Cox regression analysis. Results: This study included 344 patients with histologically proven FMP (median age, 54 years; 40% female). The median follow-up was 600 days (interquartile range, 36 to 1599 days) and the cumulative risk of death or heart transplantation at 90 days was 29% (n=98). Results from multivariable Cox regression analysis showed that older age, nonsinus rhythm, low left ventricular wall motion ( Conclusions: The results from analyses of data from this multicenter registry demonstrated that patients with FMP are at a higher risk of death or heart transplantation in real-world settings. These observations inform which clinical and pathologic findings may be useful for prognostication in FMP. Registration: URL: https://www.umin.ac.jp/ctr ; Unique identifier: UMIN000039763.

Inappropriate and multiple medications affect the prognosis of patients with acute decompensated heart failure (ADHF). However, in ADHF patients with decreased renal function, there have been no reports on prognostic factors, including medication data, or models for predicting cardiac events.To develop a model including medication data to predict cardiac events in ADHF patients with decreased renal function.This retrospective cohort study included 443 first-time admitted ADHF patients with decreased renal function (estimated glomerular filtration rate 60 mL/min/1.73 mThe incidence of cardiac events within one year after discharge was 20.1%. By multivariable analysis, we observed that atrial fibrillation, weight loss 5%, brain natriuretic peptide ≥ 200 pg/mL, polypharmacy, and beta-blockers use below target dosage were significantly associated with an increased risk of cardiac events. The developed model, the cardiac events rate in the high-risk group was significantly higher than in the low-risk group (41.0 vs. 9.2%, p 0.001).The developed model for predicting cardiac events will be useful in decision-making to support appropriate early management of ADHF patients with decreased renal function.

Background Left atrial (LA) volume and left ventricular longitudinal strain (LVLS) have significant prognostic values for major cardiovascular events (MACEs). Prognostic values of LA reservoir functional indices measured by 3-dimensional (3D) speckle-tracking echocardiography (STE) were evaluated.Methods and Results:A total of 264 patients, who underwent 2-dimensional (2D) echocardiography and 3DSTE for various underlying heart diseases, were followed up to record MACE. After a mean follow up of 547±435 days, 30 patients developed MACE: 7 cardiac deaths, 6 strokes, 1 non-fatal myocardial infarction, and 22 admissions for heart failure (5 of these had cardiac death after discharge, whereas 1 sustained stroke after discharge). Receiver operating characteristic curve analysis was performed to determine the optimal cut-off levels of 4 LA functional indices: LA emptying fraction (LAEmpF), LA longitudinal strain (LALS), LA circumferential strain (LACS), and LA area change ratio (LAAC), using 3DSTE. Among these factors, 2DLVLS, 3DLAEmpF, and 3DLALS demonstrated a higher hazard ratio (>5.0) than other variables. The 3DLAEmpF and 3DLALS had a higher average treatment effect (ATE) and ATE on the treated (ATT), respectively, than the other indices after propensity score matching. Addition of 3DLAEmpF to the base model using clinical variables and LV ejection fraction or 2DLVLS demonstrated higher prognostic power. Conclusions LAEmpF calculated using 3DSTE possessed additive prognostic values for the prediction of MACE.

Aims Guidelines for the diagnosis and treatment of acute and chronic heart failure (HF) provided by the European Society of Cardiology state that echocardiography is recommended for the assessment of the myocardial structure and function of subjects with suspected HF including HF with reduced (HFrEF), mid‐range (HFmrEF), and preserved ejection fraction (HFpEF) as class I of recommendation and level C of evidence. However, the impact of timing of echocardiography on survival for hospitalized HF patients or the prevalence of echocardiography during their stay has not yet been fully investigated. Therefore, we designed and conducted a prospective multicentre study, Optimal Timing of Echocardiography for Heart Failure Inpatients in Japanese Institutions (OPTIMAL) study, to investigate and evaluate the prevalence of echocardiography during the in‐hospital stay of HF patients, and the impact of timing of echocardiography on their survival. Methods and results OPTIMAL was based on a nationwide, prospective, multicentre registry at 10 institutions in Japan endorsed by the Japanese Society of Echocardiography. A total of 601 patients hospitalized with HF were enrolled between August 2016 and July 2018 at the participating centres. Their mean age was 73.9 ± 13.0 years, left ventricular ejection fraction was 37.0% (26.0–50.0), and 256 patients (42.6%) were female. Admission echocardiography (admission echo) was categorized as either standard or point‐of‐care echocardiography performed within 3 days of admission, as was pre‐discharge echocardiography (pre‐discharge echo) within 3 days of discharge. The primary endpoint was defined as cardiovascular death over a median follow‐up period of 18.9 months (9.3–26.5 months). Admission echo was performed for 476 patients (79.2%) and pre‐discharge echo for 216 patients (35.9%). The primary endpoint of cardiovascular death occurred in 65 patients (10.8%). Kaplan–Meier curve findings indicated that survival of patients with pre‐discharge echo was significantly better than that of patients without it (log‐rank P

The Trifecta valve has been reported to have valve dysfunction months to years after operation. However there were no reports of intraoperative valve dysfunction. A 73-year-old man with aortic stenosis underwent aortic valve replacement using a 21-mm Trifecta valve with Glide Technology (Trifecta GT; Abbot). Hemodynamics were collapsed after aortic declamp because of severe regurgitation, and we replaced it with another biologic valve. We noticed that the removed valve's mount post was open outwardly more than usual. Despite the progress of the biologic valve, dysfunction like this case can occur during operation, and it is important to reoperate immediately even if there is no obvious tear or deformity.

BACKGROUND The prognostic value of indices for left atrial volumes (LAV) and reservoir function measured by 3D speckle-tracking analysis (3DSTA) has not been determined. Methods and Results: LA maximal and minimal volume indices (LAVImax, LAVImin), and LA emptying fraction (LAEmpF) were measured via 2D echocardiography (2DE) and 3DSTA in 514 patients (62% male, mean age: 66±15 years) with various cardiovascular diseases. Two cutoff values using normal±2SD (cutoff criterion 1) and receiver-operating characteristic analysis (cutoff criterion 2) were evaluated. During a mean follow-up of 720±383 days, MACE (cardiac death, nonfatal myocardial infarction, stroke and admission for heart failure) occurred in 98 patients. Kaplan-Meier survival analysis showed both cutoff criteria measured by 2DE and 3DSTA had significant predictive power for MACE (P

Direct oral anticoagulants (DOACs) are safe and efficacious when compared to warfarin for patients with venous thromboembolism (VTE). However, bleeding is a major side effect of anticoagulant therapy in VTE patients. Discontinuation of the DOACs associated to adverse events such as bleeding. The HAS-BLED score predicts warfarin-associated hemorrhage. However, little is known about risk factors for DOAC-associated minor bleeding in VTE patients. We aimed to identify risk factors for minor bleeding in VTE patients that were treated with edoxaban, rivaroxaban, or apixaban. We retrospectively evaluated the data of 212 VTE patients who received treatment with a DOAC. The study endpoint was defined as the occurrence of minor bleeding. Logistic regression analysis was used to determine risk factors that were significantly associated with minor bleeding. A total of 36 (17.0%) patients experienced minor bleeding, with rates of 15.7%, 0%, and 21.3% for edoxaban, rivaroxaban, and apixaban, respectively. In the multivariate analysis, bleeding history or predisposition [odds ratio (OR) 6.083, 95% confidence interval (CI) 2.131-17.364, p=0.001] and cancer (OR 6.397, 95% CI 2.858-14.317, p

Background Left atrial (LA) dilation is associated with morbidity of atrial fibrillation (AF). However, little is known about pulmonary vein (PV) dilation. Purpose We investigated the PV volume in the patients with AF. Methods and results We performed 3dimensional computed tomography (3DCT) in 155 patients and divided them into three groups: 19 patients without AF (non‐AF group, mean age 66 ± 12 years), 50 with paroxysmal AF (PAF group, mean age 67 ± 8 years) and 24 with persistent AF (PeAF group, mean age 64 ± 10 years). The absence of AF was diagnosed in patients with a cardiac implantable electronic device for at least 1 year (mean: 59 ± 37 months). We determined the PV volume as the total volume from the orifice to the first branch of each PV. According to the echocardiographic data, the LA dimension (LAD) and LA volume index (LAVI) were largest in the PeAF group followed by the PAF and non‐AF group. According to the morphometric data obtained on 3D‐CT, the PV volume was similar in PeAF and PAF groups but significantly smaller in the non‐AF group (median value: 24 vs 21 vs 14 mL, respectively). According to the receiver operating characteristic curve analysis, the area under the curve for the PV volume in the presence of AF was 0.80, and the optimum cut‐off value was 17 mL (sensitivity 74%, specificity 80%). Conclusion The PV volume might be useful for predicting the presence of AF before increases in the LAD and LAVI on echocardiography.

Anderson-Fabry disease is a rare X-linked lysosomal storage disease caused by α-galactosidase A (α-GalA) gene variants and characterized by a large genotypic and phenotypic spectrum. Enzyme replacement therapy (ERT) using recombinant α-GalA has been approved for > 10 years as a specific therapy for the disease. However, the long-term clinical efficacy for cardiac manifestations has been equivocal because it depends on several factors such as genotype, sex, age, and disease severity at the initiation of ERT. We report the differences in the clinical effects of ERT continued for > 10 years in three patients with the same genotype. Left ventricular hypertrophy and myocardial dysfunction progressed in the heterozygote proband even under ERT, although disease progression was prevented in two sons of Case 1.

Purpose Plasma globotriaosylsphingosine (lyso-Gb3) is a promising secondary screening biomarker for Fabry disease. Here, we examined its applicability as a primary screening biomarker for classic and late-onset Fabry disease in males and females. Methods Between 1 July 2014 and 31 December 2015, we screened 2,360 patients (1,324 males) referred from 169 Japanese specialty clinics (cardiology, nephrology, neurology, and pediatrics), based on clinical symptoms suggestive of Fabry disease. We used the plasma lyso-Gb3 concentration, α-galactosidase A (α-Gal A) activity, and analysis of the α-Gal A gene (GLA) for primary and secondary screens, respectively. Results Of 8 males with elevated lyso-Gb3 levels (≥2.0 ng ml–1) and low α-Gal A activity (≤4.0 nmol h–1 ml–1), 7 presented a GLA mutation (2 classic and 5 late-onset). Of 15 females with elevated lyso-Gb3, 7 displayed low α-Gal A activity (5 with GLA mutations; 4 classic and 1 late-onset) and 8 exhibited normal α-Gal A activity (1 with a classic GLA mutation and 3 with genetic variants of uncertain significance). Conclusion Plasma lyso-Gb3 is a potential primary screening biomarker for classic and late-onset Fabry disease probands.

Coronavirus Disease-19 (COVID-19)-associated acute pericarditis is a rare complication. Several cases have been reported, but those reports have not discussed any imaging findings. Here, we report a case of a 76-year-old female diagnosed with COVID-19-associated pericarditis without pneumonia, and present image findings of the patient’s contrast-enhanced CT.

Objectives: In the field of exercise physiology, there has been great interest in exploring circulating microRNAs (miRs) as potential biomarkers. However, it remains to be determined whether circulating miRs reflect cardiorespiratory fitness. The aim of this study was to investigate the association between circulating levels of specific miRs and cardiorespiratory fitness evaluated by cardiopulmonary exercise testing (CPET) after acute myocardial infarction (MI). Methods: Twenty patients who had had an acute MI were included. All patients underwent CPET in the convalescent phase. Quantitative real-time polymerase chain reaction analyses for miR-181 members (a/b/c) and miR-484 were performed to determine the expression levels in the peripheral blood of the included patients and healthy control subjects (n=5). Results: Post-MI patients showed impaired exercise tolerance and ventilatory efficiency in CPET analysis. Compared with controls, circulating levels of miR-181a and 181c were gradually and significantly elevated through the 1st to 7th days after acute MI, whereas miR-181b and miR-484 were not. Circulating miR levels did not correlate with clinical or echocardiographic parameters. However, circulating levels of miR-181c and miR-484 on the 7th day showed significant positive correlations with the anaerobic threshold and peak oxygen consumption from CPET analysis. Moreover, miR-181c levels were inversely associated with the ventilatory inefficiency index. Patients with high exercise capacity after MI showed significantly higher expressions of circulating miR-181c and miR-484 than those with low exercise capacity. Conclusions: The results of this pilot study suggest that circulating levels of miR-181c and miR-484 after acute MI may be predictive biomarkers of post-MI cardiorespiratory fitness.

Background Hypertrophic cardiomyopathy (HCM) is a most prevalent primary myocardial disorder with heterogeneous clinical features. However, there have been few studies on clinical features of HCM as a prospective cohort. In 2015, we established a large-scale registration survey of patients with HCM throughout Japan, named J-HCM registry study. Purpose The aim of this study was to clarify the clinical features of Japanese patients with HCM. Methods J-HCM registry study is a prospective, multicenter investigation, consisting of 24 hospitals. This time, we present the baseline clinical characteristics in this survey. Results Total 1484 patients were registered. The ages at registration and at diagnosis were 65±15 and 56±17 years, respectively, and 806 patients (54%) were men. Majority of the patients (95%) was NYHA class I or II. With regard to subtypes of HCM, there were 526 patients (36%) in the HCM with left ventricular (LV) outflow tract obstruction, 126 patients (8%) in the mid-ventricular obstruction, 57 patients (4%) in the end-stage phase characterized by LV ejection fraction Conclusions In this multicenter registration survey of patients with HCM, the baseline clinical characteristics were almost similar to several retrospective large-scale cohorts in Western countries except older age and less symptomatic state. This study will provide important knowledge regarding management of HCM. Figure 1 Funding Acknowledgement Type of funding source: None

Background Early reported beneficial effects of cardiac rehabilitation (CR) have recently been disputed. The present study aimed to investigate the clinical impact of CR on the mid-term outcomes of patients following ST-segment elevation myocardial infarction (STEMI) treated with currently available management. Methods This study reviewed 145 consecutive patients who underwent primary coronary intervention and were discharged without any disability after STEMI during 2013–2015. Results Among the patients, 66 (45.5%) completed an outpatient CR program (CR group) and 79 were their non-CR counterparts or patients who dropped out of the program (N-D group). There were no between-group differences in patient demographics and clinical profiles, including door-to-balloon times and prescriptions. A total of 27 patients developed major adverse cardiac and cerebrovascular events (MACCE) during follow-up. The MACCE-free survival rates were 88% and 76% in the CR and N-D groups, respectively (log-rank, p=0.04). Cox proportional analysis demonstrated that inclusion in the N-D group was a significant predictor of MACCEs (HR, 2.36; 95% CI, 1.07–5.74; p=0.03). In the CR group, peak oxygen consumption and ventilatory efficiency determined by cardiopulmonary exercise testing significantly improved after the program (p Conclusions The impact of CR on the mid-term prognosis of patients with STEMI, even in the current myocardial infarction management era, was beneficial.

Background: Acute decompensated heart failure (ADHF) is the most common cause of readmissions in the hospital. ADHF patients are associated with polypharmacy. It is a common problem among elderly patients due to frequently occurring multiple morbidities and is associated with the use of potentially inappropriate medications (PIMs). The aim of this study was to examine the association between PIMs and all-cause mortality in elderly ADHF patients. Methods: This retrospective study included ADHF patients who were admitted to the Showa University Fujigaoka Hospital between January 2015 and August 2016. We investigated the proportion of patients taking at least one PIM at admission and the characteristics of patients at admission. PIMs were defined based on the Screening Tool of Older People’s potentially inappropriate Prescriptions (STOPP). Multiple Cox regression analysis was performed to examine the association between PIM use and all-cause mortality. Results: A total of 193 elderly patients (median age 81 years, interquartile range (IQR) 65 - 99 years) were included in the study. All-cause death occurred in 30 patients. The median number of medications at admission was 7 (IQR 0 - 18). The number of medications (greater than or equal to six) at admission was associated with mortality. Multivariate Cox regression analysis revealed that systolic blood pressure (SBP) < 100 mm Hg at admission, chronic obstructive pulmonary disease (COPD), and use of non-steroidal anti-inflammatory drugs (NSAIDs) at admission were independent predictors for all-cause mortality. Conclusions: The medical staff should attempt to stop unnecessary medications that are prone to be inappropriate prescribing. In particular, prescription of NSAIDs should be carefully assessed and monitored. Cardiol Res. 2020;11(4):239-246 doi: https://doi.org/10.14740/cr1078

Pulmonary vein stenosis and occlusion are some of the major complications after radiofrequency ablation of atrial fibrillation. The prevalence of pulmonary vein stenosis and pulmonary vein occlusion decreased remarkably after the advent of area circumferential ablation and antral isolation; however

Background Left atrial functional indices measured by two dimensional echocardiography (2DE) have prognostic significance for major cardiovascular events (MACE). Retrospective analysis using propensity score matching to assess the prognostic values of left atrial reservoir function measured by three dimensional speckle tracking analysis (3DSTA) was performed. Methods Two hundred sixty-four patients (Male 60%, 65±15yo) who underwent both clinically indicated 2DE and 3DSTA for various underlying heart diseases from January 4, 2013 to February 28, 2016 were followed to record MACE. Patients with significant mitral valve disease, history of pulmonary vein isolation and persistent atrial fibrillation were excluded. Maximum and minimum left atrial volume (LAVImax and LAVImin), left atrial emptying fraction (LAEmpF), peak global LA area ratio (LAAC), circumferential strain (LACS) and longitudinal strain (LALS) were measured using 3DSTA. Standard indices including peak global LVLS (2DLVLS) and left ventricular ejection fraction (LVEF) and LA volume index were also assessed by 2DE. Cutoff line for LA reservoir functional indices and 2DLVLS was determined using ROC analysis. Average treatment effect for the treated (ATT), Average treatment effect (ATE) for each index were calculated after propensity score matching for clinical indices (age, sex, coronary artery disease, hypertension, diabetes, eGFR Results During a mean follow-up of 547±435 days, MACE developed in 30 patients (7 cardiac death 6 stroke, 1 nonfatal MI, 22 admission for heart failure). Age, coronary artery disease (CAD), diabetes mellitus (DM), chronic kidney disease (CKD: eGFR< 45ml/min/1.73m2), LVEF, 2DLVLS, LAVImax and LAVImin by 2DE and all LA indices by 3DSTA had significant prognostic value by univariate analysis. LAEmpF and LALS by 3DSTA had higher ATT, ATE and Log rank χ2 than other LA indices and 2DLVLS. The model added LALS or LAEmpF by 3DSTA had higher prognostic value (LALS propensity score matching Conclusion LALS and LAEmpF by 3DSTA showed additive prognostic value for MACE.

BACKGROUND The ideal surgical technique for ischemic mitral regurgitation (MR) is controversial. We introduced an extended posterior mitral leaflet (PML) augmentation technique for functional MR with severe tethering, which detached the PML from the annulus almost completely and augmented it with a large 3×6-cm oval pericardial patch. Methods and Results: A total of 17 mitral repairs using the new technique were performed for ischemic MR with no 30-day mortality and 2 hospital deaths. The NYHA class was III in 47% and IV in 13%. The EuroSCORE II was 9.7±4.9. The ring size was 32±1.4 mm. Concomitant coronary bypass was performed in 67% and left ventricular repair in 28%. The mechanism of leaflet closure was evaluated using transthoracic echocardiography in 15 survivors. MR decreased to none or trivial with a significant increase in coaptation length (Pre: 4.6±0.8 mm vs. Post: 9.8±2.5 mm; P

The purposes of this study were to investigate whether heart failure (HF) with reduced ejection fraction (HFrEF) and heart failure with preserved ejection fraction (HFpEF) share echocardiographic predictors and elucidate E/(e' × s') that predicts major adverse cardiovascular events (MACE) independent of other echocardiographic parameters in each HF group. We assessed tissue Doppler-derived parameters [E/e', E/(e' × s')] and left ventricular and right ventricular longitudinal strains in HFrEF (n = 340) and HFpEF (n = 102). Left ventricular and right ventricular longitudinal strains were significantly higher and E/(e' × s') was lower in patients with HFpEF compared with those with HFrEF, whereas E/e' was similar between the groups. During a median follow-up of 342 days, MACE developed in 95 patients with HFrEF and 29 with HFpEF. The univariable analysis revealed similar echocardiographic predictors between the 2 groups, including E/e', E/(e' × s') and pulmonary artery systolic pressure. No 2-dimensional speckle tracking-derived parameter remained significant in multivariable models in each HF group. E/(e' × s') was an only independent predictor with an incremental prognostic value over the Meta-analysis Global Group in Chronic Heart Failure score and was superior to the E/e' ratio in both HFrEF and HFpEF. In conclusion, despite differences in echocardiography-based cardiac function parameters between HFrEF and HFpEF, these HF phenotypes shared the same echocardiographic predictors of future MACE. E/(e' × s') was an only independent predictor for future cardiac events in both HF populations.

Aim: The vascular endothelium plays a key role in the pathophysiology of atherosclerosis. Flow-mediated dilation (FMD) is a novel way of assessing endothelial function. Cilostazol is a unique antiplatelet drug that also has the potential to improve endothelial function. The objective of this present study was to investigate the effects of cilosatzol on endothelial function as assessed by FMD. Methods: Fifty-one patients with coronary artery disease (CAD) were assigned to one of two groups: the Cilostazol(+) group (with cilostazol) and Cilostazol(−) group (without cilostazol). In addition to conventional dual antiplatelet therapy with aspirin and clopidogrel/ticlopidine, the Cilostazol(+) group (n = 27) was also given cilostazol (100 mg/day). The Cilostazol(−) group (n = 24) did not receive cilostazol. FMD was assessed at enrollment and after 6–9 months. Results: The FMD of both the Cilostazol(+) and Cilostazol(−) groups remained similar at 5.2 (interquartile range: 3.8–8.5) to 5.4 (interquartile range: 4.2–6.7) (P = 0.29) and 5.0 (interquartile range: 3.6–6.4) to 4.9 (interquartile range: 4.0–7.0) (P = 0.38), respectively. However, the diameters of the baseline and maximal brachial arteries tended to increase in the Cilostazol(−) group (baseline: 4.2 ± 0.7 to 4.4 ± 0.7, P = 0.18; maximal: 4.5 ± 0.7 to 4.6 ± 0.7 P = 0.22), whereas that of the Cilostazol(−) group tended to decrease (baseline: 4.1 ± 0.6 to 3.9 ± 0.5, P = 0.10; maximal: 4.3 ± 0.7 to 4.1 ± 0.5, P = 0.05). The rates of change in the baseline diameter (Cilostazol(+): 3.7 ± 9.8% vs. Cilostazol(−): −3.8 ± 12.2%, P = 0.03) and maximal diameter (Cilostazol(+): +3.1 ± 8.9% vs. Cilostazol(−): −4.4 ± 12.0%, P = 0.02) were significantly different. Conclusion: Although cilostazol didn't affect the FMD, there was a significant difference in the rates of change in baseline and maximal brachial artery diameter. This may have a beneficial effect in patients with cardiovascular disease.

Anderson-Fabry disease is a rare X-linked lysosomal storage disease caused by α-galactosidase A (α-GalA) gene variants and characterized by a large genotypic and phenotypic spectrum. Enzyme replacement therapy (ERT) using recombinant α-GalA has been approved for10 years as a specific therapy for the disease. However, the long-term clinical efficacy for cardiac manifestations has been equivocal because it depends on several factors such as genotype, sex, age, and disease severity at the initiation of ERT. We report the differences in the clinical effects of ERT continued for10 years in three patients with the same genotype. Left ventricular hypertrophy and myocardial dysfunction progressed in the heterozygote proband even under ERT, although disease progression was prevented in two sons of Case 1.

A 52-year-old woman with intermittent complete atrioventricular (AV) block detected on exercise was admitted to the hospital. Echocardiography revealed lesions on the right ventricular side of the interventricular septum and free wall of the basal inferolateral area. Gadolinium-enhanced cardiovascular magnetic resonance (CMR) imaging revealed the mass and wall thickening at the same locations with late gadolinium enhancement (LGE). Focal uptake at the septal lesion was detected using 67Ga scintigraphy. Focal on diffuse intense uptake in the lesions was observed on Fluorine-18 fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) imaging. Whole-body CT and laboratory evaluations uncovered no signs of malignant tumors in other organs. Ophthalmologic evaluation revealed ophthalmologic sarcoidosis. Although the result of endomyocardial biopsy was negative, the presence of cardiac sarcoidosis was strongly suggested on the basis of the new Japanese guidelines published in 2017. AV conduction disturbance and tracer accumulation on 67Ga scintigraphy completely disappeared after 2 weeks of steroid therapy. The size of mass, inferolateral wall thickness in echocardiography and CMR, and standardized uptake value (SUV) of the masses on 18F-FDG PET also decreased over time.

Plasma globotriaosylsphingosine (lyso-Gb3) is a promising secondary screening biomarker for Fabry disease. Here, we examined its applicability as a primary screening biomarker for classic and late-onset Fabry disease in males and females.Between 1 July 2014 and 31 December 2015, we screened 2,359 patients (1,324 males) referred from 168 Japanese specialty clinics (cardiology, nephrology, neurology, and pediatrics), based on clinical symptoms suggestive of Fabry disease. We used the plasma lyso-Gb3 concentration, α-galactosidase A (α-Gal A) activity, and analysis of the α-Gal A gene (GLA) for primary and secondary screens, respectively.Of 8 males with elevated lyso-Gb3 levels (≥2.0 ng mlPlasma lyso-Gb3 is a potential primary screening biomarker for classic and late-onset Fabry disease probands.

Multiple coronary fistulae that drain into the left ventricle are rarely associated with apical hypertrophic cardiomyopathy.1 On the other hand, associations of left ventricular noncompaction (LVNC) and multiple coronary fistulae or hypertrophic cardiomyopathy have previously been reported in several cases.2,3 We report here a unique case of apical hypertrophy of the left ventricle, multiple coronary–left ventricular fistulae, and a morphological structure of the left ventricular myocardium mimicking LVNC. A 59-year-old man was admitted to our hospital for fever and rapidly progressing dyspnea that had persisted for 10 days. He had been diagnosed with apical hypertrophic cardiomyopathy in his 30s, although he had no regular medical checkups and was asymptomatic. On admission, the patient had mild wheezes audible in the midportion of the right lung and a diastolic murmur heard at the Erb and the cardiac apical area. Chest x-ray and computed tomography revealed cardiac enlargement with increased pulmonary vasculature, granular shadows in the lower areas of both lungs, and increased density of the right middle lung area. These findings suggested failure of the left side of the heart with pneumonia. A 12-lead electrocardiogram (ECG) …

Introduction: The aim of this study was to evaluate the changes in cardiac performance and myocardial blood perfusion by single photon emission computed tomography (SPECT) in patients upgrading to cardiac resynchronization therapy (CRT) from right ventricular apical pacing (RVAP). Methods: Eleven patients (age, 70±7.7 years; pacing career, 95±48.8 months) with chronic RVAP were studied. Their baseline characteristics included New York Heart Association (NYHA) class ≥2, with left ventricular end-diastolic volume (LVEDV) >55 mm or left ventricular ejection fraction (LVEF)

Introduction: Intra-cardiac echocardiography (ICE) which has some benefits, can be used to obtain detailed anatomy of the heart chambers or large vessels, and the catheter positions, and it has been considered useful for improving the outcome of the ablation. In the present study, we performed pulmonary vein isolation (PVI) under real time monitoring of ICE imaging utilizing an ICE catheter placed at the junction of the left atrium (LA) and PVs (LA-PV junction). Methods: PVI for atrial fibrillation (AF) was performed in 30 cases with drug-resistant AF (mean age: 66-years-old; including 22 males). An ICE catheter utilizing a 9 MHz frequency was inserted into the LA via the atrial septum, and placed at the LA-PV junction. Circumferential ablation was performed in the LA outside of the PV ostium, encircling both the superior and inferior ostia together under ICE imaging. Results: The anatomy of the LA to the PVs and catheter sites were clearly identified by the ICE during the procedure, which enabled a precise and safe catheter manipulation with minimal fluoroscopy. Further, the wall thickness of the PV and LA, and position of the esophagus could be obtained by ICE, facilitating care in adjusting the power and/or duration of the current delivery. Conclusion: ICE imaging of the LA-PV junction permitted real time monitoring of the target sites for PVI during the ablation procedure, and was considered a useful technique for performing PVI.

Background: Signal-averaged electrocardiograms (SAE) have mainly been studied in high risk patients with ischemic heart disease. A systemic study of the normal values and the comparative results using analytical methods has not been reported. Methods: We recorded SAE results in 200 apparently healthy subjects (100 males and 100 females, average age 28.9 years). The obtained signals were analyzed by for the time-domain (TD), frequencydomain (fast Fourier transform [FFT]), and spectral turbulence (ST) analyses. The normal values for these parameters, including differences according to gender, were evaluated. The correlation between body characteristics and individual measurements was also evaluated for each analytical method. Results were compared using previously reported criteria and gender-specific criteria. Results: The normal values for TD and FFT analyses, but not ST analysis, showed differences related to gender. A significant correlation was observed between the body characteristics noted in the TD and the FFT analyses, but this correlation was not seen for all parameters in the ST analysis. According to the conventional criteria, the rate of positive diagnosis was 9% for TD analysis, 4.5% for FFT analysis, and 2% for ST analysis. When gender-specific criteria were applied, it was 1% for TD analysis, 3.5% for FFT analysis, and 0.5% for ST analysis. However, the positive results for subjects that were found using TD analysis were discordant with the results found using FFT and ST analyses in those subjects. Conclusions: The normal values for TD and FFT analyses showed gender-related differences that were not seen using ST analysis. Gender-specific criteria are recommended when using TD analysis because it decreased positive results for healthy subjects. However, as discordant results were obtained among the various analyses, the selection of the method for analyzing the SAE and interpretation of the results should be performed with caution.

The possible contribution of localized conduction delay and abnormal action potentials to ventricular fibrillation (VF) was studied by applying an anisotropic cardiac computer model to clinical cases of the Brugada-type electrocardiogram (ECG), which shows right bundle branch block (RBBB), a normal QT interval, ST-segment elevation, and late r′ in leads V 1 and V 2 . The anisotropic heart model was composed of 50,000 discrete units with a spatial resolution of 1.5 mm and was mounted in a human torso model. The longitudinal/transverse conduction velocity ratio was 3:1. For the normal ECG, a conduction velocity of 0.75 m/s was required. In the abnormal area of the right anterior epicardial wall, the conduction velocity was set at 0.2 m/s, with decreasing action potential amplitude and 10% prolonged action potential duration. The ECG features of ST-segment elevation and Brugada-type right bundle branch block pattern were simulated. The action potential duration was able to change dynamically with coupling interval of stimulation, with a ratio of 9% for normal ventricular muscle and 50% for Purkinje fibers. Five successive stimuli were applied to the left lateral epicardium 300 ms after the first sinus excitation, and sustained VF was induced with the transmural conduction delay at the right anterior ventricle as a block increasing the vulnerability. At the initiation of VF, reentry circuits were shown around the border zone of the right epicardium and were very heterogeneous around the conduction delayed area and septal area. In an area with the characteristics of nontransmural conduction delay, sustained VF was prevented, and the pattern of transient right bundle branch block appeared on the simulated ECG and body surface potential maps. The late r′ wave was calculated in the precordial leads and right anterior site on the body surface potential maps. These results suggest that increased multipolarity in the border zone between the Purkinje fibers and delayed conduction area in the right ventricle might play an important role as a functional block for the persistence of VF.

加算平均心電図の―解析法であるspectro-temporal mapping (STM法) の本邦における正常人の波形パターンおよび正常値を検討する目的で健常成人200例 (男性100例, 女性100例) を対象に加算平均心電図を記録し, STM法にて解析した.STM解析はHaberlらの方法に準じてArrhythmia Researoh Technology (ART) 社のソフトウェアFFT-plus version 4.0を用いて行った.得られた3次元スペクトル波形および従来の判定基準であるNF (factor of normality) の値を検討した.正常例においてもX誘導18%, Y誘導27%, Z誘導20%, composite誘導38%に5dB以上の高周波成分 (50～200Hz) が認められた.その主要因はQRS終末部の傾きの鋭い部位を解析区間に含むことにより出現する高周波成分であった.この成分の出現率は女性に比し男性で高く (p

加算平均心電図における周波数解析法 (FFT) の正常値を検討する目的で, 健常成人200名 (男女各100名, 平均年齢30歳および27歳) を対象に, 加算平均心電図を記録後FFTを施行した.FFTは120ms幅のBlackman-Harris windowを用い, 開始点はQRS終点より20ms内側とし, arearatio (AR=20-50Hz/10-50Hz×103) を算出した.X, Y, Zの各誘導およびその平均より求めたARは正規分布を示さず, 対数変換により正規化された.ARはいずれの誘導においても, 男性より女性で有意に大であった.身長, 体重, 体表面積, 年齢は男性群が有意に大であった.対象全例ではこれらの体格指標とZ誘導を除く各誘導とAR間には負の相関を認めたが, 男女別ではこれを認めなかった.年齢とARにはいずれの誘導でも相関は認めなかった.FFTにおけるARの正常値設定に際しては, 性差が認められることに留意するのみならず, その要因となる因子を明らかにする必要性が示唆された.

加算平均心電図 (SAE) の心筋梗塞 (Ml) 後の致死性不整脈・突然死の予知に対する意義をprospectiveに検討した.1986年2月～1990年1月に急性心筋梗塞にて本院に入院した連続186例のうちSAEを記録, 分析可能であった130例について追跡調査した.平均追跡期間は4.7±2.4年間である.持続性心室頻拍・心室細動および突然死を不整脈事故と定義しその発生を調査したところ, 不整脈事故は発生は異常SAE群26例中6例 (23%) , また正常SAE群で104例中2例 (2%) と異常SAE群で有意に高率であった (P