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Nonalcoholic fatty liver disease (NAFLD) is associated with an increased risk of cardiovascular events. Although the association between NAFLD and aortic valve sclerosis has been described, the prevalence and prognostic implications of NAFLD among patients with severe aortic stenosis (AS) have not been described. In addition, the effect of the presence of severe tricuspid regurgitation (TR) on the prevalence of NAFLD remains unexplored. Accordingly, we investigated the prognostic implications of NAFLD among patients with severe AS with and without concomitant significant TR. A total of 538 patients (aged 80 ± 7 y, 49.6% men) who underwent noncontrast computed tomography before transcatheter aortic valve implantation (TAVI) between 2007 and 2019 were included. NAFLD was defined as a liver-to-spleen attenuation ratio1.0 on noncontrast computed tomography. NAFLD was present in 118 patients (21.9%). There were no significant differences in pulmonary arterial pressure, right atrial pressure, or the prevalence of significant TR between patients with and without NAFLD. During a median follow-up of 47 months (interquartile range 20 to 70 months), 224 patients (41.6%) died. Univariate Cox regression analysis demonstrated that NAFLD was not significantly associated with all-cause death among patients treated with TAVI (hazard ratio 1.32, 95% confidential interval 0.97 to 1.79, p = 0.07). In conclusion, among patients with severe AS who underwent TAVI, the prevalence of significant TR and the clinical outcomes were similar in patients with and without NAFLD.

The prevalence of Fabry disease (FD) in male patients with left ventricular hypertrophy (LVH) is about 1%. From the perspective of performing more efficient screening with measurement of α-galactosidase (α-Gal) activity, it is important to raise the pretest probability.We retrospectively investigated the prevalence of FD in 701 male patients with LVH who already had been screened by measurement of α-Gal activity in eight hospitals. From the viewpoint of enzymatic screening, we validated previously reported clinical features of FD including the electrocardiographic and echocardiographic characteristics with comparing each clinical determinant between patients with FD and non-FD patients. We finally aimed to establish a new screening approach for the detection of patients at high risk of FD.There were five FD patients (0.7%) in the 701 male patients with LVH. Those five patients with FD all had the cardiac variant type and age at detection of LVH was ≥35 years in all patients. In LVH patients with LV ejection fraction (EF) ≥ 50%, Pend-Q interval 40 msec, SV1 + RV5 4.0 mV, and diffuse LVH were important determinants of FD. In LVH patients with LVEF 50%, asymmetric septal hypertrophy and posterior wall motion abnormality seemed to be associated with FD.In our retrospective study, the prevalence of FD in male patients with LVH was found to be 0.7%. We established the efficient combinations of clinical determinants using age at detection of LVH, Pend-Q interval, high voltage, and LVH pattern in an echocardiogram.

D-dimer levels can predict ischemic stroke in patients with acute heart failure (AHF). However, the effects of direct oral anticoagulants on D-dimer levels have not been investigated during admission for AHF in patients with atrial fibrillation (AF). This study examined D-dimer levels immediately after admission and following edoxaban initiation as a sub-analysis of a multi-center study that investigated the pharmacokinetics and pharmacodynamics of edoxaban in patients with nonvalvular AF (NVAF) and AHF.Hospitalized patients with NVAF and AHF received edoxaban according to the label. The primary measure was the change in D-dimer levels on 7 consecutive days after admission for AHF. We also investigated differences according to prior edoxaban use (de novo at the time of admission or continuation).In 10/13 (76.9%) de novo patients, D-dimer levels exceeded the reference value (1.0 µg/mL) at admission (mean, 2.12 µg/mL) and subsequently decreased in 9 patients (at final blood sampling: mean, 1.12 µg/mL); 1 patient did not fall below the reference value due to stasis dermatitis. In the continuation group, most patients had D-dimer levels below the reference value from Day 1 (mean, 0.93 µg/mL), and levels remained stable or decreased (at final blood sampling: mean, 0.49 µg/mL). No events of stroke were observed.D-dimer levels may be elevated in patients with NVAF and AHF, particularly in those without prior anticoagulant treatment. Edoxaban may be effective for lowering and keeping D-dimer levels, a biomarker for predicting ischemic stroke, below the reference value in patients with NVAF and AHF.

Background Although cardiac resynchronization therapy (CRT) provided functional and clinical improvement in patients with heart failure (HF) and electrical intraventricular conduction disturbances, some patients had re-worsening left ventricular (LV) function after a favorable CRT response. We analyzed the clinical variables and cardiac outcomes associated with this re-worsening LV function after CRT. Methods In this study, 71 patients with CRT response who received CRT between 2006 and 2017 were included. CRT response was defined as a “≥ 10% improvement in LV ejection fraction (LVEF) on follow-up.” Patients were classified into two groups: (i) persistent: (n = 48, 68%), defined as those with a CRT response and (ii) re-worsening: (n = 23, 32%), consisting of those who fell out of the definition of a CRT response after an initial CRT response. Results Half of the patients in the re-worsening group failed to maintain a CRT response from two years upwards. A longer duration from HF diagnosis to CRT implantation, nonspecific intraventricular conduction delay (NIVCD) on electrocardiogram at CRT implantation, and a lower increased LVEF at initial CRT response were independent predictors for the re-worsening group. Patients in the re-worsening group had a higher incidence rate for HF hospitalization and cardiac deaths, compared with those in the persistent group. Conclusion One-third of CRT responders experienced re-worsening LVEF, which was associated with poor outcomes. CRT responders with NIVCD, longer HF duration, and a lower increased LVEF at initial CRT response should be monitored with caution.

BackgroundCurrent guidelines recommend placing an implantable cardiac defibrillator for patients with cardiac sarcoidosis (CS) and severely impaired left ventricular ejection fraction (LVEF) of ≤35%. In this study, we aimed to explore the association between mild or moderate LVEF impairment and fatal ventricular arrhythmic event (FVAE).MethodsHere, 401 patients with CS without sustained ventricular arrhythmia at diagnosis were retrospectively analyzed. The primary endpoint was an FVAE, which was defined as the combined endpoint of documented ventricular tachycardia or ventricular fibrillation and sudden cardiac death. Two cut-off points for LVEF were used; sex-specific lower threshold of normal range of LVEF, 52% for men and 54% for women, and LVEF of 35% which is used in the current guidelines.ResultsDuring a median follow-up of 3.2 years, 58 FVAEs were observed, and the 5- and 10-year estimated incidences of FVAE were 16.8% and 23.0%, respectively. All patients were classified into three groups according to LVEF: impaired LVEF group, mild to moderate impairment of LVEF group, and maintained LVEF group. Multivariable competing risk analysis showed that both the impaired LVEF group (HR: 3.02, 95% CI: 1.25–7.32) and the mild to moderate impairment of LVEF group (HR: 2.12, 95% CI: 1.02–4.40) were associated with a higher incidence of FVAE than the maintained LVEF group after adjustment for covariates.ConclusionsPatients with CS are at a high risk of FVAEs, regardless of documented ventricular arrhythmia at the time of diagnosis. In patients with CS, even mild to moderate impairment of LVEF is associated with FVAEs.Clinical PerspectiveWhat is KnownPatients with cardiac sarcoidosis (CS) are at a higher risk of fatal ventricular arrhythmic event (FVAE).Current guidelines adopt left ventricular ejection fraction (LVEF) ≤35% as a cut-off value for Class I indication for implantable cardioverter defibrillators (ICD) implantation.What the Study AddsCumulative incidence curves showed that the 5-year FVAE risk in patients with CS with preserved LVEF was 7%, which was as high as that of non-ischemic cardiomyopathy with reduced LVEF.For risk stratification of future fatal ventricular arrhythmic events, even milder left ventricular ejection fraction impairment, compared to that currently suggested by guidelines, needs to be considered as a risk factor in patients with cardiac sarcoidosis.Preventive strategies for fatal ventricular arrhythmic events and sudden cardiac death using an implantable cardiac defibrillator according to individualized risk stratification need to be developed and evaluated in clinical studies of patients with cardiac sarcoidosis.

Native T1 mapping is used to assess myocardial tissue characteristics without gadolinium contrast agents. The focal T1 high-intensity region can indicate myocardial alterations. This study aimed to identify the association between the native T1 mapping including the native T1 high region and left ventricular ejection fraction (LVEF) recovery in patients with dilated cardiomyopathy (DCM). Patients with newly diagnosed DCM (LVEF of 5 SD in the remote myocardium. Recovered EF was defined as a follow-up LVEF of ≥ 45% and an LVEF increase of ≥ 10% after 2 years from baseline. Seventy-one patients met the inclusion criteria for this study. Forty-four patients (61.9%) achieved recovered EF. Logistic regression analysis showed that the native T1 value (OR: 0.98; 95% CI: 0.96–0.99; P = 0.014) and the native T1 high region (OR: 0.17; 95% CI: 0.05–0.55; P = 0.002), but not late gadolinium enhancement, were independent predictors of recovered EF. Compared with native T1 value alone, combined native T1 high region and native T1 value improved the area under the curve from 0.703 to 0.788 for predicting recovered EF. Myocardial damage, which was quantified using native T1 mapping and the native T1 high region were independently associated with recovered EF in patients with newly diagnosed DCM.

Introduction Chronic obstructive pulmonary disease (COPD) and aortic stenosis (AS) are the most common diseases in aging population that their prevalence and percental change in mortality increase over the years. In severe AS, aortic valve replacement (AVR) is the only treatment that has demonstrated to improve survival, however the presence of comorbidities increases the operative risk and influences negatively on the outcomes after AVR. Therefore, the definition of COPD varies across the studies and is not always based on the use of pulmonary functional tests. Accordingly, the aim of the present study is to evaluate the association between pulmonary functional parameters and all-cause mortality after AVR in a large cohort of patients with severe AS. Methods Total of 400 patients (78.0 year-old, 56.7% men) with severe AS and documented preoperative pulmonary functional test (PFT) were retrospectively analyzed. Demographic and clinical characteristics were collected from electronic medical records while echocardiography was performed and measured according to the recommendations. PFTs were performed prior to AVR and categories defined in the Society of Thoracic Surgeons (STS) Adult Cardiac Surgery Database: normal pulmonary function was defined by an FEV1 >75% of predicted; mild COPD if FEV1 was 60–75% of predicted; moderate COPD if FEV1 was 50–59% of predicted and severe COPD when FEV1 Results Mild, moderate and severe COPD were documented in 75 (19%), 31 (8%) and 22 (5%) while the remaining 68% had normal PFTs. Patients with moderate and severe COPD had significantly larger LV mass and LV end-systolic volume whereas LV ejection fraction was significantly lower. The FVC, FEV1, Tiffeneau index, VC, PEF, and IC were the worst among patients with moderate and severe COPD (per definition) (p Conclusion Patients with moderate and severe COPD had higher LV hypertrophy and reduced LV ejection fraction while PFT parameters were the worst among these patients. The survival rates were significantly lower in patients with moderate and severe COPD compared with patients without COPD. In addition to other clinical factors, any grade of COPD was associated with 2-fold increased risk of all cause-mortality. Funding Acknowledgement Type of funding sources: None.

Background Right ventricular (RV) pacing causes left ventricular (LV) dyssynchrony sometimes resulting in pacing‐induced cardiomyopathy. However, RV pacing for hypertrophic obstructive cardiomyopathy is one of the treatment options. LV flow energy loss (EL) using vector flow mapping (VFM) is a novel hemodynamic index for assessing cardiac function. Our study aimed to elucidate the impact of RV pacing on EL in normal LV function and hypertrophic cardiomyopathy (HCM) patients. Methods A total of 36 patients with dual‐chamber pacemakers for sick sinus syndrome or implantable cardioverter defibrillators for fatal ventricular tachyarrhythmias were enrolled. All patients were divided into two groups: 16 patients with HCM (HCM group) and others (non‐HCM group). The absolute changes in EL under AAI (without RV pacing) and DDD (with RV pacing) modes were assessed using VFM on color Doppler echocardiography. Results In the non‐HCM group, the mean systolic EL significantly increased from the AAI to DDD modes (14.0 ± 7.7 to 17.0 ± 8.6 mW/m, P = .003), whereas the mean diastolic EL did not change (19.0 ± 12.3 to 17.0 ± 14.8 mW/m, P = .231). In the HCM group, the mean systolic EL significantly decreased from the AAI to DDD modes (26.7 ± 14.2 to 21.6 ± 11.9 mW/m, P, In non‐HCM patients, the RV pacing decreased the vortex area and the circulation and in HCM patients, it increased the vortex area and the circulation. The RV pacing significantly increased systolic energy loss in the non‐HCM group (P = .003), but it significantly decreased in the HCM group (P

BackgroundHealthcare workers (HCWs) treating and caring for patients with emerging infectious diseases often experience psychological distress. However, the psychological impact and behavior change of the coronavirus disease 2019 (COVID-19) pandemic among HCWs are still unknown. This study aimed to investigate the worries and concerns of HCWs regarding the COVID-19 pandemic.MethodsIn this cross-sectional survey, a web-based questionnaire was distributed among HCWs working in hospitals or clinics across Japanese medical facilities from April 20 to May 1, 2020. The questionnaire comprised items on demographics, worries and concerns, perceptions regarding the sufficiency of information, and behavioral changes pertaining to the COVID-19 pandemic.ResultsA total of 4386 HCWs completed the survey; 1648 (64.7%) were aged 30-39 years, 2379 (54.2%) were male, and 782 (18.1%) were frontline HCWs, directly caring for patients with COVID-19 on a daily basis. 3500 HCWs (79.8%) indicated that they were seriously worried about the pandemic. The most frequent concern was the consequence of becoming infected on their family, work, and society (87.4%). Additionally, the majority (55.5%) had restricted social contact and almost all HCWs endorsed a shortage in personal protective equipment (median, 8/9 (interquartile range; 7-9) on a Likert scale). There was no significant difference in the degree of worry between frontline and non-frontline HCWs (8/9 (7-9) vs. 8/9 (7-9), p=0.25). Frontline HCWs, compared to non-frontline HCWs, were more likely to have the need to avoid contact with families and friends (24.8% vs. 17.8%, pConclusionsBoth frontline and non-frontline HCWs expressed comparable concerns regarding the COVID-19 pandemic. Because HCWs, especially frontline HCWs, reported that they cannot be obliged to do avoid their duty, effective mental health protection strategies should be developed and implemented for HCWs.

Clinical parameters with correlation to diuretic effects after initiation of sodium-glucose cotransporter-2 (SGLT2) inhibitors are unclear. We aimed to identify the factors associated with the diuretic effect observed following the initiation of SGLT2 inhibitors in patients with diabetes having an acute heart failure (HF). Fifty-six patients included were hospitalized for acute HF with diabetes and started on SGLT2 inhibitors. Changes in urine volume (ΔUV) and blood/urine laboratory parameters before and during the first 4 days of therapy were evaluated. Data were prospectively obtained under clinically stable conditions after initial HF treatment. UV increased following the initiation of SGLT2 inhibitors [UV at baseline (BL): 1383 ± 479 mL/day; ΔUV over 4 days: + 189 ± 358 mL/day]. Multivariate analysis revealed no association between BL-hemoglobin A1c or BL-estimated glomerular filtration rate and ΔUV. Conversely, higher BL-fasting plasma glucose (FPG) and higher BL-urine N-acetyl-β-D-glucosaminidase (NAG) were associated with a higher ΔUV. ΔUV was inversely associated with ΔFPG and ΔNAG, and positively associated with Δurinary sodium excretion. Elevated FPG and NAG both improved over 4 days of treatment. In conclusion, the diuretic effect of SGLT2 inhibitors was glycemia-dependent, and was associated with a reduction in elevated renal-tubular markers in hospitalized HF complicated with diabetes.

Background Clinical significance of left atrial (LA) function and geometry in patients with dilated cardiomyopathy (DCM) remains uncertain. Hypothesis LA geometric parameters assessed by cardiac magnetic resonance (CMR) predict the prognosis in patients with DCM. Methods The present study included patients with DCM and sinus rhythm who underwent CMR between December 2007 and April 2018. LA volume was measured using CMR. LA sphericity index was computed as the ratio of the measured maximum LA volume by the volume of a sphere with maximum LA length diameter. Results We included 255 patients in this study. During the mean follow‐up of 3.92 years, hospitalization for HF occurred in 37 patients. The LA sphericity index was significantly higher in patients with hospitalization for HF than in those without (0.78 ± 0.35 vs. 0.58 ± 0.18, p

A 44-year-old man diagnosed with idiopathic dilated cardiomyopathy was admitted to our hospital with acute decompensated heart failure. Seven years before this admission, the first introduction of medication resulted in left ventricular (LV) recovery, which was sustained for several years. However, the patient stopped taking his medication, resulting in worsening of the LV function. Despite the second introduction of medication, the LV function did not improve. We performed cardiac magnetic resonance imaging and an endomyocardial biopsy, which revealed the significant development of cardiac fibrosis that had not been present at the time of the initial diagnosis.

Background: Left ventricular reverse remodeling by cardiac resynchronization therapy (CRT) can significantly improve the prognosis for heart failure (HF) patients. The time course of CRT response remains poorly characterized. Method and Results: This retrospective study included 99 HF patients who received a CRT device at a single center from 2006 to 2017. CRT response was defined as ≥10% improvement in left ventricular ejection fraction on follow-up. They were divided into three groups: early responders (ERs): 49 patients with CRT response by the six-month follow-up; late responders (LRs): 17 patients with CRT response between six-month and three-years; and non-responders (NRs): 33 patients with no CRT response. The ERs and LRs had significantly lower rates of HF hospitalization and all-cause deaths than the NRs. Multivariate logistic regression analysis identified that narrower QRS duration before implantation (p = 0.046) and the presence of moderate to severe mitral regurgitation (MR) at the six-month follow-up (p = 0.035) as independent predictors of NRs. Conclusions: Regardless of the timing, HF patients who have CRT response have a better long-term prognosis than NRs. The Severity of MR can predict whether a patient with no response at the six-month follow-up will be NRs.

ObjectivesDiagnosis of cardiac sarcoidosis (CS) without histological evidence remains controversial. This study aimed to compare characteristics and outcomes of histologically proven versus clinically diagnosed cases of CS, which were adjudicated using Heart Rhythm Society or Japanese Circulation Society criteria.MethodsA total of 512 patients with CS (age: 62±11 years, female: 64.3%) enrolled in the multicentre registry were studied. Histologically confirmed patients were classified as ‘biopsy-proven CS’, while those with the presence of strongly suggestive clinical findings of CS without histological evidence were classified as ‘clinical CS’. Primary outcome was a composite of all-cause death, heart failure hospitalisation and ventricular arrhythmia event.ResultsIn total, 314 patients (61.3%) were classified as biopsy-proven CS, while 198 (38.7%) were classified as clinical CS. Patients classified under clinical CS were associated with higher prevalence of left ventricular dysfunction, septal thinning, and positive findings in fluorodeoxyglucose-positron emission tomography or Gallium scintigraphy than those under biopsy-proven CS. During median follow-up of 43.7 (23.3–77.3) months, risk of primary outcome was comparable between the groups (adjusted HR: 1.24, 95% CI: 0.88 to 1.75, p=0.22). Similarly, the risks of primary outcome were comparable between patients with clinical isolated CS who did not have other organ/tissue involvement, and biopsy-proven isolated CS (adjusted HR: 1.23, 95% CI: 0.56 to 2.70, p=0.61).ConclusionsA substantial number of patients were diagnosed with clinical CS without confirmatory biopsy. Considering the worse clinical outcomes irrespective of the histological evidence, the diagnosis of clinical CS is justifiable if imaging findings suggestive of CS are observed.

Aims This study evaluated the prognosis and prognostic factors of patients with cardiac sarcoidosis (CS), an underdiagnosed disease. Methods and results Patients from a retrospective multicentre registry, diagnosed with CS between 2001 and 2017 based on the 2016 Japanese Circulation Society or 2014 Heart Rhythm Society criteria, were included. The primary endpoint was a composite of all-cause death, hospitalization for heart failure, and documented fatal ventricular arrhythmia events (FVAE), each constituting exploratory endpoints. Among 512 registered patients, 148 combined events (56 heart failure hospitalizations, 99 documented FVAE, and 49 all-cause deaths) were observed during a median follow-up of 1042 (interquartile range: 518–1917) days. The 10-year estimated event rates for the primary endpoint, all-cause death, heart failure hospitalizations, and FVAE were 48.1, 18.0, 21.1, and 31.9%, respectively. On multivariable Cox regression, a history of ventricular tachycardia (VT) or fibrillation [hazard ratio (HR) 2.53, 95% confidence interval (CI) 1.59–4.00, P Conclusion Although mortality is relatively low in CS, adverse events are common, mainly due to FVAE. Patients with low LVEF, with high BNP levels, with VT/fibrillation history, and requiring ablation to treat VT are at high risk.

Background The relationship between atrial high-rate episode (AHRE) burden (i.e., the frequency of atrial tachyarrhythmia) and heart failure (HF) risk is unclear. We hypothesized that new-onset and higher burden of AHRE are associated with HF. Methods and Results: We included 104 consecutive patients with cardiac implantable electronic devices (CIEDs) capable of continuous atrial rhythm monitoring. Patients with AF history were excluded. To stratify patients, AHREs were evaluated only during the initial 1 year after CIED implantation. The primary endpoint was all-cause death or new-onset or worsening HF that required unplanned hospitalization or readjustment of HF drug therapy. At 1 year after CIED implantation, 34/104 patients (33%) exhibited AHREs. No difference in basal clinical characteristics except for left ventricular ejection fraction between patients with and without new-onset AHREs was found. AHRE groups had more HF events than the non-AHRE group. All patients were divided into 3 groups based on AHRE burden: none, low, and high. Worsening HF was observed in 12 patients (12%). Cox hazard analysis revealed that AHRE and higher AHRE burden were independent predictive factors for worsening HF. The high group showed a higher risk for HF than the non-AHRE groups, but no significant difference was found between the low- and non-AHRE groups. Conclusions New-onset higher AHRE burden was associated with subsequent risk for HF in patients with CIEDs.

Aims Intestinal oedema is one of the manifestations associated with right‐sided heart failure (HF), which is known to be associated with poorer patient outcomes. We attempted to reveal the association between intestinal oedema and diuretic resistance in hospitalized patients with acute HF. Methods and results Among 213 hospitalized patients with acute HF, abdominal ultrasonography was performed under clinically stable conditions after initial HF treatments. The association among abdominal ultrasonographic parameters, maintenance doses of loop diuretics, and responsiveness to initial loop diuretic treatment was evaluated. Higher mean colon wall thickness (CWT) independently correlated with a higher dose of loop diuretics at enrolment (adjusted β = 0.198, P = 0.0004). Increased mean CWT also correlated with poor response to oral loop diuretics as an initial treatment, whereas it did not correlate with the response to intravenous loop diuretics. Discrimination of non‐responders to initial oral loop diuretics resulted in a sensitivity of 0.772 and a specificity of 0.733 using a mean CWT cut‐off value of ≥3 mm. Conclusions In hospitalized patients with acute HF, a strong correlation was identified among the severity of intestinal oedema, required quantities as maintenance loop diuretic doses, and poor responsiveness to oral loop diuretics at admission.

In November 2020, atezolizumab plus bevacizumab became available for the treatment of hepatocellular carcinoma (HCC), and its efficacy is expected as a new treatment option for HCC. However, the occurrence of immune-related adverse events (irAEs) associated with the administration of immune checkpoint inhibitors is a major concern in clinical practice. We reported a case of irAE-induced myocarditis after the treatment for HCC. Based on the symptoms and echocardiographic findings, we suspected irAE-induced myocarditis and acute heart failure, and the patient was admitted to the hospital for further investigation and treatment. From starting the patient on therapy with methylprednisolone succinate sodium, the laboratory data and symptoms tended to improve. The patient was discharged to home on the 25th day of treatment. Because the number of patients with irAE myocarditis is expected to increase in clinical practice in the near future, further accumulation and investigation of cases are necessary.

Background: In patients with heart failure (HF), discontinued medical therapy because of adverse events (AE) is associated with high mortality. Patients with type 2 diabetes mellitus (T2DM) treated with sodium-glucose co-transporter 2 inhibitors (SGLT2i) have a lower risk of HF, but AE sometimes occur with the introduction of SGLT2i. In order to use SGLT2i safely in patients with HF, we investigated factors associated with AE following the introduction of SGLT2i. Methods and Results: AE were defined as hypotension or an increase in serum creatinine ≥0.3 mg/dL by the fifth day after SGLT2i introduction. Sixty-four hospitalized patients with HF and T2DM treated with an SGLT2i were enrolled in this study. Patients were divided into 2 groups: with AE (n=13, 20.3%) and without (n=51, 79.7%). On logistic regression analysis, female sex, hemoglobin ≥15.2 g/dL, serum creatinine ≥1.05 mg/dL, and cardiac index on echocardiography ≤2.15 L/min/m2, were significantly associated with AE. A scoring system was constructed to predict AE according to significant variables (area under the receiver operating characteristic curve, 0.83; P

Background Left atrial (LA) enlargement is associated with cardiac events in patients with dilated cardiomyopathy (DCM). However, the clinical significance of LA function and geometry in patients with DCM remains uncertain. Cardiac magnetic resonance (CMR) is the current gold standard modality to assess LA function and geometry. Purpose The aim of the present study was to investigate the clinical significance of LA parameters assessed by CMR in patients with DCM. Methods The present study included patients with DCM and sinus rhythm who underwent CMR between December 2007 and April 2018. LA volume was measured using CMR. LA empty fraction was calculated according to LA volume differences. The LA sphericity index was computed as the ratio of the measured LA maximum volume to the volume of a sphere with maximum LA length diameter (Figure 1A). We investigated the relationship between LA parameters measured by CMR and hospitalization for heart failure (HF). Results We included 255 patients in this study. During the mean follow-up of 3.66 [1.85–6.25] years, hospitalization for HF occurred in 37 patients (14.5%). Although there were no significant differences in LA volume and LA empty fraction between patients with HF hospitalization and those without, LA sphericity index was significantly higher in patients with HF hospitalization than in those without (0.78±0.35 vs. 0.58±0.18, P Conclusions LA sphericity index was an independent predictor of hospitalization for HF. Assessment of LA geometric parameter including LA sphericity might be useful for risk stratification toward worsening HF in patients with DCM. Figure 1 Funding Acknowledgement Type of funding source: None

Background Re-worsening left ventricular ejection fraction (LVEF) after initial recovery occurs in some patients with dilated cardiomyopathy (DCM). However, prevalence and predictors of re-worsening LVEF in longitudinal follow-up are unclear. Late gadolinium enhancement of cardiovascular magnetic resonance (LGE-CMR) can evaluate the damage of myocardial tissue. Purpose This study sought to evaluate the clinical parameters including LGE-CMR to predict re-worsening LVEF in patients with recent-onset DCM. Methods We included patients with recent-onset DCM who had an LVEF 5% LVEF from baseline and had an LVEF≥45% after medical therapy. Patients were divided into three groups: (1) Improved: defined as those with sustained LVEF ≥45% after initial LVEF recovery; (2) Re-worse: those with decreased >5% and had an LVEF Results Of 138 patents, 82 patients (59%) were the Improved group, 42 patients (30%) were the Re-worse group, and 14 (10%) were the Not-improved group. Loess curves of long-term LVEF trajectories showed that LVEF in the Re-worse group increased first 2 years and declined slowly thereafter (Fig. 1A). Re-worsening LVEF occurred 4.5±2.2 years after initial LVEF recovery. Multivariate logistic regression analysis demonstrated that LGE area at baseline (Odds ratio: 1.09, 95% confidence interval (CI) 1.02–1.18, p=0.014) and Log brain natriuretic peptide (BNP) at initial LVEF recovery (Odds ratio: 1.53, 95% confidence interval (CI) 1.01–2.31, p=0.042) were independent predictors for Re-worsening LVEF. Kaplan Meier analysis demonstrated that the risk of cardiac events in the Re-worse group was significantly higher (hazard ratio: 3.93, 95% CI 1.49–10.36, p=0.006) than in the Improved group and lower risk than in the Not-improved group (hazard ratio: 0.28, 95% CI 0.12–0.62, p=0.002) (Fig. 1B). Conclusion Re-worsening LVEF occurred in 30% of patients in patients with recent-onset DCM. LGE area and BNP at initial LVEF recovery were independently associated with re-worsening LVEF after initial LVEF recovery. Figure 1 Funding Acknowledgement Type of funding source: None

Our aim is to clarify the factors for early diagnosis of cardiac sarcoidosis (CS) in patients with complete atrioventricular block (CAVB) and its impact on cardiac function after corticosteroid therapy.A total of 15 CS patients with CAVB who underwent corticosteroid therapy were retrospectively analyzed. Patients were divided into two groups according to the time from the first CAVB onset to the diagnosis of CS. Clinical characteristics and outcomes were compared between the early diagnosis group (within 1 year; group E, n = 10) and the late diagnosis group (over 1 year; group L, n = 5).The history of extracardiac sarcoidosis (60 versus 0%, P = 0.0440) and abnormal findings on echocardiography (70 versus 0%, P = 0.0256) at the CAVB onset were significantly more frequent in group E than in group L. The change of left ventricular ejection fraction (LVEF) and brain natriuretic peptide (BNP) levels was significantly better in group E than in group L (0.8 ± 2.8 versus -32.4 ± 3.9%, P < 0.0001; -11.1 ± 16.0 versus 161.8 ± 35.8 pg/mL, P = 0.0013, respectively). After corticosteroid therapy, the LVEF and BNP levels were also significantly better in group E than in group L (53.3 ± 10.7 versus 37.0 ± 9.3%, P = 0.0128; 63.0 ± 46.4 versus 458.8 ± 352.0 pg/mL, P = 0.0027).The diagnosis may be delayed in CS patients with CAVB without history of extracardiac sarcoidosis. Abnormal findings on echocardiography contributed to the early diagnosis of CS. Therefore, the diagnosis of CS may be missed or delayed in patients without them. Time delay from the CAVB onset to the CS diagnosis may exacerbate the cardiac function.

Re-worsening left ventricular ejection fraction (LVEF) is observed in some patients with dilated cardiomyopathy (DCM) despite initial improvements in LVEF. We analyzed cardiac outcomes and clinical variables associated with this re-worsening LVEF. A total of 180 newly diagnosed DCM patients who received only pharmacotherapy were enrolled. Echocardiography was performed after 6, 12, 24, and 36 months after initiation of pharmacotherapy. Patients were divided into three groups: (1) Improved: (n = 113, 63%), defined as those > 10% increase in LVEF after 12 months and no decrease (> 10%) between 12 and 36 months; (2) Re-worse: (n = 12, 7%), those with > 10% increase in LVEF after 12 months but with decrease (> 10%) between 12 and 36 months; and (3) Not-improved: (n = 55: 30%), those with no increase in LVEF (> 10%) after 12 months. Patients with re-worse group were older (P = 0.04) and had higher brain natriuretic peptide (BNP) levels after 12 months (P = 0.002) than those in the Improved group. Major cardiac events (sudden death, implantation of a ventricular assist device, and death due to heart failure,) were observed in 13 (7%) patients after 36 months of pharmacotherapy. Multivariate analysis revealed that the Re-worse group had a higher risk for cardiac events (hazard ratio 11.7, 95% confidence interval 1.9-90.7, P = 0.01) than the Improved group, but had a similar risk compared with the Not-improved group. Re-worsening LVEF was associated with poor cardiac outcomes in newly diagnosed DCM patients. Age and persistently high-BNP levels after improvement in LVEF were significantly associated with re-worsening LVEF.

An interaction between the intestine and cardiovascular disease has been suggested. We thought to clarify the association between intestinal conditions and clinical outcomes in patients with heart failure (HF). Hemodynamic parameters in intestinal vessels [superior mesenteric artery (SMA), inferior mesenteric artery (IMA), and portal vein (PV)] and average colon wall thickness (aCWT) from the ascending colon to sigmoid colon were evaluated in 224 hospitalized HF patients. Echocardiographic parameters and composite event rates (all-cause mortality, readmission for HF deterioration, major ventricular arrhythmias) were also examined. Higher PV congestion index (CI) and aCWT were observed in patients with New York Heart Association (NYHA) class III/IV. Higher PVCI [hazard ratio (HR) per + 1 standard deviation (SD) 1.50, p 0.01] and aCWT (HR per + 1 SD 1.45, p 0.01) were independently associated with higher composite event rates during the follow-up of 122 ± 68 days. None of SMA/IMA hemodynamic parameters were associated with NYHA class or composite event rates. Higher right ventricular end-diastolic dimension (38 ± 7 vs 34 ± 9 mm, p 0.01) and lower tricuspid annual plane systolic excursion (15 ± 5 vs 19 ± 5 mm, p 0.001) were observed in patients with higher PVCI ( 0.031 cm s) and aCWT ( 2.8 mm) relative to those in others. In conclusion, increased portal congestion and intestinal edema were associated with severe HF symptoms and poor outcomes in hospitalized HF patients, in addition to being associated with impaired right-sided cardiac function.

Background Right ventricular (RV) pacing causes left ventricular (LV) dysfunction. On the other hand, RV pacing for hypertrophic obstructive cardiomyopathy (HOCM) is an established treatment. LV flow energy loss (EL) is a new hemodynamic index for assessing cardiac function. However, the impact of RV pacing on EL remains unknown. Objective The objective of this study was to investigate the EL by RV pacing on normal LV function and hypertrophic cardiomyopathy (HCM). Methods A total of 28 patients underwent echocardiography for EL assessment under AAI (without RV pacing) and DDD (with all RV pacing) mode. Among them, 16 were sick sinus syndrome (SSS) patients with normal LV function, and 12 were HCM patients. EL was calculated from color Doppler images using a vector flow mapping. Results There were no significant difference in patients' background parameters between the SSS and the HCM groups. In the SSS group, mean systolic EL was significantly increased from AAI to DDD mode (14.9 to 19.2 mW/m, P Change of flow energy loss Conclusion RV pacing increased mean systolic EL in normal LV function, but decreased in HCM with or without LV outflow obstruction. In the patients with HCM, the impact of RV pacing on EL is different compared with normal LV function.

Background Normalization of left ventricular (LV) contraction is a strong surrogate marker of favorable prognosis in patients with dilated cardiomyopathy (DCM). Although several studies have reported the individual predictive significance of clinical and imaging parameters for LV recovery in patients with DCM, there have been no reports on the scoring systems that combine these multifactorial parameters. Methods and results In 406 idiopathic patients with DCM, there were 185 (46%) with LV recovery at 1-year follow-up after pharmacotherapy, which was defined as improvements in LV ejection fraction of ≥+10% together with absolute values of ≥50%. Multivariate analysis demonstrated that eight baseline clinical factors and the absence of late gadolinium enhancement (negative LGE) on magnetic resonance imaging were independently associated with LV recovery. The highest odds ratio for the prediction of LV recovery was negative LGE (odds ratio: 5.62, 95% confidence interval: 2.97–10.6; p Conclusion Combined assessment using clinical and imaging parameters has a high prognostic value for predicting response to pharmacotherapy in patients with DCM.

The clinical impact of left ventricular (LV) segmental wall motion abnormalities (SWMA) in patients with idiopathic dilated cardiomyopathy (IDCM) has not been well elucidated.Among 100 consecutive IDCM patients with follow-up visits, we enrolled 85 after excluding those with left bundle branch block and/or ventricular pacemaker implantation. LV wall motion was assessed using left ventriculography scored for 7 segments according to the American Heart Association classification as follows: 0, normokinesis; 1, hypokinesis; 2, akinesis; and 3, dyskinesis. SWMA were defined as a score dispersion of more than 1 degree among the segments.SWMA was exhibited by 26 patients. Kaplan-Meier curves demonstrated that the patients with SWMA (SWMA+) had a significantly higher cardiac event-free rate than the patients without SWMA (P < 0.001). Cox proportional hazards analysis showed that SWMA+ was an independent predictor of cardiac events (P = 0.03; hazard ratio = 3.38; 95% confidence interval [CI], 1.11-10.8). Furthermore, multiple regression analysis showed that SWMA+ was an independent predictor of decreased LV end-systolic dimension index after optimal pharmacotherapy (β = -0.24; 95%CI, -9.12 to -0.73; P = 0.02).SWMA is common in patients with IDCM and is independently associated with a poor prognosis and less morphometric and functional improvement of LV in response to pharmacotherapy.

Tafamidis meglumine is a novel medicine that has been shown to slow the progression of peripheral neurological impairment in patients with hereditary transthyretin amyloidosis (ATTR). However, the efficacy of tafamidis against ATTR-related cardiac amyloidosis remains unclear. A 72-year-old woman had cardiac hypertrophy and axonopathy in her lower legs. Endomyocardial biopsy revealed an infiltrative cardiomyopathy consistent with amyloidosis. Immunostaining and genetic studies confirmed the diagnosis of ATTR, and tafamidis was started subsequently. Two years after the initiation of tafamidis treatment, electromyography demonstrated no change in the axonopathy in her lower legs; however, electrocardiography displayed QRS prolongation, and echocardiography disclosed an increase in interventricular septal thickness. Endomyocardial biopsy indicated that transthyretin amyloid infiltration of the myocardium was not reduced. In this case, there was no apparent progression of axonopathy, although there were signs of worsening amyloid cardiomyopathy during the treatment with tafamidis.

Objectives The aim of this study was to evaluate the association between late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging and ventricular arrhythmias or sudden cardiac death (SCD) in patients with dilated cardiomyopathy (DCM). Background Risk stratification for SCD in DCM needs to be improved. Methods A systematic review and meta-analysis were conducted. A systematic search of PubMed and Ovid was performed, and observational studies that analyzed the arrhythmic endpoint (sustained ventricular arrhythmia, appropriate implantable cardioverter-defibrillator [ICD] therapy, or SCD) in patients with DCM, stratified by the presence or absence of LGE, were included. Results Twenty-nine studies were included, accounting for 2,948 patients. The studies covered a wide spectrum of DCM, with a mean left ventricular ejection fraction between 20% and 43%. LGE was significantly associated with the arrhythmic endpoint both in the overall population (odds ratio: 4.3; p 35% (odds ratio: 5.2; p Conclusions Across a wide spectrum of patients with DCM, LGE is strongly and independently associated with ventricular arrhythmia or SCD. LGE could be a powerful tool to improve risk stratification for SCD in patients with DCM. These results raise 2 major questions to be addressed in future studies: whether patients with LGE could benefit from primary prevention ICDs irrespective of their left ventricular ejection fractions, while patients without LGE might not need preventive ICDs despite having severe left ventricular dysfunction.