Type 1 diabetes, its complications, and non-ischemic cardiomyopathy: a mendelian randomization study of European ancestry.

BACKGROUND: Type 1 diabetes (T1D) is a significant risk factor for a range of cardiovascular diseases. Nonetheless, the causal relationship between T1D and non-ischemic cardiomyopathy (NICM) remains to be elucidated. Furthermore, the mechanisms responsible for the progression from T1D to NICM have not been definitively characterized. OBJECTIVE: The aim of this study was to conduct a Mendelian randomization (MR) study to investigate the causal effects of T1D and its complications on the development of NICM. Additionally, this study aimed to conduct a mediation analysis to identify potential mediators within this correlation. METHODS: Genetic variants were used as instrumental variables for T1D. The summary data for T1D were obtained from two genome-wide association study datasets. The summary data for T1D with complications and NICM were obtained from the Finnish database. Two-sample MR, multivariable MR and mediation MR were conducted in this study. RESULTS: The study revealed a causal association between T1D, T1D with complications, and NICM (with odds ratios of 1.02, 95% CI 1.01-1.04, p = 1.17e-04 and 1.03, 95% CI 1.01-1.05, p = 3.15e-3). Even after adjusting for confounding factors such as body mass index and hypertension, T1D remained statistically significant (with odds ratio of 1.02, 95% CI 1.01-1.04, p = 1.35e-4). Mediation analysis indicated that monokine induced by gamma interferon may play a mediating role in the pathogenesis of T1D-NICM (mediation effect indicated by odds ratio of 1.005, 95% CI 1.001-1.01, p = 4.9e-2). CONCLUSION: The study demonstrates a causal relationship between T1D, its complications, and NICM. Additionally, monokine induced by gamma interferon may act as a potential mediator in the pathogenesis of T1D-NICM.

MRI Assessment of Myocardial Deformation for Risk Stratification of Major Arrhythmic Events in Patients With Non-Ischemic Cardiomyopathy Eligible for Primary Prevention Implantable Cardioverter Defibrillators.

BACKGROUND: Implantable cardioverter-defibrillator (ICD) intervention is an established prophylactic measure. Identifying high-benefit patients poses challenges. PURPOSE: To assess the prognostic value of cardiac magnetic resonance imaging (MRI) parameters including myocardial deformation for risk stratification of ICD intervention in non-ischemic cardiomyopathy (NICM) while accounting for competing mortality risk. STUDY TYPE: Retrospective and prospective. POPULATION: One hundred and fifty-nine NICM patients eligible for primary ICD (117 male, 54 ± 13 years) and 49 control subjects (38 male, 53 ± 5 years). FIELD STRENGTH/SEQUENCE: Balanced steady state free precession (bSSFP) and three-dimensional phase-sensitive inversion-recovery late gadolinium enhancement (LGE) sequences at 1.5 T or 3 T. ASSESSMENT: Patients underwent MRI before ICD implantation and were followed up. Functional parameters, left ventricular global radial, circumferential and longitudinal strain, right ventricular free wall longitudinal strain (RV FWLS) and left atrial strain were measured (Circle, cvi42). LGE presence was assessed visually. The primary endpoint was appropriate ICD intervention. Models were developed to determine outcome, with and without accounting for competing risk (non-sudden cardiac death), and compared to a baseline model including LGE and clinical features. STATISTICAL TESTS: Wilcoxon non-parametric test, Cox's proportional hazards regression, Fine-Gray competing risk model, and cumulative incidence functions. Harrell's c statistic was used for model selection. A P value <0.05 was considered statistically significant. RESULTS: Follow-up duration was 1176 ± 960 days (median: 896). Twenty-six patients (16%) met the primary endpoint. RV FWLS demonstrated a significant difference between patients with and without events (-12.5% ± 5 vs. -16.4% ± 5.5). Univariable analyses showed LGE and RV FWLS were significantly associated with outcome (LGE: hazard ratio [HR] = 3.69, 95% CI = 1.28-10.62; RV FWLS: HR = 2.04, 95% CI = 1.30-3.22). RV FWLS significantly improved the prognostic value of baseline model and remained significant in multivariable analysis, accounting for competing risk (HR = 1.73, 95% CI = 1.12-2.66). DATA CONCLUSIONS: In NICM, RV FWLS may provide additional predictive value for predicting appropriate ICD intervention. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY: Stage 5.

Competency based curriculum for cardiovascular magnetic resonance: A position statement of the Society for Cardiovascular Magnetic Resonance.

This position statement guides cardiovascular magnetic resonance (CMR) imaging program directors and learners on the key competencies required for Level II and III CMR practitioners, whether trainees come from a radiology or cardiology background. This document is built upon existing curricula and was created and vetted by an international panel of cardiologists and radiologists on behalf of the Society for Cardiovascular Magnetic Resonance (SCMR).

Hemoglobin is an independent predictor of improvement exercise tolerance in male patients with non-ischemic cardiomyopathy.

Exercise intolerance is a symptom of chronic heart failure (CHF). The magnitude of exercise tolerance, as measured by peak oxygen uptake (peak VO2), is strongly associated with prognosis in patients with CHF. We aimed to evaluate the factors associated with improved exercise tolerance in patients with HF. In this prospective study, we recruited patients who were diagnosed with non-ischemic cardiomyopathy between September 2017 and September 2021. All patients underwent cardiopulmonary exercise testing at discharge and 6 months after enrollment. The patients were stratified according to whether peak VO2 was increased or not at 6 months. One hundred patients with a reduced left-ventricular ejection fraction (LVEF < 50%) were enrolled. Improvement of peak VO2 was observed in 74 patients. In male patients, hemoglobin level was higher in the increased peak VO2 group than in the non-increased group (15.0 ± 1.9 g/dL vs. 13.1 ± 2.1 g/dL; p < 0.01). Baseline hemoglobin level was positively correlated with the percentage change in peak VO2 (Spearman's r = 0.248, p = 0.040). Kaplan-Meier analysis demonstrated that adverse cardiac events were significantly less frequent in the increased peak VO2 group than in the non-increased group (log-rank test, p = 0.032). Multivariate logistic regression analysis identified hemoglobin level as an independent predictor of improved peak VO2 [odds ratio (OR) 1.60; 95% confidence interval (CI) 1.05-2.44; p = 0.027]. Baseline hemoglobin level is an independent predictor of improved peak VO2 in male patients with non-ischemic cardiomyopathy.

Review of cardiovascular imaging in the Journal of Nuclear Cardiology 2022: positron emission tomography, computed tomography, and magnetic resonance.

In 2022, the Journal of Nuclear Cardiology® published many excellent original research articles and editorials focusing on imaging in patients with cardiovascular disease. In this review of 2022, we summarize a selection of articles to provide a concise recap of major advancements in the field. In the first part of this 2-part series, we addressed publications pertaining to single-photon emission computed tomography. In this second part, we focus on positron emission tomography, cardiac computed tomography, and cardiac magnetic resonance. We specifically review advances in imaging of non-ischemic cardiomyopathy, cardio-oncology, infectious disease cardiac manifestations, atrial fibrillation, detection and prognostication of atherosclerosis, and technical improvements in the field. We hope that this review will be useful to readers as a reminder to articles they have seen during the year as well as ones they have missed.

Two-Year Outcomes of Primary Prophylactic Use of Defibrillators for Ischemic and Non-Ischemic Cardiomyopathy　- Propensity Score-Matched Analysis From the Nippon Storm Study.

BACKGROUND: In 2016, the DANISH study reported negative results regarding the efficacy of implantable cardioverter-defibrillators (ICDs) in patients with non-ischemic cardiomyopathy (NICM) and reduced left ventricular ejection fraction (LVEF). In this study we determined the efficacy of using ICDs for primary prophylaxis in patients with NICM.Methods and Results: We selected 1,274 patients with underlying cardiac disease who were enrolled in the Nippon Storm Study. We analyzed the data of 451 patients with LVEF ≤35% due to NICM or ischemic cardiomyopathy (ICM) who underwent ICD implantation for primary prophylaxis (men, 78%; age, 65±12 years; LVEF, 25±6.4%; cardiac resynchronization therapy, 73%; ICM, 33%). After propensity score matching, we compared the baseline covariates between groups: NICM (132 patients) and ICM (132 patients). The 2-year appropriate ICD therapy risks were 27.7% and 12.2% in the NICM and ICM groups, respectively (hazard ratio, 0.390 [95% confidence interval, 0.218-0.701]; P=0.002). CONCLUSIONS: This subanalysis of propensity score-matched patients from the Nippon Storm Study revealed that the risk of appropriate ICD therapy was significantly higher in patients with NICM than in those with ICM.

Changes in extracellular matrix in failing human non-ischemic and ischemic hearts with mechanical unloading.

Ischemic and non-ischemic cardiomyopathies have distinct etiologies and underlying disease mechanisms, which require in-depth investigation for improved therapeutic interventions. The goal of this study was to use clinically obtained myocardium from healthy and heart failure patients, and characterize the changes in extracellular matrix (ECM) in ischemic and non-ischemic failing hearts, with and without mechanical unloading. Using tissue engineering methodologies, we also investigated how diseased human ECM, in the absence of systemic factors, can influence cardiomyocyte function. Heart tissues from heart failure patients with ischemic and non-ischemic cardiomyopathy were compared to explore differential disease phenotypes and reverse remodeling potential of left ventricular assisted device (LVAD) support at transcriptomic, proteomic and structural levels. The collected data demonstrated that the differential ECM compositions recapitulated the disease microenvironment and induced cardiomyocytes to undergo disease-like functional alterations. In addition, our study also revealed molecular profiles of non-ischemic and ischemic heart failure patients and explored the underlying mechanisms of etiology-specific impact on clinical outcome of LVAD support and tendency towards reverse remodeling.

A two-stage cardiac PET and late gadolinium enhancement MRI co-registration method for improved assessment of non-ischemic cardiomyopathies using integrated PET/MR.

PURPOSE: Respiratory motion causes mismatches between PET images of the myocardium and the corresponding cardiac MR images in cardiac integrated PET/MR. The mismatch may affect the attenuation correction and the diagnosis of non-ischemic cardiomyopathies. In this study, we present a two-stage cardiac PET and MR late gadolinium enhancement (LGE) co-registration method, which seeks to improve diagnostic accuracy of non-ischemic cardiomyopathies via better image co-registration using an integrated whole-body PET/MR system. METHODS: The proposed PET and LGE two-stage co-registration method was evaluated through comparison with one-stage direct co-registration and no-registration. One hundred and ninety-one slices of LGE and forty lesions were studied. Two trained nuclear medicine physicians independently assessed the displacement between LGE and PET to qualitatively evaluate the co-registration quality. The changes of the mean SUV in the normal myocardium and the LGE-enhanced lesions before and after image co-registration were measured to quantitatively evaluate the accuracy and value of image co-registration. RESULTS: The two-stage method had an improved image registration score (4.93 ± 0.89) compared with the no-registration method (3.49 ± 0.84, p value < 0.001) and the single-stage method (4.23 ± 0.81, p value < 0.001). Furthermore, the two-stage method led to increased SUV value in the myocardium (3.87 ± 2.56) compared with the no-registration method (3.14 ± 1.92, p value < 0.001) and the single-stage method (3.32 ± 2.16, p value < 0.001). The mean SUV in the LGE lesion significantly increased from 2.51 ± 2.09 to 2.85 ± 2.35 (p value < 0.001) after the two-stage co-registration. CONCLUSION: The proposed two-stage registration method significantly improved the co-registration between PET and LGE in integrated PET/MR imaging. The technique may improve diagnostic accuracy of non-ischemic cardiomyopathies via better image co-registration. REGISTERED NO: DF-2020-085,2020.04.30.

Highlights of the Virtual Society for Cardiovascular Magnetic Resonance 2022 Scientific Conference: CMR: improving cardiovascular care around the world.

The 25th Society for Cardiovascular Magnetic Resonance (SCMR) Annual Scientific Sessions saw 1524 registered participants from more than 50 countries attending the meeting virtually. Supporting the theme "CMR: Improving Cardiovascular Care Around the World", the meeting included 179 invited talks, 52 sessions including 3 plenary sessions, 2 keynote talks, and a total of 93 cases and 416 posters. The sessions were designed so as to showcase the multifaceted role of cardiovascular magnetic resonance (CMR) in identifying and prognosticating various myocardial pathologies. Additionally, various social networking sessions as well as fun activities were organized. The major areas of focus for the future are likely to be rapid efficient and high value CMR exams, automated and quantitative acquisition and post-processing using artificial intelligence and machine learning, multi-contrast imaging and advanced vascular imaging including 4D flow.

Catheter ablation of ventricular tachycardia in dilated-phase hypertrophic cardiomyopathy: Substrate characterization and ablation outcome.

INTRODUCTION: Catheter ablation is a therapeutic option to suppress ventricular tachycardia (VT) in the setting of dilated-phase hypertrophic cardiomyopathy (DHCM). However, the characteristics of the arrhythmogenic substrate and the ablation outcome are not fully illustrated. METHOD: A total of 23 ablation procedures for drug-refractory sustained monomorphic VTs in 13 DHCM patients (60 ± 11 years, one female, the left ventricular [LV] ejection fraction 39% ± 9%, the LV mass index 156 ± 39 g/m2 ) were performed. The distribution of VT substrate as endocardial or epicardial/intramural was based on detailed mapping and ablation response during VT. RESULT: Two patients underwent ablation of sustained monomorphic VT that was not scar-mediated tachycardia. Of the remaining 11 patients, eight (73%) patients had VT substrate in the basal regions, most frequently at the epicardial and/or intramural basal antero-septum. None of the patients had VT substrate located at the LV inferolateral region. Ablation at the right ventricular septum and the aortic cusps was done in four and five patients, respectively. Other approaches including bipolar and chemical ablations, were done in three and two patients, respectively. Six (55%) out of 11 patients (two patients lost follow-up) had VT recurrence. All the six patients had basal substrate. However, anti-tachycardia pacing was sufficient for VT termination except in one patient. CONCLUSION: Catheter ablation of VT in patients with DHCM is challenging because of the predominant basal anteroseptal epicardial/intramural location of arrhythmogenic substrate. An ablation approach from multiple sites and/or adjunctive interventional techniques are often required.

PACAP-38 and PAC1 Receptor Alterations in Plasma and Cardiac Tissue Samples of Heart Failure Patients.

Pituitary adenylate cyclase activating polypeptide-38 (PACAP-38) is a multifunctional neuropeptide, which may play a role in cardioprotection. However, little is known about the presence of PACAP-38 in heart failure (HF) patients. The aim of our study was to measure the alterations of PACAP-38 like immunoreactivity (LI) in acute (n = 13) and chronic HF (n = 33) and to examine potential correlations between PACAP-38 and HF predictors (cytokines, NT-proBNP). Tissue PACAP-38 LI and PAC1 receptor levels were also investigated in heart tissue samples of patients with HF. Significantly higher plasma PACAP-38 LI was detected in patients with acute HF, while in chronic HF patients, a lower level of immunoreactivity was observed compared to healthy controls (n = 13). Strong negative correlation was identified between plasma PACAP-38 and NT-proBNP levels in chronic HF, as opposed to the positive connection seen in the acute HF group. Plasma IL-1 ß, IL-2 and IL-4 levels were significantly lower in chronic HF, and IL-10 was significantly higher in patients with acute HF. PACAP-38 levels of myocardial tissues were lower in all end-stage HF patients and lower PAC1 receptor levels were detected in the primary dilated cardiomyopathy group compared to the controls. We conclude that PACAP-38 and PAC1 expression correlates with some biomarkers of acute and chronic HF; therefore, further studies are necessary to explore whether PACAP could be a suitable prognostic biomarker in HF patients.

Acute and long-term outcomes of VT radiofrequency catheter ablation in patients with versus without an intramural septal substrate.

INTRODUCTION: Aim of this study was to evaluate efficacy and safety of ventricular tachycardia (VT) catheter ablation in patients with structural heart disease (SHD) in relation to the presence of an intramural septal substrate. METHODS: Consecutive patients undergoing VT ablation between January 2019 and October 2020 were included. All patients were stratified based on the presence of relevant septal substrate and freedom from VT recurrences were analyzed. RESULTS: In total, 199 consecutive patients (64.2 ± 13.0 years; 89% male; 55% ischemic cardiomyopathy (ICM)) undergoing VT ablation were included. 129/199 patients (65%) showed significant septal substrate (55/90 patients (61%) with non-ischemic cardiomyopathy (NICM) compared to 74/109 patients (68%) with ICM; p = 0.37). Acute procedural success with elimination of all inducible VTs was achieved in 66/70 patients (94%) without and in 103/129 patients (80%) with a septal substrate (p = 0.007). In the cohort including patients with a clinical FU, 15/60 patients (25%) without a septal substrate and 48/123 patients (39%) with a septal substrate experienced VT recurrence during a FU of 8.1 ± 5.9 months (p = 0.069). CONCLUSION: Presence of septal VT substrate in patients with a structural heart disease or coronary artery disease is common. Acute success of VT catheter ablation was significantly higher and mid-term success tended to be higher in patients without a septal substrate.

Protective role of ErbB3 signaling in myeloid cells during adaptation to cardiac pressure overload.

BACKGROUND: Myeloid cells play an important role in a wide variety of cardiovascular disorders, including both ischemic and non-ischemic cardiomyopathies. Neuregulin-1 (NRG-1)/ErbB signaling has recently emerged as an important factor contributing to the control of inflammatory activation of myeloid cells after an ischemic injury. However, the role of ErbB signaling in myeloid cells in non-ischemic cardiomyopathy is not fully understood. This study investigated the role of ErbB3 receptors in the regulation of early adaptive response using a mouse model of transverse aortic constriction (TAC) for non-ischemic cardiomyopathy. METHODS AND RESULTS: TAC surgery was performed in groups of age- and sex-matched myeloid cell-specific ErbB3-deficient mice (ErbB3MyeKO) and control animals (ErbB3MyeWT). The number of cardiac CD45 immune cells, CD11b myeloid cells, Ly6G neutrophils, and Ly6C monocytes was determined using flow cytometric analysis. Five days after TAC, survival was dramatically reduced in male but not female ErbB3MyeKO mice or control animals. The examination of lung weight to body weight ratio suggested that acute pulmonary edema was present in ErbB3MyeKO male mice after TAC. To determine the cellular and molecular mechanisms involved in the increased mortality in ErbB3MyeKO male mice, cardiac cell populations were examined at day 3 post-TAC using flow cytometry. Myeloid cells accumulated in control but not in ErbB3MyeKO male mouse hearts. This was accompanied by increased proliferation of Sca-1 positive non-immune cells (endothelial cells and fibroblasts) in control but not ErbB3MyeKO male mice. No significant differences in intramyocardial accumulation of myeloid cells or proliferation of Sca-1 cells were found between the groups of ErbB3MyeKO and ErbB3MyeWT female mice. An antibody-based protein array analysis revealed that IGF-1 expression was significantly downregulated only in ErbB3MyeKO mice hearts compared to control animals after TAC. CONCLUSION: Our data demonstrate the crucial role of myeloid cell-specific ErbB3 signaling in the cardiac accumulation of myeloid cells, which contributes to the activation of cardiac endothelial cells and fibroblasts and development of an early adaptive response to cardiac pressure overload in male mice.

Implications of multiple late gadolinium enhancement lesions on the frequency of left ventricular reverse remodeling and prognosis in patients with non-ischemic cardiomyopathy.

BACKGROUND: Non-ischemic cardiomyopathy (NICM) is a heterogeneous disease, and its prognosis varies. Although late gadolinium enhancement (LGE)-cardiovascular magnetic resonance (CMR) demonstrates a linear pattern in the mid-wall of the septum or multiple LGE lesions in patients with NICM, the therapeutic response and prognosis of multiple LGE lesions have not been elucidated. This study aimed to investigate the frequency of left ventricular (LV) reverse remodeling (LVRR) and prognosis in patients with NICM who have multiple LGE lesions. METHODS: This single-center retrospective study included 101 consecutive patients with NICM who were divided into 3 groups according to LGE-CMR results: patients without LGE (no LGE group = 48 patients), patients with a typical mid-wall LGE pattern (n = 29 patients), and patients with multiple LGE lesions (n = 24 patients). LVRR was defined as an increase in LV ejection fraction (LVEF) ≥ 10 % and a final value of LVEF > 35 %, which was accompanied by a decrease in LV end-systolic volume ≥ 15 % at 12-month follow-up using echocardiography. The frequency of composite cardiac events, defined as sudden cardiac death (SCD), aborted SCD (non-fatal ventricular fibrillation, sustained ventricular tachycardia, or adequate implantable cardioverter-defibrillator therapies), and heart failure death or hospitalization for worsening heart failure, were summarized and compared between the groups. RESULTS: Among the 3 groups, the frequency of LVRR was significantly lower in the multiple lesions group than in the no LGE and mid-wall groups (no LGE vs. mid-wall vs. multiple lesions: 49 % vs. 52 % vs. 19 %, p = 0.03). There were 24 composite cardiac events among the patients: 2 in patients without LGE (hospitalization for worsening heart failure; 2), 7 in patients of the mid-wall group (SCD; 1, aborted SCD; 1 and hospitalization for worsening heart failure; 5), and 15 in patients of the multiple lesions group (SCD; 1, aborted SCD; 8 and hospitalization for worsening heart failure; 6). The multiple LGE lesions was an independent predictor of composite cardiac events (hazard ratio: 11.40 [95 % confidence intervals: 1.49-92.01], p = 0.020). CONCLUSIONS: Patients with multiple LGE lesions have a higher risk of cardiac events and poorer LVRR. The LGE pattern may be useful for an improved risk stratification in patients with NICM.

Right ventricular free wall strain in acutely decompensated heart failure patients with ischemic and non-ischemic cardiomyopathy.

AIMS: Right ventricular (RV) dysfunction is a predictor of adverse outcomes among patients with HF with reduced ejection fraction (HFrEF); however, differences in RV parameters in HFrEF patients with ischemic (ICM) and non-ischemic cardiomyopathies (NICM) are not well understood. We investigated echocardiographic characteristics, including RV strain, in patients with acute decompensated heart failure (ADHF) and compared patients with ICM and NICM etiology. METHODS: Consecutive patients who presented with ADHF and NYHA class III-IV were prospectively enrolled if they had LVEF < 40% and history of ICM or NICM. All patients underwent clinical exam, laboratory evaluation and 2-D echocardiographic assessment of the left ventricular (LV) and RV function, LV and RV global longitudinal strain (LVGLS, RVGLS), and RV free wall strain (RVfwLS). RESULTS: Of 84 patients, 44 had ICM and 40 NICM. The groups had similar blood pressure, NT-proBNP, and echocardiographic parameters of LV function including LVGLS. Absolute RVGLS values were lower than RVfwLS values in both groups. Patients with NICM had significantly lower RVfwLS, but not RVGLS, compared to patients with ICM (-13% to -17%, p = 0.006). Similar differences in RVfwLS were seen in patients in NYHA class III (NICM vs ICM: -13% and -17%, respectively, 95% CI: -8.5 to -.5) and NYHA class IV (NICM vs ICM: -13.8% and -17%, respectively, 95% CI: -6.4 to -.59). CONCLUSION: Among patients hospitalized with ADHF, patients with nonischemic etiology compared with the patients with ICM, have more severe RV dysfunction measured by RVfwLS, despite similar extent of LV impairment and the same functional limitation class.

CMR-Based Risk Stratification of Sudden Cardiac Death and Use of Implantable Cardioverter-Defibrillator in Non-Ischemic Cardiomyopathy.

Non-ischemic cardiomyopathy (NICM) is one of the most important entities for arrhythmias and sudden cardiac death (SCD). Previous studies suggest a lower benefit of implantable cardioverter-defibrillator (ICD) therapy in patients with NICM as compared to ischemic cardiomyopathy (ICM). Nevertheless, current guidelines do not differentiate between the two subgroups in recommending ICD implantation. Hence, risk stratification is required to determine the subgroup of patients with NICM who will likely benefit from ICD therapy. Various predictors have been proposed, among others genetic mutations, left-ventricular ejection fraction (LVEF), left-ventricular end-diastolic volume (LVEDD), and T-wave alternans (TWA). In addition to these parameters, cardiovascular magnetic resonance imaging (CMR) has the potential to further improve risk stratification. CMR allows the comprehensive analysis of cardiac function and myocardial tissue composition. A range of CMR parameters have been associated with SCD. Applicable examples include late gadolinium enhancement (LGE), T1 relaxation times, and myocardial strain. This review evaluates the epidemiological aspects of SCD in NICM, the role of CMR for risk stratification, and resulting indications for ICD implantation.

Biventricular Thrombi Associated with Cardiac Systolic Dysfunction and Disseminated Intravascular Coagulation from Heat Stroke.

A 58-year-old man with non-ischemic cardiomyopathy visited a hospital once a month after his first hospitalization for heart failure. Three months later, he presented with consciousness impairment and heat stroke. Blood tests showed multiple organ failure, and echocardiography revealed biventricular thrombi. After admission, intensive care was provided, and anticoagulation therapy was initiated. The echocardiographic findings in the third week confirmed the complete disappearance of thrombi. Biventricular thrombi associated with disseminated intravascular coagulation from heat stroke is rare. We report the case of a patient who was treated with anticoagulation therapy only, without surgical intervention.

Subclinical Cardiomyopathy in Miyoshi Myopathy Detected by Late Gadolinium Enhancement Cardiac Magnetic Resonance Imaging.

Dysferlin is a sarcolemmal protein present in muscle cells. It is responsible for muscle membrane repair. Dysferlin gene (DYSF) mutation, resulting in deficiency in this protein, is termed dysferlinopathy. Clinically, it manifests as early adulthood onset of muscle weakness with markedly elevated creatine kinase levels. The main phenotypes are limb-girdle muscular dystrophy type 2B (LGMD2B), affecting proximal muscles, and Miyoshi myopathy (MM), affecting distal muscles. Dysferlin is also present in cardiomyocytes, and case reports have emerged of cardiac abnormalities in dysferlinopathy. While routine methods of cardiac screening, namely, electrocardiography or echocardiography, are convenient and noninvasive, they often exhibit insufficient diagnostic sensitivity for detecting subclinical cardiac remodeling during early stages of cardiomyopathy. Cardiac magnetic resonance imaging though can provide accurate assessment of cardiac chamber sizes and function. With gadolinium administration, it can also detect areas of myocardial scarring and fibrosis. Early diagnosis of neuromuscular disease-related cardiomyopathy is of clinical significance, as appropriate treatment can retard myocardial fibrosis, delaying cardiomyopathy progression. We present a case of a patient with MM incidentally diagnosed with concomitant cardiomyopathy.

Intra-Left Ventricular Hemodynamics Assessed with 4D Flow Magnetic Resonance Imaging in Patients with Left Ventricular Thrombus.

Left ventricular thrombus (LVT) has been identified to be crucial in patients with reduced ejection fraction (EF). Three-dimensional cine phase-contrast magnetic resonance imaging (4D flow MRI) can visualize the intra-LV vortex during diastole and quantify the maximum flow velocity (Vmax) at the apex. In this study, we investigated whether the change in the intra-LV vortex was associated with the presence of LVT in patients with cardiac disease.In total, 36 patients (63.5 ± 11.9 years, 28 men, 12/24 with/without LVT) with diffuse LV dysfunction underwent 4D flow MRI. The relative vortex area using streamline images and Vmax of blood flow toward the apex at the apical left ventricle were evaluated. The correlation between the relative vortex area and Vmax was assessed using Pearson's correlation coefficient. The ability to detect LVT was evaluated using the area under the curve (AUC) of the receiver operating characteristic.The relative vortex area was found to be smaller (27 ± 10% versus 45 ± 11%, P = 0.000026), whereas Vmax at the apical left ventricle was lower (19.1 ± 4.4 cm/second versus 27.4 ± 8.9 cm/second, P = 0.0006) in patients with LVT. Vmax at the apical left ventricle demonstrated significant correlations with the relative vortex area (r = 0.43, P = 0.01) and relative transverse length of the vortex (r = 0.45, P = 0.007). The AUC was 0.91 for the relative vortex area, whereas it was 0.80 for Vmax in the apical left ventricle.A smaller LV vortex and lower flow velocity at the LV apex were associated with LVT in patients with reduced EF.

A novel risk model for mortality and hospitalization following cardiac resynchronization therapy in patients with non-ischemic cardiomyopathy: the alpha-score.

BACKGROUND: Non-ischemic cardiomyopathy (NICM) has been associated with a better left ventricle reverse remodeling response and improved clinical outcomes after cardiac resynchronization therapy (CRT). The aims of our study were to identify the predictors of mortality and heart failure hospitalization in patients treated with CRT and design a risk score for prognosis. METHODS: A cohort of 422 consecutive NICM patients with CRT was retrospectively enrolled between January 2010 and December 2017. The primary endpoint was all-cause mortality and heart transplantation. RESULTS: In a multivariate analysis, the predictors of all-cause death were left atrial diameter [Hazard ratio (HR): 1.056, 95% confidence interval (CI): 1.020-1.093, P = 0.002]; non-left bundle branch block [HR: 1.793, 95% CI: 1.131-2.844, P = 0.013]; high sensitivity C-reactive protein [HR: 1.081, 95% CI: 1.029-1.134 P = 0.002]; and N-terminal pro-B-type natriuretic peptide [HR: 1.018, 95% CI: 1.007-1.030, P = 0.002]; and New York Heart Association class IV [HR: 1.018, 95% CI: 1.007-1.030, P = 0.002]. The Alpha-score (Atrial diameter, non-LBBB, Pro-BNP, Hs-CRP, NYHA class IV) was derived from each independent risk factor. The novel score had good calibration (Hosmer-Lemeshow test, P > 0.05) and discrimination for both primary endpoints [c-statistics: 0.749 (95% CI: 0.694-0.804), P < 0.001] or heart failure hospitalization [c-statistics: 0.692 (95% CI: 0.639-0.745), P < 0.001]. CONCLUSION: The Alpha-score may enable improved discrimination and accurate prediction of long-term outcomes among NICM patients with CRT.