Nonischemic Dilated Cardiomyopathy Research Articles

Left ventricle mechanical dispersion is a new universal marker of malignant ventricular tachyarrhythmias in patients with structural heart disease

Aim. To evaluate the possibility to use parameters of global, segmental longitudinal left ventricle (LV) strain and LV mechanical dispersion (MD) as new possible markers of malignant ventricular tachyarrhythmias (MVT) in patients with structural heart disease and reduced and intermediate LV ejection fraction (EF).Methods. The study included 113 patients (105 male, age 66 [59;73] year) with ischemic (n=89) or non-ischemic (n=24) dilated cardiomyopathy and LVEF<50%, implanted cardioverter-defibrillators, cardiac resynchronization therapy devices-defibrillators, pacemakers or documented MVT. All patients underwent transthoracic echocardiography with an assessment of speckle-tracking echocardiography parameters (global and segmental longitudinal LV strain, LV MD). Comparative univariate and ROC-analyses were performed between patients with and without MVT separately for patients with LVEF <35% (n=60) and LVEF 36-50% (n=53) LVEF.Results. The group of patients with LVEF ≤35% with MVT (n=30) was characterized by lower values of longitudinal strain of the basal segment of the LV septum wall (-5[-6,5;-0,5] vs -6,8[-11;-4.4], р=0,01, respectively) and the middle segment of the LV inferior wall (-3[-6;2] vs -6[-9;-1,5], р=0,04, respectively). The group of patients with LVEF 36-50% with MVT (n=33) was distinguished by a large value of LV end-diastolic volume (166,5[146,3;193] vs 156[133,8;165,5], р=0,04, respectively). The greater values of LV MD were noted in both groups of patients with MVT. The optimal cut-off value of LV MD was 120 ms (area under the ROC curve 0.817, sensitivity-73,3%, specificity-80%) for patients with LVEF≤35%, and 90 ms (area under the ROC curve 0.761, sensitivity-72.7%, specificity-75%) for patients with LVEF 36-50%.Conclusion. Speckle-tracking echocardiography parameters may serve as additional markers of increased risk of MVT in patients with structural heart disease and impaired LVEF. Only LV MD is useful for risk stratification of MVT in patients with either reduced or intermediate LVEF.

FRI506 Severe Dilated Cardiomyopathy As First Manifestation Of Thyrotoxicosis- A Case Report

Abstract Disclosure: Z. Saeed: None. C. Labib: None. W. Althunibat: None. M. Varatharajah: None. Introduction: Hyperthyroidism is a common metabolic disorder with prominent cardiovascular manifestations ranging from palpitations, angina-like chest pain, exercise intolerance and dyspnea to high-output heart failure and dilated cardiomyopathy. Approximately 6% of patients with hyperthyroidism/thyrotoxicosis develop symptoms of heart failure and less than 1% develop dilated cardiomyopathy with reduced left ventricular systolic function. Here we present a case of a 55-year-old man who presented with symptoms of heart failure and was diagnosed with dilated cardiomyopathy due to thyrotoxicosis. CASE PRESENTATION: 55-year-old man with a past medical history of vitiligo and cirrhosis presented to the emergency department with complaint of lower extremity swelling, cough, dyspnea on exertion, chest discomfort, palpitations and lethargy for two weeks. He also had a weight gain of 14 lbs. in this period. In the emergency department he had a heart rate of 118 beats/minute, physical exam revealed bilateral fine hand tremors, bibasilar crackles and a 2/6 holosystolic murmur on chest auscultation as well as hyperactive deep tendon reflexes. EKG showed atrial fibrillation with a rate of 184 beats/min. Initial chest imaging showed cardiomegaly, perihilar congestive changes and small right pleural effusion. CT abdomen & pelvis demonstrated moderate volume ascites. Lab work showed a TSH of 0.00 mIU/L (0.5-5.0. mIU/L), T4 Free 5.3 ng/dL (0.9-2.3 ng/dL), Troponin T 36 ng/L, pro BNP 3150 pg/ml. He was given furosemide, propranolol, hydrocortisone, a bolus of diltiazem and was started on diltiazem and heparin drip and admitted to the hospital. Endocrinology was consulted and he was started on methimazole and methylprednisolone. Transthoracic echocardiogram showed an ejection fraction of 15%. Thyroid ultrasound was negative for any nodules or mass. Cardiac catheterization was done which revealed moderate pulmonary hypertension, non-ischemic dilated cardiomyopathy. Patient was discharged home on methimazole and guideline directed medical therapy for heart failure. He followed outpatient with Endocrinology and Cardiology. Six months after his hospital admission, repeat TSH was 1.71 mIU/L after treatment. Repeat echocardiography showed an improvement in ejection fraction from 15% to 35-40%. DISCUSSION: Prolonged untreated hyperthyroidism leads to high output heart failure characterized by an increase in heart rate, cardiac contractility and cardiac output which in some cases progresses to dilated cardiomyopathy with impaired left ventricular systolic function. Heart failure with reduced ejection fraction is a frequently encountered problem, most commonly due to ischemic cardiomyopathy. Thyrotoxicosis is one of the rare and reversible causes of nonischemic dilated cardiomyopathy which must be promptly diagnosed and treated in order to prevent life threatening complications. Presentation: Friday, June 16, 2023

Associations between Oral Glucose-Lowering Agents and Increased Risk for Life-Threatening Arrhythmias in Patients with Type 2 Diabetes Mellitus-A Literature Review.

Background and Objectives: The relationship between type 2 diabetes mellitus (T2DM) and cardiovascular (CV) morbidity and mortality is well-established. Ventricular arrhythmias (VA) are frequently diagnosed in patients with T2DM, especially in those with associated coronary syndrome, non-ischemic dilated cardiomyopathy (NIDCM), and heart failure (HF). In these patients, VA and sudden cardiac arrest (SCA) are considered responsible for more than 50% of CV deaths. Newly developed glucose-lowering agents (GLA) seem not only to ameliorate CV morbidity and mortality, but also to reduce the risk of VA and SCA. Materials and Methods: We researched the medical literature on Pub-Med, Clarivate, and Google Scholar for original articles published in the last five years that debated the possible effects of various GLA on ventricular arrhythmias. Results: We identified nineteen original articles, nine of them debating the antiarrhythmic effects of sodium-glucose cotransporter-2 inhibitors (SGLT2i); Conclusions: The results concerning the impact of various GLA on VA/SCA were heterogeneous depending on the pharmacological class studied, with some of them having neutral, positive, or negative effects. Although it appears that SGLT2i reduces the prevalence of atrial fibrillation and SCA, their effect on VA is not conclusive.

Potential Prognostic Relevance of Left-Ventricular Global Longitudinal Strain and of the Summation of the Mitral and Tricuspid Regurgitation Volume in Patients with Non-Ischemic Dilated Cardiomyopathy.

The aim of this pilot study was to determine the potential prognostic relevance of novel multidirectional myocardial and volumetric echocardiographic parameters in patients with non-ischemic dilated cardiomyopathy (NIDCM). Multidirectional myocardial parameters (longitudinal, radial, and circumferential left-ventricular (LV) strain using speckle tracking echocardiography) and a new volumetric parameter (the sum of the mitral and tricuspid regurgitation volume (mitral-tricuspid regurgitation volume) were assessed. The cardiovascular (CV) outcome was a composite of cardiac death and hospitalization for heart failure (HF) at 1 year. Approximately 102 patients were included in this pilot study. The mean LV ejection fraction (LVEF) was 28.4 ± 8.9%. During a follow-up of 1 year, the CV outcome occurred in 39 patients (10 HF deaths, and 36 hospitalizations for HF). The LV global longitudinal systolic strain (GLS) and mitral-tricuspid regurgitation volume were the main parameters that were seen to be significantly altered in the comparison of patients with events vs. those without events (GLS (absolute values) 7.4 ± 2.7% vs. 10.3 ± 2.6%; mitral-tricuspid regurgitation volume 61.1 ± 20.4 mL vs. 40.9 ± 22.9 mL, respectively; p-value < 0.01). In line with these findings, in a multivariate continuous logistic regression analysis, the GLS and mitral-tricuspid regurgitation volume were the main parameters associated with worse CV outcomes (GLS: OR 0.77 (95%CI 0.65-0.92); mitral-tricuspid regurgitation volume OR 1.09 (95%CI 1.01-1.25)), whereas the radial and circumferential LV global strain and mitral regurgitation volume and tricuspid regurgitation volume were not linked to the CV outcome. Furthermore, in a receiver operating characteristic curve analysis, a GLS cutoff of <7.5% and mitral-tricuspid regurgitation volume > 60 mL were the identified values for the parameters associated with worse CV outcomes. The findings of this pilot study suggest that the GLS and a novel volumetric parameter (the sum of the mitral and tricuspid regurgitation volume) are linked to worse CV outcomes in patients with non-ischemic dilated cardiomyopathy. Hence, these promising results warrant further validation in larger studies.

Care and Monitoring of Pregnant Patients With Left Ventricular Assist Devices.

Cardiovascular disease is one of the leading causes of maternal mortality in the United States. Although still rare, pregnancy in patients with left ventricular assist devices (LVADs) is becoming more common. Typical indications for the use of LVADs in reproductive-aged females include ischemic cardiomyopathy, nonischemic (familial) dilated cardiomyopathy, peripartum cardiomyopathy, and some forms of myocarditis. An LVAD drains blood through a cannula placed into the apex of the left ventricle and then returns it to the proximal aorta bypassing the aortic valve allowing hemodynamic support in parallel with the native circulation. The physiologic changes associated with pregnancy, mainly increased blood volume and hypercoagulability, may adversely affect patients with LVADs, leading to many experts recommending against pregnancy. Maternal-fetal medicine specialists should have a central role within a multidisciplinary team required to provide optimal care for this high-risk group of patients.

Right ventricular systolic function and mechanical dyssynchrony in ischemic or non-ischemic dilated cardiomyopathy: A speckle-tracking study.

This study assessed RV dyssynchrony (irrespective to QRS duration) and RV systolic function in non-ischemic dilated cardiomyopathy (NIDCM) versus ischemic dilated cardiomyopathy (IDCM) patients by using different echo-Doppler modalities. Eighty-five cases (48 patients with DCM [whether ischemic or non-ischemic] and 37 age-matched healthy controls) were studied. Conventional echo-Doppler study, tissue Doppler (TDI), and speckle tracking (STE) were carried out to measure LV and RV systolic function. Time-to-peak negative longitudinal strain at the four RV sites were assessed by TDI derived strain and 2D speckle tracking. Patients with DCM (whether ischemic or non-ischemic) had significantly lower fractional area change, RV tricuspid annular systolic velocity (p<.001 for both), tricuspid annular plane systolic excursion (p=.01), RV-GLS whether TDI or 2D derived (p<.001). Twenty-nine patients (60%) showed right intraventricular delay (RV4SD>60ms). The RV-dyssynchrony index was negatively correlated to %FAC (r=-.362, p=.01), RV Sm (r=-.312, p=.04), and 2D-RV GLS (r=-.305, p=.05). Insignificant higher RV-dysynchrony index was detected in NIDCM compared to IDCM group; however, the basal septal segment was significantly delayed in dilated group. More impaired RV systolic function was detected in ischemic group. 2D STE and TDI showed a significant correlation in the assessment of the right-intraventricular delay (p=.001). Right-intraventricular dyssynchrony are detectable in patients with dilated cardiomyopathy (whether ischemic or non-ischemic) with a higher statistically insignificant value in non-ischemic group by using tissue Doppler imaging and 2D speckle tracking. More impairment of the RV systolic function was noticed in the ischemic group. Impaired RV systolic function was associated with right intraventricular delay.

Prognostic implication of liver fibrosis scores in patients with non-ischemic dilated cardiomyopathy.

Liver fibrosis was associated with adverse outcomes in various cardiovascular diseases. The current risk stratification of non-ischemic dilated cardiomyopathy (NIDCM) still largely depends on the left ventricular ejection fraction (LVEF). At present, the relationship between liver fibrosis and prognosis in patients with NIDCM remains blank. A total of 433 NIDCM patients were analysed in this study. Liver fibrosis was assessed by three liver fibrosis scores (LFS), including aspartate aminotransferase to platelet ratio index (APRI), aspartate aminotransferase/alanine aminotransferase ratio (AST/ALT ratio), and gamma-glutamyltransferase to platelet ratio (GPR). The primary endpoint was defined as all-cause mortality or heart transplantation (ACM/HTx). During a median follow-up period of 1.7 years, 140 ACM/HTx events occurred. Positive associations were observed between LFS and ACM/HTx. Patients with elevated APRI, AST/ALT ratio, and GPR scores exhibited increased ACM/HTx (all P < 0.05). Intermediate-to-high APRI [hazard ratio (HR) 1.66, 95%CI 1.06-2.61, P = 0.027], AST/ALT ratio (HR 1.59, 95%CI 1.07-2.36, P = 0.021), and GPR (HR 1.64, 95%CI 1.11-2.42, P = 0.013) were independently associated with increased risk of ACM/HTx, even after adjusting for LVEF and other covariates. The positive relationship remains consistent across different subgroups, including those with diabetes and obesity. Elevated liver fibrosis scores were associated with a worse outcome beyond LVEF in patients with NIDCM, which may provide additional prognostic value in the management of NIDCM.

Clonal Hematopoiesis Has Prognostic Value in Dilated Cardiomyopathy Independent of Age and Clone Size

BackgroundClonal hematopoiesis (CH) gives rise to mutated leukocyte clones that induce cardiovascular inflammation and thereby impact the disease course in atherosclerosis and ischemic heart failure. CH of indeterminate potential refers to a variant allele frequency (VAF) (a marker for clone size) in blood of ≥2%. The impact of CH clones—including small clone sizes (VAF <0.5%)—in nonischemic dilated cardiomyopathy (DCM) remains largely undetermined. ObjectivesThe authors sought to establish the prognostic impact of CH in DCM including small clones. MethodsCH is determined using an ultrasensitive single-molecule molecular inversion probe technique that allows detection of clones down to a VAF of 0.01%. Cardiac death and all-cause mortality were analyzed using receiver-operating characteristic curve–optimized VAF cutoff values. ResultsA total of 520 DCM patients have been included. A total of 109 patients (21%) had CH driver mutations, of which 45 had a VAF of ≥2% and 31 <0.5%. The median follow-up duration was 6.5 years (IQR: 4.7-9.7 years). DCM patients with CH have a higher risk of cardiac death (HR: 2.33 using a VAF cutoff of 0.36%, 95% CI: 1.24-4.40) and all-cause mortality (HR: 1.72 using a VAF cutoff of 0.06%, 95% CI: 1.10-2.69), independent of age, sex, left ventricular ejection fraction, and NYHA functional classification. ConclusionsCH predicts cardiac death and all-cause mortality in DCM patients with optimal thresholds for clone size of 0.36% and 0.06%, respectively. Therefore, CH is prognostically relevant, independent of clone size in patients with DCM.

Impact of late gadolinium-enhanced cardiac MRI on arrhythmic and mortality outcomes in nonischemic dilated cardiomyopathy: updated systematic review and meta-analysis

Risk stratification based mainly on the impairment of left ventricular ejection fraction has limited performance in patients with nonischemic dilated cardiomyopathy (NIDCM). Evidence is rapidly growing for the impact of myocardial scar identified by late gadolinium enhancement (LGE) cardiac magnetic resonance imaging (CMR) on cardiovascular events. We aim to assess the prognostic value of LGE on long-term arrhythmic and mortality outcomes in patients with NIDCM. PubMed, Scopus, and Cochrane databases were searched from inception to January 21, 2022. Studies that included disease-specific subpopulations of NIDCM were excluded. Data were independently extracted and combined via random-effects meta-analysis using a generic inverse-variance strategy. Data from 60 studies comprising 15,217 patients were analyzed with a 3-year median follow-up. The presence of LGE was associated with major ventricular arrhythmic events (pooled OR: 3.99; 95% CI 3.08, 5.16), all-cause mortality (pooled OR: 2.14; 95% CI 1.81, 2.52), cardiovascular mortality (pooled OR 2.83; 95% CI 2.23, 3.60), and heart failure hospitalization (pooled OR: 2.53; 95% CI 1.78, 3.59). Real-world evidence suggests that the presence of LGE on CMR was a strong predictor of adverse long-term outcomes in patients with NIDCM. Scar assessment should be incorporated as a primary determinant in the patient selection criteria for primary prophylactic implantable cardioverter-defibrillator placement.

Late gadolinium enhancement distribution patterns in non-ischaemic dilated cardiomyopathy: genotype-phenotype correlation.

Late gadolinium enhancement (LGE) is frequently found in patients with dilated cardiomyopathy (DCM); there is little information about its frequency and distribution pattern according to the underlying genetic substrate. We sought to describe LGE patterns according to genotypes and to analyse the risk of major ventricular arrhythmias (MVA) according to patterns. Cardiac magnetic resonance findings and LGE distribution according to genetics were performed in a cohort of 600 DCM patients followed at 20 Spanish centres. After exclusion of individuals with multiple causative gene variants or with variants in infrequent DCM-causing genes, 577 patients (34% females, mean age 53.5 years, left ventricular ejection fraction 36.9 ± 13.9%) conformed to the final cohort. A causative genetic variant was identified in 219 (38%) patients, and 147 (25.5%) had LGE. Significant differences were found comparing LGE patterns between genes (P < 0.001). LGE was absent or rare in patients with variants in TNNT2, RBM20, and MYH7 (0, 5, and 20%, respectively). Patients with variants in DMD, DSP, and FLNC showed a predominance of LGE subepicardial patterns (50, 41, and 18%, respectively), whereas patients with variants in TTN, BAG3, LMNA, and MYBPC3 showed unspecific LGE patterns. The genetic yield differed according to LGE patterns. Patients with subepicardial, lineal midwall, transmural, and right ventricular insertion points or with combinations of LGE patterns showed an increased risk of MVA compared with patients without LGE. LGE patterns in DCM have a specific distribution according to the affected gene. Certain LGE patterns are associated with an increased risk of MVA and with an increased yield of genetic testing.

Serum albumin and prognosis in elderly patients with nonischemic dilated cardiomyopathy.

Hypoalbuminemia was extensively used to diagnose malnutrition in older adults. Malnutrition was associated with mortality in elderly patients with cardiovascular diseases. The relationship between hypoalbuminemia and clinical outcomes in elderly patients with nonischemic dilated cardiomyopathy (NIDCM) remains unknown. A total of 1058 consecutive patients with NIDCM (age ≥60 years) were retrospectively enrolled from January 2010 to December 2019. Univariate and multivariate analyses were performed to assess the association of hypoalbuminemia with clinical outcomes. Patients with hypoalbuminemia were older (69.29 ± 6.67 vs. 67.61 ± 5.90 years, P < 0.001) and had higher prevalence of in-hospital and long-term death than those without (6.9 vs. 1.7%, 50.7 vs. 35.2%, P < 0.001). Logistic regression analysis showed that hypoalbuminemia was significantly related to in-hospital death [odds ratio (OR): 4.334, 95% confidence interval (CI): 2.185-8.597, P < 0.001]. Kaplan-Meier survival analysis showed that patients with hypoalbuminemia had worse prognosis than those with nonhypoalbuminemia (log-rank χ2 28.96, P < 0.001). After adjusting for age, serum creatinine, HDL-C, AST/ALT hypoalbuminemia, LVEF and diabetes, hypoalbuminemia remained an independent predictor for long-term death (hazard ratio 1.322, 95% CI 0.046-1.670, P = 0.019). Hypoalbuminemia was associated with increased risk of in-hospital and long-term mortality in elderly patients with NIDCM.

Abstract P3079: Integrating Existing Cardiac Single-cell RNA Sequencing Datasets To Uncover The Role Of B-cells In Heart Disease

Introduction: The role of B-cells in human heart failure remains unclear. To address this knowledge gap, we analyzed publicly available datasets of scRNAseq and snRNAseq from patients with various forms of cardiomyopathy. Methods and Results: We used two public datasets including single cells or single nuclei RNAseq data collected from the myocardium of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), dilated cardiomyopathy (DCM), idiopathic cardiac diseases (ICD), or healthy controls. The first dataset comprised 30 healthy controls and 22 non-ischemic DCM; the second contained 18 controls and 61 with DCM, ARVC, or ICD. Data were normalized and scaled, a UMAP plot was generated, and cells were classified using ScType. B-cells were merged into a single matrix, and the final dataset contained 1100 cells from 32 healthy controls, 59 DCM, and 8 ARVC patients. Four different B-cell clusters were identified. The top 100 biomarkers from each cluster were used to identify B-cell subtypes. Cluster 1 was identified as transitional splenic B-cells; Cluster 2 as plasma cells; Cluster 3 as follicular splenic B-cells; Cluster 4, present only in DCM, as immature B cells. Ingenuity Pathway Analysis (IPA) of the genes enriched in each cluster highlighted that different B cell clusters displayed distinct biological activity, ranging from phagosome formation to B cell receptor signaling and cytokine production. Differentially expressed gene analysis comparing healthy controls to DCM and ARVC showed both similar and disease-specific gene expression patterns in the various B cell clusters. For instance, cluster 2 showed upregulation of oxidative phosphorylation in both DCM and ARVC. On the other hand, DCM cluster 2 showed downregulation of CD40 signaling, while in ARVC the same cluster showed downregulation of IL-6 signaling (Z score &gt;2, p&lt;0.05). Discussion: Our findings suggest that human heart failure is associated with perturbations in B cell gene expression, with both shared and disease-specific features. This data supports the notion that B cells likely play a role in human cardiomyopathy. Further, this data suggests that B-cells play distinct roles in disparate forms of cardiac disease.

Quantitative Profiling of Serum Carnitines Facilitates the Etiology Diagnosis and Prognosis Prediction in Heart Failure.

The perturbation of fatty acid metabolism in heart failure (HF) has been a critical issue. It is unclear whether the amounts of circulating carnitines will benefit primary etiology diagnosis and prognostic prediction in HF. This study was designed to assess the diagnostic and prognostic values of serum carnitine profiles between ischemic and non-ischemic derived heart failure. HF patients (non-ischemic dilated cardiomyopathy: DCM-HF, n = 98; ischemic heart disease: IHD-HF, n = 63) and control individuals (n = 48) were enrolled consecutively. The serum carnitines were quantitatively measured using the UHPLC-MS/MS method. All patients underwent a median follow-up of 28.3 months. Multivariate Cox regression analysis was performed during the prognosis evaluation. Amongst 25 carnitines measured, all of them were increased in HF patients, and 20 acylcarnitines were associated with HF diagnosis independently. Seven acylcarnitines were confirmed to increase the probability of DCM diagnosis independently. The addition of isobutyryl-L-carnitine and stearoyl-L-carnitine to conventional clinical factors significantly improved the area under the receiver operating characteristic curve (ROC) from 0.771 to 0.832 (p = 0.023) for DCM-HF diagnosis (calibration test for the composite model: Hosmer-Lemeshow χ2 = 7.376, p = 0.497 > 0.05). Using a multivariate COX survival analysis adjusted with clinical factors simultaneously, oleoyl L-carnitine >300 nmol/L (HR = 2.364, 95% CI = 1.122-4.976, p = 0.024) and isovaleryl-L-carnitine <100 nmol/L (HR = 2.108, 95% CI = 1.091-4.074, p = 0.026) increased the prediction of all-cause mortality independently, while linoleoyl-L-carnitine >420 nmol/L, succinyl carnitine >60 nmol/L and isovaleryl-L-carnitine <100 nmol/L increased the risk of HF rehospitalization independently. Serum carnitines could not only serve as diagnostic and predictive biomarkers in HF but also benefit the identification of HF primary etiology and prognosis.

Different expression and prognostic value of troponin in ischemic cardiomyopathy and non-ischemic dilated cardiomyopathy

BackgroundEarly risk stratification of patients with ischemic cardiomyopathy (ICM) and non-ischemic dilated cardiomyopathy (NIDCM) may be beneficial for therapies.MethodsWe retrospectively enrolled all patients admitted for acute heart failure (HF) between January 2019 and December 2021 in Zhongshan Hospital Fudan University, dividing them according to etiology (ICM or NIDCM). Cardiac troponin T (TNT) concentration was compared between two groups. Risk factors for positive TNT and in-hospital all-cause mortality were investigated with regression analysis.ResultsA total of 1525 HF patients were enrolled, including 571 ICM and 954 NIDCM. The TNT positive patients were not different between the two groups (41.3% in ICM group vs. 37.8% in NIDCM group, P = 0.215). However, the TNT value in ICM group were significantly higher than that in NIDCM group (0.025 (0.015–0.053) vs. 0.020 (0.014–0.041), P = 0.001). NT-proBNP was independently associated with TNT in both ICM and NIDCM group. Although the in-hospital all-cause mortality did not show much difference between the two groups (1.1% vs. 1.9%, P = 0.204), the NIDCM diagnosis was associated with reduced risk of mortality after multiple adjustments (OR 0.169, 95% CI 0.040–0.718, P = 0.016). Other independent risk factors included the level of NT-proBNP (OR 8.260, 95% CI 3.168–21.533, P < 0.001), TNT (OR 8.118, 95% CI 3.205–20.562, P < 0.001), and anemia (OR 0.954, 95% CI 0.931–0.978, P < 0.001). The predictive value of TNT and NT-proBNP for all-cause mortality was similar. However, the best cutoff values of TNT for mortality were different between ICM and NIDCM groups, which were 0.113 ng/mL and 0.048 ng/mL, respectively.ConclusionThe TNT level was higher in ICM patient than that in NIDCM patients. TNT was an independent risk factor for in-hospital all-cause mortality for both ICM and NIDCM patients, although the best cutoff value was higher in ICM patients.

NON ISCHEMIC CARDIOMYOPATHY – A CASE REPORT

Cardiomyopathies are a group of diseases characterized by the structural and functional abnormalities of the heart muscles in the absence of other illnesses that might be responsible for the observed myocardial anomaly. Hypertrophic and dilated cardiomyopathies are the most prevalent types. Rare types include restrictive cardiomyopathy and restrictive cardiomyopathy with arrhythmia. Dilated cardiomyopathy has a documented prevalence of 36 cases per 100,000 population in Europe and North America, and its yearly incidence varies between 5 and 7.9 cases per 100,000 population. Non-ischemic dilated cardiomyopathy, which causes heart failure, dramatically increases the global burden of cardiovascular diseases. For both men and women, acute dilated cardiomyopathy is mainly brought on by myocarditis. This case was present in Aga Khan University Hospital; and was selected to be reported. The diagnosis in the case scenario is non-ischemic cardiomyopathy associated with heart failure and pulmonary oedema.

Septal Midwall Late Gadolinium Enhancement in Ischemic Cardiomyopathy and Nonischemic Dilated Cardiomyopathy—Characteristics and Prognosis

Septal midwall late gadolinium enhancement (LGE) is a characteristic finding on cardiac magnetic resonance imaging (CMR) in nonischemic dilated cardiomyopathy (DCM) and is associated with adverse events. Its significance in ischemic cardiomyopathy (ICM) is unknown. With this multicenter observational study, we aimed to study the characteristics of septal midwall LGE and evaluate its prognostic value in ICM. A total of 1,084 patients with an impaired left ventricular (LV) ejection fraction (<50%) on LGE-CMR, either because of ICM (53%) or DCM, were included retrospectively. Septal midwall LGE was defined as midmyocardial stripe-like or patchy LGE in septal segments and was present in 10% of patients with ICM compared with 34% of patients with DCM (p<0.001). It was significantly associated with larger LV volumes and lower LV ejection fraction, irrespective of etiology. The primary endpoint was all-cause mortality and secondary endpoint was ventricular arrhythmias (VAs), including resuscitated cardiac arrest, sustained VA, and appropriate implantable cardioverter-defibrillator (ICD) therapy. During a median follow-up of 2.7years, we found a significant association between septal midwall LGE and mortality in patients with DCM (hazard ratio [HR] 1.92, p=0.03), but not in patients with ICM (HR 1.35, p=0.39). Risk of VAs was significantly higher in patients with septal midwall LGE on CMR, both in DCM (HR 2.80, p<0.01) and in ICM (HR 2.70, p<0.01). In conclusion, septal midwall LGE, typically seen in DCM, was also present in 10% of patients with ICM and was associated with increased LV dilation and worse function, irrespective of etiology. When present, septal midwall LGE was associated with adverse outcome.

Prediction of adverse outcomes in nonischemic dilated cardiomyopathy: A CMR-based nomogram

ObjectivesTo develop and validate a novel nomogram score to predict outcomes in patients with nonischemic dilated cardiomyopathy (NIDCM) over a long follow-up period. MethodsA total of 335 consecutive NIDCM patients who underwent cardiac magnetic resonance (CMR) imaging were retrospectively enrolled. Comprehensive clinical evaluation and imaging investigation were obtained, including measurements of late gadolinium enhancement (LGE) and feature tracking (FT) images. All patients were followed up for a composite endpoint of major adverse cardiac events (MACE) including all-cause mortality and heart transplantation. These patients were randomly divided into development and validation cohorts (7:3). ResultsMACE occurred in 87 (37.2%) out of 234 patients in the development cohort, and in 31 (30.7%) out of 101 patients in the validation cohort. Five variables including NYHA class III-IV, NT-proBNP, beta-blocker medication, LGE presence, and LV global longitudinal strain (GLS) were found to be significantly associated with MACE and were used for constructing the nomogram. The nomogram achieved good discrimination with C-indexes in development and validation cohorts respectively. The calibration curve for 1-, 3-, and 5-year survival probability also showed high coherence between the predicted and actual probability of MACE. Decision curve analysis identified the model was significantly better net benefit in predicting MACE. ConclusionA novel nomogram score of a predictive model that incorporates clinical factors and imaging features was constructed, which could be conveniently used to facilitate risk evaluation in patients with NIDCM.

Left ventricular torsion as a prognostic marker in patients with dilated cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Background Left ventricular (LV) torsion is the wringing motion of the ventricle around its long axis induced by contracting obliquely oriented myofibers in the LV wall and it predicts functional capacity more appropriately than LV ejection fraction (EF). Purpose This study was intended to evaluate reduced LV torsion as a potential marker of disease severity and a predictor of hospitalization for worsening heart failure in patients with non-ischemic dilated cardiomyopathy (DCM). Methods This was a single center prospective observational study wherein 127 patients with a diagnosis of non-ischemic DCM with a LVEF &amp;lt;50% were enrolled. In addition, 48 age and sex matched healthy individuals without any prior history of cardiovascular diseases and normal LVEF were enrolled as controls. All these patients underwent detailed clinical assessment including symptomatology, NYHA function class evaluation and routine blood investigations including CPK-MB levels. In addition, all enrolled subjects underwent 2D transthoracic echocardiography along with speckle tracking imaging to determine the LV global longitudinal strain (LV-GLS), LV twist and LV torsion. Based on the measurement of LV torsion, patients were stratified into two groups: preserved LV torsion (&amp;gt;0.59 degrees/cm) and reduced LV torsion (&amp;lt;0.59 degrees/cm). These patients were followed up for six months in terms of MACE events. Results Reduced LV torsion was reported in 52/127 (40.9%) patients with DCM. These patients had significantly lower LVEF, altered LV geometry (base-apical distance) and higher levels of CPK-MB. Additionally, patients with reduced LV torsion had a worsened functional class and a greater frequency of hospital admissions (34 vs 6; P&amp;lt;0.001) over 6 months of follow-up as compared to those with preserved LV torsion. Multivariate logistic regression reported reduced LV torsion to be an independent predictor of MACE events over six months of follow-up (OR:4.20; P=0.001). Conclusion Findings from our study suggested that LV torsion can serve as an additional prognostic marker in patients with non-ischemic DCM.

Prognostic value of left-ventricular hemodynamic forces by echocardiography in patients with non-ischemic dilated cardiomyopathy

Abstract Funding Acknowledgements Type of funding sources: None. Background Dilated cardiomyopathy (DCM) is a non-ischemic heart disease with reduced left-ventricular (LV) ejection fraction (LVEF) bearing high risk for heart failure (HF) and death (1). The evaluation of the LV by transthoracic echocardiography (TTE) is essential for diagnosis, prognosis, and follow-up, but conventional TTE parameters do not allow for proper risk assessment (2). Hemodynamic forces (HDF) by TTE may provide important information on LV mechanics (3), but their prognostic value in DCM is unknown. Purpose To test whether HDF provide prognostic value beyond conventional echocardiography parameters in DCM patients. Methods In this retrospective longitudinal study, we included patients diagnosed with DCM between 2015 and 2019 who underwent TTE and had coronary artery disease ruled out by coronary angiography. Patients were excluded from this analysis if at first available TTE were hospitalized or symptomatic for HF, had moderate-severe valvular heart disease, had atrial fibrillation during TTE evaluation, and if follow-up was &amp;lt;12 months. Major cardiovascular events (MACE) were considered as a composite of all-cause death, HF hospitalization, and need for ambulatory intravenous diuretic therapy (HF decompensation). LV HDF were analyzed on conventional TTE apical 2-, 3-, and 4-chamber view images with a prototype software (Medis Suite Qstrain, The Netherlands). HDF were decomposed into apex-base (HDFab) and lateral-septal (HDFls) components, and HDF angle (combination of both components, ranging from 0° to 90°) was computed (4). Results A total of 109 patients were included, but 12 patients were subsequently excluded for suboptimal TTE image quality. Thus, 97 patients were included in the study: 67 (69%) were males, mean age was 62±14 years, median follow-up was 4.2 [3.1–5.1] years, and mean LVEF was 39.2±8.6%. Mean HDFab, HDFls, and HDF angle were 5.73±1.94%, 1.65±0.53%, and 67.5±5.7°, respectively. MACE occurred in 19 patients (19.6%). Patients experiencing MACE had higher HDF angle (70.8±4.7 vs. 66.8±5.6°, p = 0.006), lower HDFls (1.31±0.41 vs. 1.71±0.53%, p = 0.003) but similar HDFab (5.4±1.7 vs. 5.8±2.0%, p = 0.393) compared with patients without MACE. On Cox univariate analysis age (HR 1.09 [95% CI 1.04–1.15], p&amp;lt;0.001) and HDF angle (HR 1.16 [95% CI 1.04–1.30], p = 0.007) were associated with MACE, while other conventional TTE parameters, including LVEF and LV longitudinal strain, were not. HDF angle was an independent predictor of MACE after adjusting for age, LVEF, and LV longitudinal strain on separate multivariate Cox analyses. HDF angle &amp;gt; 70° was consistently associated with worse prognosis (HR 2.68 [95% CI 1.09–6.60], p = 0.032). Conclusions HDF angle is an independent predictor of MACE onset in non-ischemic DCM patients. Higher HDF angle may be a marker of late worsening in myocardial performance in patients with reduced LVEF.

Is myocardial fibrosis in patients with non-ischemic dilated cardiomyopathy associated with the whole-heart myocardial mechanics?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Non-ischemic dilated cardiomyopathy (NIDCM) is a severe form of primary myocardial disease related with progressive heart failure (HF) and a poor prognosis. In patients with NIDCM, myocardial fibrosis (MF) is associated to adverse cardiovascular outcomes and should be evaluated quickly in each case of NIDCM [1,2]. Purpose To evaluate the relationships between the whole-heart myocardial strain parameters and presence of late gadolinium enhancement (LGE) in a prospective cohort of NIDCM patients. Methods The study group consisted of 101 patients with NIDCM who were referred for a cardiovascular magnetic resonance (CMR) and speckle tracking echocardiography examination. We analyzed MF by LGE, and the whole-heart (both ventricles and atria) myocardial mechanics were evaluated by speckle tracking. All patients underwent a comprehensive clinical evaluation, laboratory test assessment, electrocardiogram, Holter monitoring. The exclusion criteria were ischemic coronary disease, primary valvular heart disease, chronic kidney disease (eGFR &amp;lt;30 ml/min/1.73 m2), under the age of 18, a poor echocardiographic or CMR image quality, inflammatory myocardial disease, previous pulmonary embolism, peripartum cardiomyopathy and toxic damage. Results The study group was divided into two groups according to the presence of MF fibrosis. The presence of MF was associated to worse of the whole-heart myocardial strain parameters. Both ventricles or atria were more dilated in the group with MF. The strongest relationships were detected between presence of MF and mechanical strain parameters of the left side of the heart (left ventricle ejection fraction (LVEF) (r=0.662, 25 p&amp;lt;0.001), left ventricle global circumferential strain (LV GCS) (r=-0.609, p&amp;lt;0.001), left ventricle global longitudinal strain (LV GLS) (r=-0.586, p&amp;lt;0.001), left atrial reservoir strain (LASr) (r=0.588, p&amp;lt;0.001). Logistic regression analysis revealed that the last three mentioned strain parameters were independently associated with presence of MF. Conclusions In patients with NIDCM, the whole-heart myocardial mechanics are worse in patients‘ presence of MF. LV GLS, LV GCS, and LARs are the best 2D echocardiographic mechanical strain parameters to prognosticate the presence of MF.