Assessment of ICD eligibility in non-ischaemic cardiomyopathy patients: a position statement by the Task Force of the Dutch Society of Cardiology.

International guidelines recommend implantation of an implantable cardioverter-defibrillator (ICD) in non-ischaemic cardiomyopathy (NICM) patients with a left ventricular ejection fraction (LVEF) below 35% despite optimal medical therapy and a life expectancy of more than 1 year with good functional status. We propose refinement of these recommendations in patients with NICM, with careful consideration of additional risk parameters for both arrhythmic and non-arrhythmic death. These additional parameters include late gadolinium enhancement on cardiac magnetic resonance imaging and genetic testing for high-risk genetic variants to further assess arrhythmic risk, and age, comorbidities and sex for assessment of non-arrhythmic mortality risk. Moreover, several risk modifiers should be taken into account, such as concomitant arrhythmias that may affect LVEF (atrial fibrillation, premature ventricular beats) and resynchronisation therapy. Even though currently no valid cut-off values have been established, the proposed approach provides a more careful consideration of risks that may result in withholding ICD implantation in patients with low arrhythmic risk and substantial non-arrhythmic mortality risk.

Transcoronary mapping with an over-the-wire multielectrode catheter in scar-related ventricular tachycardia patients.

AIMS: The usefulness of coronary venous system mapping has been reported for assessing intramural and epicardial substrates in patients with scar-related ventricular tachycardia (VT). However, there has been little data on mapping from coronary arteries. We investigated the safety and utility of mapping from coronary arteries with a novel over-the-wire multielectrode catheter in scar-related VT patients. METHODS AND RESULTS: Ten consecutive scar-related VT patients with non-ischaemic cardiomyopathy who underwent mapping from a coronary artery were analysed. Six patients underwent simultaneous coronary venous mapping. High-density maps were created by combining the left ventricular endocardium and coronary vessels. Substrate maps were created during the baseline rhythm with 2438 points (IQR 2136-3490 points), including 329 (IQR 59-508 points) in coronary arteries. Abnormal bipolar electrograms were successfully recorded within coronary arteries close to the endocardial substrate in seven patients. During VT, isthmus components were recorded within the coronary vessels in three patients with no discernible isthmus components on endocardial mapping. The ablation terminated the VT from an endocardial site opposite the earliest site in the coronary arteries in five patients. CONCLUSION: The transcoronary mapping with an over-the-wire multielectrode catheter can safely record abnormal bipolar electrograms within coronary arteries. Additional mapping data from the coronary vessels have the potential to assess three-dimensional ventricular substrates and circuit structures in scar-related VT patients.

Comparison of Clinical Course and Outcomes between Dilated and Hypokinetic Non-Dilated Cardiomyopathy.

BACKGROUND: By definition, dilated cardiomyopathy (DCM) is characterized by enlargement of the left ventricular (LV) cavity, and systolic dysfunction. However, in 2016 ESC introduced a new clinical entity - hypokinetic non-dilated cardiomyopathy (HNDC). HNDC is defined as LV systolic dysfunction without LV dilatation. However, the diagnosis of HNDC has so far rarely been made by a cardiologist, and it is unknown whether "classic" DCM differs from HNDC in terms of clinical course and outcomes. OBJECTIVES: Comparison of heart failure profiles and outcomes between patients with "classic" dilated (DCM) and HNDCs. METHOD: We retrospectively analysed 785 DCM patients, defined as impaired left ventricle (LV) systolic function (ejection fraction [LVEF] <45%) in the absence of coronary artery disease, valve disease, congenital heart disease, and severe arterial hypertension. "Classic" DCM was diagnosed when LV dilatation was present (LV end-diastolic diameter >52 mm/58 mm in women/men); otherwise, HNDC was diagnosed. After 47 ± 31 months, the all-cause mortality and composite endpoint (all-cause mortality, heart transplant - HTX, left ventricle assist device implantation - LVAD) were assessed. RESULTS: There were 617 (79%) patients with LV dilatation. Patients with "classic" DCM differed from HNDC in terms of clinically relevant parameters [hypertension (47% vs. 64%, p = 0.008), ventricular tachyarrhythmias (29% vs. 15%, p = 0.007), NYHA class (2.5 ± 0.9 vs. 2.2 ± 0.8, p = 0.003)], had lower cholesterol (LDL: 2.9 ± 1.0 vs. 3.2 ± 1.1 mmol/L, p = 0.049), and higher N-terminal pro-brain natriuretic peptide (3,351 ± 5,415 vs. 2,563 ± 8584 pg/mL, p = 0.0001) and required higher diuretics dosages (57.8 ± 89.5 vs. 33.7 ± 48.7 mg/day, p ≤ 0.0001). All of their chambers were larger (LVEDd: 68.3 ± 4.5 vs. 52.7 ± 3.5 mm, p < 0.0001) and they had lower LVEF (25.2 ± 9.4 vs. 36.6 ± 11.7%, p < 0.0001). During the follow-up, there were 145 (18%) composite endpoints ("classic" DCM vs. HNDC: 122 [20%] vs. 26 [18%], p = 0.22): deaths (97 [16%] vs. 24 [14%], p = 0.67), HTX (17 [4%] vs. 4 [4%], p = 0.97) and LVAD (19 [5%] vs. 0 [0%], p = 0.03). Both groups did not differ in terms of all-cause mortality (p = 0.70), cardiovascular (CV) mortality (p = 0.37) and composite endpoint (p = 0.26). CONCLUSIONS: LV dilatation was absent in more than one-fifth of DCM patients. HNDC patients had less severe heart failure symptoms, less advanced cardiac remodelling, and required lower diuretics dosages. On the other hand, "classic" DCM and HNDC patients did not differ in terms of all-cause mortality, CV mortality, and composite endpoint.

Implantable defibrillator therapy and mortality in patients with non-ischaemic dilated cardiomyopathy : An updated meta-analysis and effect on Dutch clinical practice by the Task Force of the Dutch Society of Cardiology.

BACKGROUND: Primary prophylactic implantable cardioverter-defibrillators (ICDs) in patients with non-ischaemic cardiomyopathy (NICMP) remains controversial. This study sought to assess the benefit of ICD therapy with or without cardiac resynchronisation therapy (CRT) in patients with NICMP. In addition, data were compared with real-world clinical data to perform a risk/benefit analysis. METHODS: Relevant randomised clinical trials (RCTs) published in meta-analyses since DANISH, and in PubMed, EMBASE and Cochrane databases from 2016 to 2020 were identified. The benefit of ICD therapy stratified by CRT use was assessed using random effects meta-analysis techniques. RESULTS: Six RCTs were included in the meta-analysis. Among patients without CRT, ICD use was associated with a 24% reduction in mortality (hazard ratio [HR]: 0.76; 95% confidence interval [CI]: 0.62-0.93; P = 0.008). In contrast, among patients with CRT, a CRT-defibrillator was not associated with reduced mortality (HR: 0.74, 95% CI 0.47-1.16; P = 0.19). For ICD therapy without CRT, absolute risk reduction at 3­years follow-up was 3.7% yielding a number needed to treat of 27. CONCLUSION: ICD use significantly improved survival among patients with NICMP who are not eligible for CRT. Considering CRT, the addition of defibrillator therapy was not significantly associated with mortality benefit compared with CRT pacemaker.

Catheter Ablation for Ventricular Tachycardia in Ischaemic Versus Non-Ischaemic Cardiomyopathy: A Systematic Review and Meta-Analysis.

BACKGROUND: There are differences in substrate and ablation approaches for ventricular tachycardia (VT) in ischaemic (ICM) and non-ischaemic cardiomyopathy (NICM). OBJECTIVE: To perform a systematic review and meta-analysis comparing clinical and procedural characteristics/outcomes of VT ablation in ICM versus NICM. METHODS: Electronic databases were searched for comparative studies reporting outcomes of VT ablation in patients with ICM and NICM. Primary outcomes were acute procedural success, VT recurrence and long-term mortality. Meta-analyses were performed using random-effects modelling. RESULTS: Thirty-one (31) studies (7,473 patients; 4,418 ICM and 3,055 NICM) were included. Patients with ICM were significantly older (67.0 vs 55.3 yrs), more commonly male (89% vs 79%), had lower left ventricular ejection fraction (29% vs 38%) were less likely to undergo epicardial access (11% vs 36%) and were more likely to require haemodynamic support during ablation (relative risk [RR] 1.30; 95% CI 1.01-1.69). Acute procedural success (i.e. non-inducibility of VT) was higher in the ICM cohort (RR 1.10, 95% CI 1.05-1.15). Recurrence of VT at follow-up was significantly lower in the ICM cohort (RR 0.77; 95% CI 0.70-0.84). Peri-procedural mortality, incidence of procedural complications and long-term mortality were not significantly different between the cohorts. CONCLUSIONS: NICM and ICM patients undergoing VT ablation are fundamentally different in their clinical characteristics, ablation approaches, acute procedural outcomes and likelihood of VA recurrence. VT ablation in NICM has a lower likelihood of procedural success with increased risk of VA recurrence, consistent with known challenging arrhythmia substrate.

A speckle-tracking strain-based artificial neural network model to differentiate cardiomyopathy type.

Objectives. In heart failure, invasive angiography is often employed to differentiate ischaemic from non-ischaemic cardiomyopathy. We aim to examine the predictive value of echocardiographic strain features alone and in combination with other features to differentiate ischaemic from non-ischaemic cardiomyopathy, using artificial neural network (ANN) and logistic regression modelling. Design. We retrospectively identified 204 consecutive patients with an ejection fraction <50% and a diagnostic angiogram. Patients were categorized as either ischaemic (n = 146) or non-ischaemic cardiomyopathy (n = 58). For each patient, left ventricular strain parameters were obtained. Additionally, regional wall motion abnormality, 13 electrocardiographic (ECG) features and six demographic features were retrieved for analysis. The entire cohort was randomly divided into a derivation and a validation cohort. Using the parameters retrieved, logistic regression and ANN models were developed in the derivation cohort to differentiate ischaemic from non-ischaemic cardiomyopathy, the models were then tested in the validation cohort. Results. A final strain-based ANN model, full feature ANN model and full feature logistic regression model were developed and validated, F1 scores were 0.82, 0.79 and 0.63, respectively. Conclusions. Both ANN models were more accurate at predicting cardiomyopathy type than the logistic regression model. The strain-based ANN model should be validated in other cohorts. This model or similar models could be used to aid the diagnosis of underlying heart failure aetiology in the form of the online calculator (https://cimti.usj.edu.lb/strain/index.html) or built into echocardiogram software.

High spatial resolution free-breathing 3D late gadolinium enhancement cardiac magnetic resonance imaging in ischaemic and non-ischaemic cardiomyopathy: quantitative assessment of scar mass and image quality.

PURPOSE: To compare breath-hold (BH) with navigated free-breathing (FB) 3D late gadolinium enhancement cardiac MRI (LGE-CMR) MATERIALS AND METHODS: Fifty-one patients were retrospectively included (34 ischaemic cardiomyopathy, 14 non-ischaemic cardiomyopathy, three discarded). BH and FB 3D phase sensitive inversion recovery sequences were performed at 3T. FB datasets were reformatted into normal resolution (FB-NR, 1.46x1.46x10mm) and high resolution (FB-HR, isotropic 0.91-mm voxels). Scar mass, scar edge sharpness (SES), SNR and CNR were compared using paired-samples t-test, Pearson correlation and Bland-Altman analysis. RESULTS: Scar mass was similar in BH and FB-NR (mean ± SD: 15.5±18.0 g vs. 15.5±16.9 g, p=0.997), with good correlation (r=0.953), and no bias (mean difference ± SD: 0.00±5.47 g). FB-NR significantly overestimated scar mass compared with FB-HR (15.5±16.9 g vs 14.4±15.6 g; p=0.007). FB-NR and FB-HR correlated well (r=0.988), but Bland-Altman demonstrated systematic bias (1.15±2.84 g). SES was similar in BH and FB-NR (p=0.947), but significantly higher in FB-HR than FB-NR (p<0.01). SNR and CNR were lower in BH than FB-NR (p<0.01), and lower in FB-HR than FB-NR (p<0.01). CONCLUSION: Navigated free-breathing 3D LGE-CMR allows reliable scar mass quantification comparable to breath-hold. During free-breathing, spatial resolution can be increased resulting in improved sharpness and reduced scar mass. KEY POINTS: • Navigated free-breathing 3D late gadolinium enhancement is reliable for myocardial scar quantification. • High-resolution 3D late gadolinium enhancement increases scar sharpness • Ischaemic and non-ischaemic cardiomyopathy patients can be imaged using free-breathing LGE CMR.

Investigation and treatment for iron deficiency in heart failure: the unmet need in Lower and Middle Income Countries.

Frank iron deficiency has been associated with a wide range of cardiac and pulmonary abnormalities including non-ischaemic cardiomyopathy. Iron deficiency anaemia and isolated iron deficiency are well-defined adverse prognostic factors in non-ischaemic cardiac failure. Furthermore, iron-deficient patients in chronic heart failure with a serum ferritin of <100 µg/l or <300 µg/l with reduced transferrin saturation of <20%, who were given intravenous iron showed improved clinical outcomes. Iron deficiency with or without anaemia affects over a quarter of the world's population, but the impact of iron deficiency in heart failure and the effective management of iron deficiency in heart failure in Lower and Middle Income Countries (LMICs) is not well described. Heart failure in African cohorts occurs at a younger age than in North America and Europe and is more likely to be due to hypertension. Recent studies suggest that iron deficiency anaemia, which is very common in heart failure patients in Africa, and iron deficiency are independently associated with a poor prognosis in heart failure. Preliminary data suggest that iron deficiency in patients with heart failure can be treated with oral iron, with significant beneficial effects on haematological and physiological variables. Cost may prohibit the use of intravenous iron on a large scale in LMICs and optimal regimes to treat iron deficiency in heart failure patients with oral iron therapy remain to be defined.

The impact of frailty in older patients with non-ischaemic cardiomyopathy after implantation of cardiac resynchronization therapy defibrillator.

AIMS: Frailty status impacts the prognosis in older patients with heart disease. However, frailty status impact is unknown in patients with non-ischaemic cardiomyopathy after cardiac resynchronization therapy (CRT). METHODS AND RESULTS: Functional measures of baseline frailty and clinical data were collected for all patients with non-ischaemic cardiomyopathy before CRT defibrillator (CRT-D) implantation. The level of frailty was assessed using the Fried and Walston definition. Cox proportional hazard regression models were used to examine the association between baseline frailty and decompensated heart failure (HF) at the 12 months follow-up. The cohort study consisted of 102 patients with a mean age of 73 ± 4 years, 53% of which were male patients. Twenty-nine patients (28%) were classified as frail before CRT-D implantation. Twenty-seven patients experienced decompensated HF after CRT-D implantation at the 12-month follow-up. In the non-frail group, 12 of 73 patients (16.4%) experienced episodes of decompensated HF. In contrast, 15 of 29 (55.6%) frail patients experienced higher proportions of decompensated HF (P < 0.001). Patients who were frail (hazard ratio 4.55, 95% confidence interval 1.726-12.013) were at increased risk for the decompensated HF (P for trend = 0.002) compared with those who were not frail. CONCLUSION: Frailty is a strong predictor of adverse post-implantation outcome in patients with non-ischaemic cardiomyopathy undergoing CRT-D.

Risk factors and the effect of cardiac resynchronization therapy on cardiac and non-cardiac mortality in MADIT-CRT.

AIMS: To understand modes of death and factors associated with the risk for cardiac and non-cardiac deaths in patients with cardiac resynchronization therapy with implantable cardioverter-defibrillator (CRT-D) vs. implantable cardioverter-defibrillator (ICD) therapy, which may help clarify the action and limitations of cardiac resynchronization therapy (CRT) in relieving myocardial dysfunction. METHODS AND RESULTS: In Multicenter Automatic Defibrillator Implantation Trial-Cardiac Resynchronization Therapy (MADIT-CRT), during 4 years of follow-up, 169 (9.3%) of 1820 patients died of known causes, 108 (63.9%) deemed cardiac, and 61 (36.1%) non-cardiac. In multivariate analysis, increased baseline creatinine was significantly associated with both cardiac and non-cardiac deaths [hazard ratio (HR) 2.97, P < 0.001; HR 1.80, P = 0.035, respectively], as was diabetes (HR 1.79, P = 0.006; HR 1.73, P = 0.038, respectively), and the worst New York Heart Association Class > II more than 3 months prior to enrolment (HR 1.90, P = 0.012; HR 2.46, P = 0.010, respectively). Baseline left atrial volume index was significantly associated only with cardiac mortality (HR 1.28 per 5 unit increase, P < 0.001). Ischaemic cardiomyopathy was associated only with non-cardiac death (HR 3.54, P = 0.001). CRT-D vs. an ICD-only was associated with a reduced risk for cardiac death in patients with left bundle branch block (LBBB) (HR 0.56, P = 0.029) but was associated with an increased risk for non-cardiac death in non-LBBB patients (HR 3.48, P = 0.048). CONCLUSIONS: In MADIT-CRT, two-thirds of the deaths were cardiac and one-third non-cardiac. Many of the same risk factors were associated with both cardiac and non-cardiac mortalities. CRT-D was associated with a reduced risk for cardiac death in LBBB but an increased risk for non-cardiac death in non-LBBB. CLINICAL TRIAL REGISTRATION: Information for the MADIT-CRT main study http://www.clinicaltrials.gov, NCT00180271.

Outcomes after atrioventricular node ablation and biventricular pacing in patients with refractory atrial fibrillation and heart failure: a comparison between non-ischaemic and ischaemic cardiomyopathy.

AIMS: Atrioventricular junction ablation (AVJA) combined with biventricular (BiV) pacing (AVJA/BiV) is an effective treatment for refractory atrial fibrillation (AF) and rapid ventricular response (RVR) associated with heart failure (HF). This study compared the outcomes between patients with non-ischaemic (DCM) and ischaemic cardiomyopathy (ICM) following AVJA/BiV for AF/RVR. METHODS AND RESULTS: This was a retrospective study of 45 patients, comparing the response to AVJA/BiV in patients with ICM to those with DCM. The study compared (a) the change in echocardiographic parameters of HF (ejection fraction (EF) and left ventricular dimensions) prior to, and at least 6 months post AVJA/BiV; and (b) HF hospitalizations (HFH) and appropriate implantable cardioverter defibrillator (ICD) therapies occurring post-procedure. Ejection fraction improved significantly in the DCM group (ΔEF 11.2% ± 11.9; P< 0.01); however, EF remained unchanged (ΔEF 0.5% ± 9.9; P = NS) in the ICM group post-AVJA/BiV. Post-procedurely, HFH were significantly more common (15/18 vs. 4/25; P < 0.0001), and there were significantly more appropriate ICD therapies (9.4 ± 12.3 vs. 2.3 ± 6.1; P = 0.01) in the ICM compared with the DCM group. CONCLUSION: After AVJA/BiV, there was significantly less post-procedural echocardiographic reverse remodelling, and more HFH in the ICM compared with the DCM group. In addition, significantly more appropriate ICD therapies occurred in ICM patients post-procedure. These differences may be due to the presence of more extensive discrete myocardial scar in patients with ICM. Furthermore, it is possible that tachycardia-induced cardiomyopathy plays more of a causative role in HF in patients with AF and DCM than those with ICM.

Sex-based differences in the phenotypic expression and prognosis of idiopathic non-ischaemic cardiomyopathy: a cardiovascular magnetic resonance study.

AIMS: We sought to characterize sex-related differences in cardiovascular magnetic resonance-based cardiovascular phenotypes and prognosis in patients with idiopathic non-ischaemic cardiomyopathy (NICM). METHODS AND RESULTS: Patients with NICM enrolled in the Cardiovascular Imaging Registry of Calgary (CIROC) between 2015 and 2021 were identified. Z-score values for chamber volumes and function were calculated as standard deviation from mean values of 157 sex-matched healthy volunteers, ensuring reported differences were independent of known sex-dependencies. Patients were followed for the composite outcome of all-cause mortality, heart failure admission, or ventricular arrhythmia. A total of 747 patients were studied, 531 (71%) males. By Z-score values, females showed significantly higher left ventricular (LV) ejection fraction (EF; median difference 1 SD) and right ventricular (RV) EF (difference 0.6 SD) with greater LV mass (difference 2.1 SD; P < 0.01 for all) vs. males despite similar chamber volumes. Females had a significantly lower prevalence of mid-wall striae (MWS) fibrosis (22% vs. 34%; P < 0.001). Over a median follow-up of 4.7 years, 173 patients (23%) developed the composite outcome, with equal distribution in males and females. LV EF and MWS were significant independent predictors of the outcome (respective HR [95% CI] 0.97 [0.95-0.99] and 1.6 [1.2-2.3]; P = 0.003 and 0.005). There was no association of sex with the outcome. CONCLUSION: In a large contemporary cohort, NICM was uniquely expressed in females vs. males. Despite similar chamber dilation, females demonstrated greater concentric remodelling, lower reductions in bi-ventricular function, and a lower burden of replacement fibrosis. Overall, their prognosis remained similar to male patients with NICM.

Cardiac resynchronization therapy in heart failure based on Strauss criteria for left bundle branch block.

AIMS: The left bundle branch block (LBBB) is a strong predictor of response to cardiac resynchronization therapy (CRT). However, a significant number of patients do not respond to the treatment. The study sought to evaluate the impact of the stricter Strauss criteria for left bundle branch block (St-LBBB) on CRT response, hospitalizations, ventricular arrhythmia (VA) events and mortality. METHODS: This study is a retrospective analysis of prospectively collected data on heart failure (HF) patients with LBBB admitted for CRT implantation. Patients were divided into two groups according to the fulfilment or not of St-LBBB criteria. RESULTS: The study included 82 patients with ischaemic (ICM) and non-ischaemic (NICM) cardiomyopathy [46 (56%) with St-LBBB and 36 (44%) with non-St-LBBB]. Patients with St-LBBB showed higher CRT response rates compared with those with non-St-LBBB (P < 0.01), while the group with NICM exhibited the greatest benefit (P < 0.01). St-LBBB CRT responders displayed significantly lower rates of HF hospitalization (P < 0.0001) compared with the non-St-LBBB group. According to Kaplan-Meier time curves, this was primarily evident in patients with NICM (P < 0.0001). CRT responders displayed significantly fewer VA events (P < 0.001) and lower mortality rates (P < 0.0001) than non-responders. Kaplan-Meier estimates demonstrated a significantly lower incidence of VAs in NICM patients with St-LBBB (P = 0.049) compared with ICM patients with St-LBBB (P = 0.25). Lower mortality rates were observed in CRT responders than non-responders (P < 0.0001), with the group of NICM with St-LBBB criteria exhibiting the greatest benefit (P = 0.0238). CONCLUSIONS: Patients with NICM and St-LBBB present the greatest benefit concerning CRT response, HF hospitalizations, VA events and mortality. Although St-LBBB criteria seem to improve patient selection for CRT, more data are needed to elucidate the role of St-LBBB criteria in this setting.

Inhibition of myeloperoxidase to treat left ventricular dysfunction in non-ischaemic cardiomyopathy.

AIMS: Non-ischaemic cardiomyopathy (NICMP), an incurable disease terminating in systolic heart failure (heart failure with reduced ejection fraction [HFrEF]), causes immune activation, however anti-inflammatory treatment strategies so far have failed to alter the course of this disease. Myeloperoxidase (MPO), the principal enzyme in neutrophils, has cytotoxic, pro-fibrotic and nitric oxide oxidizing effects. Whether MPO inhibition ameliorates the phenotype in NICMP remains elusive. METHODS AND RESULTS: Prognostic information from MPO was derived from proteomic data of a large human cardiovascular health cohort (n = 3289). In a murine model of NICMP, we studied the mechanisms of MPO in this disease. In a case series, the MPO inhibitor was also evaluated in NICMP patients. Individuals with increased MPO revealed higher long-term mortality and worsening of heart failure, with impaired prognosis when MPO increased during follow-up. MPO infusion attenuated left ventricular ejection fraction (LVEF) in mice with NICMP, whereas genetic ablation or inhibition of MPO decreased systemic vascular resistance (SVR, 9.4 ± 0.7 mmHg*min/ml in NICMP vs. 6.7 ± 0.8 mmHg*min/ml in NICMP/Mpo-/-mice, n = 8, p = 0.006, data expressed as mean ± standard error of the mean) and improved left ventricular function (LVEF 30.3 ± 2.2% in NICMP vs. 40.7 ± 1.1% in NICMP/Mpo-/- mice, n = 16, p < 0.0001). Four patients diagnosed with NICMP and treated with an MPO inhibitor over 12 weeks showed increase in LVEF, decline in natriuretic peptides and improved 6-min walking distance. MPO inhibitor-related changes in the proteome of NICMP patients predicted reduced mortality when related to the changes in the proteome of the above referenced cardiovascular health cohort. CONCLUSIONS: Myeloperoxidase predicts long-term outcome in HFrEF and its inhibition elicits systemic anti-inflammatory and vasodilating effects which translate into improved left ventricular function. MPO inhibition deserves further evaluation as a novel, complementary treatment strategy for HFrEF.

Screening and risk evaluation for sudden cardiac death in ischaemic and non-ischaemic cardiomyopathy: results of the European Heart Rhythm Association survey.

The purpose of this EHRA survey was to examine the current clinical practice of screening and risk evaluation for sudden cardiac death in ischaemic and non-ischaemic cardiomyopathy with a focus on selection of candidates for implantable cardioverter-defibrillator (ICD) therapy, timing of ICD implantation, and use of non-invasive and invasive diagnostic tests across Europe. A systematic screening programme for sudden cardiac death existed in 19 out of 31 centres (61.3%). Implantation of ICDs according to the inclusion criteria of MADIT-II and SCD-HeFT trials was reported in 30 and 29% of centres, respectively, followed by MADIT-CRT (18%), COMPANION (16%), and combined MADIT and MUSTT (7%) indications. In patients with severe renal impairment, ICD implantation for primary prevention of sudden death was always avoided in 8 centres (33.3%), was not used only if creatinine level was >2.5 mg/dL in 10 centres (32.2%), and in patients with permanent dialysis in 8 centres (33.3%). Signal-averaged electrocardiography and heart rate variability were never considered as risk stratification tools in 23 centres (74.2%). Implantation of a loop recorder was performed in patients with borderline indications for ICD therapy in 6 centres (19.4%), for research purposes in 5 (16.1%), and was never performed in 20 (64.5%) centres. In conclusion, the majority of participating European centres have a screening programme for sudden cardiac death and the selection of candidates for ICD therapy was mainly based on the clinical risk stratification and not on non-invasive and invasive diagnostic tests or implantable loop recorder use.

Phenotype, outcomes and natural history of early-stage non-ischaemic cardiomyopathy.

AIMS: To characterize the phenotype, clinical outcomes and rate of disease progression in patients with early-stage non-ischaemic cardiomyopathy (early-NICM). METHODS AND RESULTS: We conducted a prospective observational cohort study of patients with early-NICM assessed by late gadolinium enhancement cardiovascular magnetic resonance (CMR). Cases were classified into the following subgroups: isolated left ventricular dilatation (early-NICM H-/D+), non-dilated left ventricular cardiomyopathy (early-NICM H+/D-), or early dilated cardiomyopathy (early-NICM H+/D+). Clinical follow-up for major adverse cardiovascular events (MACE) included non-fatal life-threatening arrhythmia, unplanned cardiovascular hospitalization or cardiovascular death. A subset of patients (n = 119) underwent a second CMR to assess changes in cardiac structure and function. Of 254 patients with early-NICM (median age 46 years [interquartile range 36-58], 94 [37%] women, median left ventricular ejection fraction [LVEF] 55% [52-59]), myocardial fibrosis was present in 65 (26%). There was no difference in the prevalence of fibrosis between subgroups (p = 0.90), however fibrosis mass was lowest in early-NICM H-/D+, higher in early-NICM H+/D- and highest in early-NICM H+/D+ (p = 0.03). Over a median follow-up of 7.9 (5.5-10.0) years, 28 patients (11%) experienced MACE. Non-sustained ventricular tachycardia (hazard ratio [HR] 5.1, 95% confidence interval [CI] 2.36-11.00, p < 0.001), myocardial fibrosis (HR 3.77, 95% CI 1.73-8.20, p < 0.001) and diabetes mellitus (HR 5.12, 95% CI 1.73-15.18, p = 0.003) were associated with MACE in a multivariable model. Only 8% of patients progressed from early-NICM to dilated cardiomyopathy with LVEF <50% over a median of 16 (11-34) months. CONCLUSION: Early-NICM is not benign. Fibrosis develops early in the phenotypic course. In-depth characterization enhances risk stratification and might aid clinical management.

Prognostic value of myocardial scar in ischaemic and non-ischaemic cardiomyopathy using cardiac magnetic resonance imaging.

AIM: The aim of this research was to evaluate the prognostic value of myocardial scar using cardiac magnetic resonance (CMR) imaging in patients with ischaemic cardiomyopathy (ICM) and non-ischaemic cardiomyopathy (NICM). METHODS: One hundred and fifty-four patients with either ICM or NICM underwent CMR with late gadolinium enhancement sequences for assessment of left ventricular ejection fraction (LVEF), and detection and quantification of any myocardial scar using three methods: manual, number of segments involved, and percentage of scarred myocardium. Patients were followed up for at least six months for clinical cardiac events. RESULTS: Patients were divided into two groups: group I, patients with ICM (58%) and group II, those with NICM (42%). Clinical presentation ranged from eventless lpar;10%) to chest pain (18%), heart failure (15%), hospitalisation (35%), syncope (1%), ventricular tachycardia (< 1%) and cardiac arrest (< 1%). The scar mass was larger in size in group I (17 ± 15%) than in group II (8 ± 13%). A direct relationship was observed between scar size and event severity (p < 0.001). An inverse relationship between LVEF and event severity was found in group I (p < 0.001) but not in group II (p = 0.128). CONCLUSIONS: Myocardial scar size was a strong predictor of clinical outcome in both the ICM and NICM patients. LVEF was less reliable in predicting morbidity in cardiomyopathy patients.

A Case of Severe Cardiomyopathy Due to Covid-Induced Myocarditis, Completely Resolved after Colchicine and Immunoglobulin Therapy.

Coronavirus 19 (COVID-19) is well known for causing acute respiratory distress syndrome. Among other systemic complications, myocarditis is a frequently reported presentation as well as complication. One systematic review reported a 14% mortality rate in patients with COVID-19 myocarditis. Endomyocardial biopsy is a definitive diagnostic test but has been a challenge to perform in most cases of COVID myocarditis due to the contagious nature of the disease. Patients presenting with new cardiomyopathy with troponin leak and arrhythmias, supported by recent COVID-19 diagnosis should be suspected for COVID-induced myocarditis. Supportive treatment has been the mainstay of treatment with limited data on immunotherapy and colchicine. Our case is about a male in his 50s who had a cardiac arrest due to ventricular fibrillations, with a positive COVID-19 test. Further workup showed severe non-ischaemic cardiomyopathy with an EF of 15-20%. He was treated with intravenous immunotherapy and colchicine. A repeat echocardiogram 3 days later showed resolution of cardiomyopathy. Our case report highlights the possible beneficial effects of immunotherapy and colchicine in viral myocarditis. LEARNING POINTS: Myocarditis should be suspected in patients with acute onset cardiomyopathy with troponin leak and no evidence of ischaemia. COVID-19 myocarditis can present with arrhythmia, which could be fatal in some cases.Even though supportive management is the mainstay of treatment for COVID-19 myocarditis, there have been reports of benefits of intravenous immunotherapy (IVIG) and colchicine.More studies are warranted to explore the beneficial effects of IVIG and colchicine not just in COVID-19 myocarditis, but also in other viral causes of myocarditis he aim of this study is also to raise awareness among healthcare professionals about the Bentall procedure in patients with type A aortic dissection involving the aortic valve.

Multicentre medicoeconomic evaluation of cardiac magnetic resonance imaging for predicting coronary artery disease in left ventricular dysfunction: The CAMAREC study design.

BACKGROUND: Cardiac magnetic resonance imaging may provide a non-invasive alternative to coronary angiography for differentiating between ischaemic and non-ischaemic cardiomyopathy in cases of unexplained reduced left ventricular ejection fraction. AIM: The CAMAREC study aims to evaluate the diagnostic accuracy of cardiac magnetic resonance imaging in predicting significant coronary artery disease in patients with reduced left ventricular ejection fraction, using coronary angiography as the gold standard for comparison. METHODS: CAMAREC is a prospective cohort study of 406 patients in 10 centres with newly diagnosed, unexplained left ventricular ejection fraction ≤ 45%. Cardiac magnetic resonance imaging and coronary angiography will be conducted within a 2-week interval, starting with cardiac magnetic resonance imaging; independent committees will review the results blindly. Primary outcome is sensitivity of detecting ischaemic scar on cardiac magnetic resonance imaging for predicting significant coronary artery disease on coronary angiography according to Felker's criteria. Secondary outcomes include specificity and positive and negative predictive values (with 95% confidence intervals) of cardiac magnetic resonance imaging for predicting significant coronary artery disease in patients with reduced left ventricular ejection fraction, kappa concordance coefficient between cardiac magnetic resonance imaging and coronary angiography for diagnosing the affected myocardial territory, and the impact of cardiac magnetic resonance imaging on revascularization decisions. Two ancillary studies will evaluate the incremental cost-effectiveness of using cardiac magnetic resonance imaging first versus coronary angiography first, and the sensitivity of pre- and postcontrast T1-mapping for predicting significant coronary artery disease in patients with reduced left ventricular ejection fraction. CONCLUSION: Our study protocol is designed to rigorously evaluate cardiac magnetic resonance imaging as a non-invasive alternative to coronary angiography in patients with unexplained reduced left ventricular ejection fraction. The results will have significant implications for patient management, and may support growing evidence for the clinical utility of cardiac magnetic resonance imaging.