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Aims: 'Hot phases', characterized by chest pain and troponin release, may represent the first clinical presentation of arrhythmogenic cardiomyopathies. Differential diagnosis with acute myocarditis is an unmet challenge for the clinicians. We sought to investigate histological and genetic features in patients with cardiomyopathy presenting with hot phases. Methods and results: We evaluated a case series of consecutive patients hospitalized for suspected 'hot‐phase cardiomyopathy' in two Italian centres from June 2017 to March 2022 (median follow‐up 18 months) that underwent both endomyocardial biopsy (EMB) and genetic testing. Apoptosis was confirmed with TUNEL assay. Among the 17 enrolled patients (mean age 34 ± 15 years, 76% male), only six patients (35%) presented standard histological and immunohistochemical markers for significant cardiac inflammation at EMB. Conversely, apoptosis was found in 13 patients (77%). Genetic testing was positive for a pathogenic/likely pathogenic (P/LP) variant in genes involved in cardiomyopathies (most frequently in DSP) in eight patients (48%), rising to 62% among patients with apoptosis on EMB. Notably, all patients without apoptosis tested negative for P/LP disease‐related variants. Left ventricular ejection fraction was lower in patients showing apoptosis at EMB compared to those without (p = 0.003). Conclusions: Apoptosis, rather than significant inflammation, was mostly prevalent in this case series of patients with 'hot‐phase' presentation, especially in carriers of variants in cardiomyopathy‐related genes. Detecting apoptosis on EMB might guide clinicians in performing genetic testing and in more tailored therapeutic choices in 'hot‐phase cardiomyopathy'. [ABSTRACT FROM AUTHOR]

Background: Prognostic stratification of acute myocarditis (AM) presenting with normal left ventricular ejection fraction (LVEF) relies mostly on late gadolinium enhancement (LGE) characterization. Left ventricular peak global longitudinal strain (LV‐GLS) measured by feature tracking analysis might improve prognostication of AM presenting with normal LVEF. Methods: Data of patients undergoing cardiac magnetic resonance (CMR) for clinically suspected AM in seven European Centres (2013–2020) were retrospectively analysed. Patients with AM confirmed by CMR and LVEF ≥50% were included. LGE was visually characterized: localized versus. non‐localized, subepicardial versus midwall. LV‐GLS was measured by dedicated software. The primary outcome was the first occurrence of an adverse cardiovascular event (ACE) including cardiac death, life‐threatening arrhythmias, development of heart failure or of LVEF <50%. Results: Of 389 screened patients, 256 (66%) fulfilled inclusion criteria: median age 36 years, 71% males, median LVEF 60%, median LV‐GLS ‐17.3%. CMR was performed at 4 days from hospitalization. At 27 months, 24 (9%) patients experienced ≥1 ACE (71% developed LVEF <50%). Compared to the others, they had lower median LV‐GLS values (−13.9% vs. −17.5%, p =.001). At Kaplan–Meier analysis, impaired LV‐GLS (both considered as > −20% or quartiles), non‐localized and midwall LGE were associated with ACEs. Patients with LV‐GLS ≤−20% did not experience ACEs. LV‐GLS remained associated with ACEs after adjustment for non‐localized and midwall LGE. Conclusion: In AM presenting with LVEF ≥50%, LV‐GLS provides independent prognostic value over LGE characterization, improving risk stratification and representing a rationale for further studies of therapy in this cohort. [ABSTRACT FROM AUTHOR]

Aims: Cardiac implantable electronic device (CIED) therapy is fundamental to the management of LMNA cardiomyopathy due to the high frequency of atrioventricular block and ventricular tachyarrhythmias. We aimed to define the role of cardiac resynchronization therapy (CRT) in impacting heart failure in LMNA cardiomyopathy. Methods and results: From nine referral centres, LMNA cardiomyopathy patients who underwent CRT with available pre‐ and post‐echocardiograms were identified retrospectively. Factors associated with CRT response were identified (defined as improvement in left ventricular ejection fraction [LVEF] ≥5% 6 months post‐implant) and the associated impact on the primary outcome of death, implantation of a left ventricular assist device or cardiac transplantation was assessed. We identified 105 patients (mean age 51 ± 10 years) undergoing CRT, including 70 (67%) who underwent CRT as a CIED upgrade. The mean change in LVEF ∼6 months post‐CRT was +4 ± 9%. A CRT response occurred in 40 (38%) patients and was associated with lower baseline LVEF or a high percentage of right ventricular pacing prior to CRT in patients with pre‐existing CIED. In patients with a European Society of Cardiology class I guideline indication for CRT, response rates were 61%. A CRT response was evident at thresholds of LVEF ≤45% or percent pacing ≥50%. There was a 1.3 year estimated median difference in event‐free survival in those who responded to CRT (p = 0.04). Conclusion: Systolic function improves in patients with LMNA cardiomyopathy who undergo CRT, especially with strong guideline indications for implantation. Post‐CRT improvements in LVEF are associated with survival benefits in this population with otherwise limited options. [ABSTRACT FROM AUTHOR]

B This article refers to 'Genetic and phenotypic profiling of supranormal ejection fraction reveals decreased survival and underdiagnosed heart failure' by I.S. Forrest I et al i ., published in this issue on pages 2118-2127. b In the last years, a fourth category of heart failure (HF), characterized by supra-normal left ventricular ejection fraction (LVEF) >65% (snLVEF), termed HF with snLVEF (HFsnEF), made its debut in the clinical arena. Other unknown features, however, may characterize patients with snLVEF, as HF medications, when present, only mildly attenuate the association with mortality.[6] Clinical cardiologists in fact may not feel themselves at ease when trying to identify these patients among those normally encountered in a HF clinic. Both snLVEF patients and snLVEF-GRS were associated with HF symptoms and medications, and elevated HF biomarkers, but not with HF diagnosis, supporting the hypothesis of its underdiagnosis. [Extracted from the article]

Abstract Background Left bundle branch block (LBBB) negatively affects prognosis in heart failure patients with a reduced left ventricular ejection fraction (LVEF). Less is known about the prognostic role of LBBB in dilated cardiomyopathy (DCM) with intermediate LVEF (between 36% and 50%). We sought to assess the role of LBBB in optimally treated DCM patients with mildly to moderately reduced LVEF and to determine the possible variables associated with subsequent LVEF reduction. Methods We retrospectively analyzed DCM patients with LVEF >35% after 3-to-9 months of optimal medical treatment (OMT) consecutively evaluated from 1990 to 2010. All-cause mortality or heart transplantation (D/HTx) and sudden cardiac death (SCD) or major ventricular arrhythmias (MVA) were considered as outcome measures. LVEF deterioration during follow-up was also considered. Results Among 280 (49%) patients that met the study criteria, 76 had LBBB (27%). During a mean follow-up of 151 months, the rates of D/HTx and SCD/MVA were similar between LBBB and not LBBB patients (p value = 0.52 and p = 0.39, respectively). Twenty-six out of 76 (34%) patients with LBBB experienced LVEF deterioration below 36%. The persistence of moderate-severe mitral regurgitation (MR), left atrial end-systolic area index and LV end-diastolic volume index emerged as independent predictors of LVEF deterioration and were associated with an increased risk of D/HTx during follow-up. Conclusions LBBB does not affect mortality in DCM patients with intermediate LVEF after OMT. However, among these patients those with persistent significant MR, left atrial and LV remodeling carries a higher risk of LVEF deterioration during follow-up. Highlights • LBBB does not affect mortality in DCM with intermediate LVEF after OMT. • One third of DCM patients with intermediate LVEF and LBBB experience LVEF worsening. • Significant MR, LAESAI and LVEDVI are independent predictors of LVEF worsening. [ABSTRACT FROM AUTHOR]

Background: Acute myocardial infarction (AMI) survivors are at risk of major adverse cardiac events and their risk stratification is a prerequisite to tailored therapeutic approaches. Biomarkers could be of great utility in this setting. Methods: We sought to evaluate the utility of the combined assessment of Galectin 3 (Gal-3) and Galectin 3 binding protein (Gal-3bp) for post-AMI risk stratification in a large, consecutive population of AMI patients. The primary outcomes were: Recurrent angina/AMI and all-cause mortality at 12 months after the index event. Results: In total, 469 patients were included. The median Gal-3bp was 9.1 μg/mL (IQR 5.8–13.5 μg/mL), while median Gal-3 was 9.8 ng/mL (IQR 7.8–12.8 ng/mL). During the 12 month follow-up, 34 patients died and 41 had angina pectoris/reinfarction. Gal-3 was associated with all-cause mortality, while Gal-3bp correlated with the risk of angina/myocardial infarction even when corrected for other significant covariates. The final multivariable model for mortality prediction included patients' age, left ventricular ejection fraction (LVEF), Gal-3, and renal function. The ROC curve estimated for this model has an area under the curve (AUC) of 0.84 (95%CI 0.78–0.9), which was similar to the area under the ROC curve obtained using the GRACE score 1-year mortality. Conclusions: The integrated assessment of Gal-3 and Gal-3bp could be helpful in risk stratification after AMI. [ABSTRACT FROM AUTHOR]

Implantable cardioverter-defibrillators (ICD) have a pivotal role in preventing major arrhythmic events in patients with severely reduced left ventricular ejection fraction (LVEF). Device replacement strategy is still controversial in patients without severely reduced left ventricular ejection fraction (LVEF) at the end of battery life. To evaluate the long-term arrhythmic outcome of patients with ICD or and cardiac resynchronization therapy defibrillators (CRT-D) with normal or intermediate LVEF at the time of device replacement. All consecutive patients with reduced ejection fraction heart failure, both from ischemic and non-ischemic origin, implanted with ICD or CRT-D for primary prevention from 2002 to 2009, were considered. The study population included patients without previous ICD interventions and without severe dysfunction (i.e. LVEF ≥ 35%) 60 [53–65] months after implantation (average battery duration). The outcome measure was the occurrence of appropriate ICD interventions in the long-term. Among the 255 patients (150 ICDs; 105 CRT-D) evaluated, 45 (18%) had LVEF ≥ 35% without ICD interventions 5-year follow-up after implantation (15 ICD [10%]; 30 CRT-D [29%]). In the long term, ICD interventions were 4 (27%) in the ICD group and 5 (17%) in the CRT-D group. Despite the absence of severe left ventricular dysfunction at the time of battery replacement, a not negligible number of patients with ICD and CRT-D maintained a high risk of appropriate interventions in the long term, suggesting the opportunity of replacing the ICD regardless the amount of LV dysfunction. • Risk-benefit balance of ICD replacement is still controversial. • Identifying patients at risk for arrhythmic events during follow-up is essential. • Improved patients appears still at risk of ventricular arrhythmias during follow-up • Device replacement is still recommended at the time of battery replacement. [ABSTRACT FROM AUTHOR]