The prognostic value of left atrial (LA) volume is well-established in acute myocardial infarction (AMI) patients. LA strain provides further patophysological insights. In the present study, we evaluated LA volume and LA strain in AMI patients including those with atrial fibrillation (AF). The aim of the study was to determine if LA strain provide additional prognostic value. Patients with AMI underwent two-dimensional echocardiography within 72 h of admission. The primary outcome was a composite of all-cause mortality and major adverse cardiovascular events. Cox regression analyses were performed. We included 501 patients and during follow-up, 132 patients (26.4%) met the primary outcome. Left ventricular (LV) global longitudinal strain (GLS) (HR 0.94 [95% CI 0.88–0.99], p = 0.029), indexed LA volume (LAVi) (HR 1.02 [95% CI 1.00–1.04], p = 0.015), and LA reservoir strain (HR 0.96 [95% CI 0.93–0.99], p = 0.017) were all independently associated with the primary outcome. A univariate Cox model conducted on the AF patients (n = 32) revealed that LA reservoir strain remained significantly associated with the primary outcome, while LV GLS and LAVi were not significant. The prognostic value of LA reservoir strain was comparable to LA volume and LV GLS, and might even be better in AF patients.

Abstract Background The left atrial (LA) volume has been demonstrated to be an important predictor of adverse outcome in patients with various cardiac conditions, including acute myocardial infarction (AMI). However, new treatment strategies in patients with AMI have led to better patient outcomes. We hypothesised that increased LA size could still predict mortality in patients with AMI despite improved treatment strategies. Methods We included patients with AMI in a prospective multicenter cohort study and the study patients were enrolled from 2014 to 2022. We recorded echocardiographic and clinical data during their index hospitalisation. Indexed LA volume (LAVi) was assessed in all patients and was used as a continuous variable in the univariate and multivariate Cox regression analysis. The study took place over a period of five years and median follow-up time was 3.8 years (range 3.1 to 5.0 years). The primary study outcomes were all-cause mortality and major adverse cardiac events (MACE). MACE was defined as hospital readmission due to myocardial infarction, cardiac arrest, stroke, heart failure, or onset of new atrial fibrillation. Results We included 487 patients (69 ± 12 years old, 26% female) with AMI. During the follow-up period all-cause mortality was 50 (10.3%) and patients who reached the primary outcomes were 153 (31.4%). The deceased patients had higher LAVi compared to survivors (40.0 ± 12.9 mL/m2 vs. 29.7 ± 11.2 mL/m2, p < 0.001). Factors associated with all-cause mortality and MACE were age, year of enrollment, left ventricular (LV) ejection fraction, LV global longitudinal strain (GLS), LV filling pressure, moderate or severe mitral regurgitation and LAVi. GLS and EF were segregated into two distinct models due to their moderately high correlation (r = 0.57, p < 0.001). LAVi remained as an independent echocardiographic predictor of primary outcomes after adjusting for the covariates above in two separates multivariable Cox regression models (hazard ratio 1.02/1.02 mL/m2 [95% CI 1.01–1.03/1.01–1.03], p = 0.006/0.003). Conclusions Our study demonstrated that LA dilatation is an independent echocardiographic predictor of mortality and MACE in patients with AMI despite improved treatment strategies. This finding highlights the potential of using LAVi as a marker for prognostication in these patients.

AIMS:Permanent pacemaker (PM) implantation is common after transcatheter aortic valve implantation (TAVI). Left ventricular mechanical dispersion (MeDi) by speckle tracking echocardiography is a marker of fibrosis that causes alterations in the conduction system. We hypothesized that MeDi can be a predictor of the need for PM implantation after TAVI. METHODS AND RESULTS:Consecutively, 200 TAVI patients were enrolled. Transthoracic echocardiography and electrocardiography examinations were recorded before TAVI to evaluate global longitudinal strain (GLS), MeDi, and conduction disturbances. PM implantation information was obtained 3 months after TAVI. Patients were stratified into PM or no PM group. Mean age was 80 + 7 years (44% women). Twenty-nine patients (16%) received PM. MeDi, QRS duration, existence of right bundle branch abnormality (RBBB), and first-degree atrioventricular (AV) block were significantly different between groups. MeDi was 57 ± 15 ms and 48 ± 12 ms in PM and no PM groups, respectively (P < 0.001). In multivariate analysis, MeDi predicted the need for PM after TAVI independently of GLS, QRS duration, RBBB, and first-degree AV block [odds ratio (OR): 1.73, 95% confidence interval (CI): 1.22-2.45] with an area under the curve (AUC) of 0.68 in receiver operating characteristic (ROC) curves. Moreover, RBBB was an independent predictor of PM need after TAVI (OR: 8.98, 95% CI: 1.78-45.03). When added to RBBB, MeDi had an incremental predictive value with an AUC of 0.73 in ROC curves (P = 0.01). CONCLUSION:MeDi may be used as an echocardiographic functional predictor of the need for PM after TAVI.

Clinical differentiation between athletes’ hearts and those with hypertrophic cardiomyopathy (HCM) can be challenging. We aimed to explore the role of speckle tracking echocardiography (STE) and cardiac magnetic resonance imaging (CMR) in the differentiation between athletes’ hearts and those with mild HCM. We compared 30 competitive endurance elite athletes (7% female, age 41 ± 9 years) and 20 mild phenotypic mutation-positive HCM carriers (15% female, age 51 ± 12 years) with left ventricular wall thickness 13 ± 1 mm. Mechanical dispersion (MD) was assessed by means of STE. Native T1-time and extracellular volume (ECV) were assessed by means of CMR. MD was higher in HCM mutation carriers than in athletes (54 ± 16 ms vs. 40 ± 11 ms, p = 0.001). Athletes had a lower native T1-time (1204 (IQR 1191, 1234) ms vs. 1265 (IQR 1255, 1312) ms, p < 0.001) and lower ECV (22.7 ± 3.2% vs. 25.6 ± 4.1%, p = 0.01). MD > 44 ms optimally discriminated between athletes and HCM mutation carriers (AUC 0.78, 95% CI 0.65–0.91). Among the CMR parameters, the native T1-time had the best discriminatory ability, identifying all HCM mutation carriers (100% sensitivity) with a specificity of 75% (AUC 0.83, 95% CI 0.71–0.96) using a native T1-time > 1230 ms as the cutoff. STE and CMR tissue characterization may be tools that can differentiate athletes’ hearts from those with mild HCM.

The potential association between endurance exercise and myocardial fibrosis is controversial. Data on exercise exposure and diffuse myocardial fibrosis in endurance athletes are scarce and conflicting. We aimed to investigate the association between exercise exposure and markers of diffuse myocardial fibrosis by cardiovascular magnetic resonance imaging (CMR) in endurance athletes. We examined 27 healthy adult male competitive endurance athletes aged 41 ± 9 years and 16 healthy controls in a cross sectional study using 3 Tesla CMR including late gadolinium enhancement and T1 mapping. Athletes reported detailed exercise history from 12 years of age. Left ventricular total mass, cellular mass and extracellular mass were higher in athletes than controls (86 vs. 58 g/m2, 67 vs. 44 g/m2 and 19 vs. 13 g/m2, all p < 0.01). Extracellular volume (ECV) was lower (21.5% vs. 23.8%, p = 0.03) and native T1 time was shorter (1214 ms vs. 1268 ms, p < 0.01) in the athletes. Increasing exercise dose was independently associated with shorter native T1 time (regression coefficient − 24.1, p < 0.05), but expressed no association with ECV. Our results indicate that diffuse myocardial fibrosis has a low prevalence in healthy male endurance athletes and do not indicate an adverse dose–response relationship between exercise and diffuse myocardial fibrosis in healthy athletes.

Background:Several reports exist of an acquired exercise-induced arrhythmogenic cardiomyopathy. Little is known about myocardial disease progression and arrhythmia prediction in this population. Objective:The study sought to explore the evolution of myocardial function and structure and its relation to incident life-threatening ventricular arrhythmias (VA), to identify markers of impending events. Methods:We included athletes (individuals with exercise doses >24 metabolic equivalent of task hours per week, >6 consecutive years, participating in organized and competitive sports) who had VA, absence of family history and known genetic variants associated with cardiac disease, and no other identified etiology, in a tertiary referral single-center, longitudinal cohort study of patients with exercise-induced arrhythmogenic cardiomyopathy (EiAC). Evolution of myocardial function and structure was assessed by repeated echocardiographic examinations during long-term follow-up. Life-threatening VA were assessed at baseline and during long-term follow-up. Results:Forty-one EiAC patients (15% women, age 45 ± 13 years) were followed for 80 (interquartile range 48-115) months. There were no changes in myocardial function or structure in the overall population during follow-up. We observed high incidence rate and high recurrence rate of life-threatening VA in EiAC patients. Subtle deterioration of right ventricular function was strongly associated with subsequent first-time VA (odds ratio 1.12, 95% confidence interval 1.01-1.25, P = .031, per 1% deterioration of right ventricular free wall longitudinal strain). Conclusion:There were no clear changes in myocardial function or structure during follow-up in the overall population, but there was a high incidence rate and high recurrence rate of life-threatening VA. Subtle right ventricular deterioration by free wall longitudinal strain was a strong predictor of impending first-time life-threatening VA during follow-up.

BackgroundObstructive sleep apnoea (OSA) can cause left atrial (LA) and left ventricular (LV) remodelling, which is linked to atrial fibrillation (AF). Whether continuous positive airway pressure (CPAP) can reverse LA and LV remodelling in patients with OSA and paroxysmal AF (PAF) has yet to be studied. We assessed the impact of CPAP treatment on LA and LV size and function in patients with OSA and PAF before and after catheter ablation.MethodsIn a randomised controlled trial, we screened patients with PAF for OSA. We enrolled patients with an Apnoea–Hypopnoea Index ≥15/hour. The burden of AF was monitored by an implantable loop recorder in all patients. Patients were then randomised to CPAP treatment or standard care. Transthoracic echocardiography was performed at baseline and after 6 and 12 months to assess LV and LA function and remodelling with advanced echocardiographic imaging techniques.ResultsWe enrolled 109 patients (63±7 years, body mass index 29.6±4.3, 76% men). 83 patients were scheduled for pulmonary vein isolation (PVI) and 26 for clinical follow-up only. 55 patients were randomised to CPAP and 54 to standard care. The burden of AF decreased significantly in patients who underwent PVI irrespective of treatment with CPAP (p for difference ≤0.001). Patients in the study group had LV ejection fraction (LVEF) and LV global longitudinal strain (GLS) within the normal range, increased LA Volume Index (LAVI), LA volume (by speckle tracking) and decreased LA reservoir strain at baseline. We did not observe any improvement in LVEF, GLS, LAVI, LA volume or LA reservoir strain in either group during the 12 months of follow-up.ConclusionsIn patients with PAF and OSA, treatment with CPAP was not associated with reverse LA remodelling within 12 months of follow-up.

Landmark studies on heart failure patients with prolonged QRS duration on the electrocardiogram (ECG) demonstrated that biventricular pacing (BIVP) was superior to right ventricular pacing (RVP).1 Detrimental long-term effects and unpredictable acute hemodynamic effect to RVP led to the existing RVP skepticism, and BIVP was favored as the standard of care implementing cardiac resynchronization therapy (CRT).2 However, despite reduction in mortality and morbidity, one third of eligible patients do not benefit from BIVP demonstrating the shortcoming of QRS duration and morphology to predict CRT response.

PurposeThe risk of sudden cardiac death (SCD) is increased during endurance competitive sports. Coronary artery disease (CAD) is the most common cause of SCD in master athletes >= 35 years old (MAs). To reduce the risk of SCD self-assessment of symptoms by questionnaire, and evaluation of cardiovascular risk-score, are recommended as pre-participation cardiovascular evaluation (PCVE). We aimed to examine whether PCVE predicts CVD in MAs with or without increased risk as measured by validated score instruments.MethodsWe performed a single-site observational cohort study of healthy MAs based on findings at PCVE. They were allocated in two different groups: those MAs with reported symptoms on the questionnaire and/or with elevated cardiovascular risk score were allocated to a symptom group (SG), while MAs with no symptoms, nor raised risk score were defined as control group (CG). Thereafter, all were examined with extended examinations: resting-ECG, cardiorespiratory exercise testing and echocardiography.ResultsTotal, 81 (18 women) MAs participated in the study. There were no differences at baseline between SG (n = 39) and CG (n = 42); sex (p = 0.11), age (55.0 +/- 9.8 vs. 51.9 +/- 11.1 years; p = 0.18), maximal oxygen uptake (49.8 +/- 7.6 vs. 51.6 +/- 7.0 ml/kg/min; p = 0.26), resting heart rate (61.4 +/- 12.8 vs. 60.2 +/- 11.0/min; p = 0.66), training hours/week (7.0 +/- 3.2 vs. 7.1 +/- 3.1; p = 0.88). After further examination, sixteen (20%) MAs were found with CVD: 12 in SG, 4 in CG (p = 0.024). The negative predictive value and specificity of the PCVE were 90% and 58%, respectively.ConclusionNegative findings on PCVE by questionnaire and cardiovascular risk-score may be a strategy to exclude subjects from preparticipation screening, thus saving resources.

Background The optimal antithrombotic therapy after transcatheter aortic valve implantation (TAVI) is unknown. Bio-prosthetic valve dysfunction (BVD) is associated with adverse outcomes and may be prevented by anticoagulation therapy. A dedicated randomized trial comparing monotherapy NOAC to single antiplatelet therapy has not been performed previously. We hypothesize that therapy with any anti-factor Xa NOAC will reduce BVD compared to antiplatelet therapy, without compromising safety.Methods ACASA-TAVI is a multicenter, prospective, randomized, open-label, blinded endpoint, all-comers trial com-paring a monotherapy anti-factor Xa NOAC strategy ( intervention arm ) with a single antiplatelet therapy strategy (control arm ) after successful TAVI. Three-hundred and sixty patients without indication for oral anticoagulation will be randomized in a 1:1 ratio to either apixaban 5 mg twice per day, edoxaban 60 mg daily, or rivaroxaban 20 mg daily for 12 months followed by acetylsalicylic acid 75 mg daily indefinitely, or to acetylsalicylic acid 75 mg daily indefinitely. The 2 co-primary outcomes are (1) incidence of Hypo-Attenuated Leaflet Thickening ( HALT ) on 4-dimensional cardiac CT at 12 months, and (2) a Safety Composite of VARC-3 bleeding events, thromboembolic events (myocardial infarction and stroke), and death from any cause, at 12 months.Results The first 100 patients had a mean age of 74 +/- 3.6 years, 33% were female, the average body-mass index was 27.9 +/- 4.4 kg/m2, and 15% were smokers. A balloon-expanded valve was used in 82% and a self-expandable valve in 18%.Conclusions The trial is planned, initiated, funded, and conducted without industry involvement.Trial Registration ClinicalTrials.gov Identifier NCT05035277.

Introduction: Obstructive sleep apnea (OSA) is known to cause left atrial (LA) remodeling. The effect of continuous positive airway pressure (CPAP) on LA remodeling in patients with OSA and paroxysmal atrial fibrillation (AF) has not been assessed. Hypothesis: We aimed to assess the impact of CPAP treatment on reverse LA deformation and volume in patients with AF and OSA. Methods: In this secondary analysis of a prospective randomized controlled trial, we screened patients with AF for OSA. The majority of patients were referred for pulmonary vein isolation (PVI). We included AF patients with OSA defined as apnea-hypopnea index (AHI) > 15/h. Patients were randomized to CPAP treatment or standard care. Transthoracic echocardiography was performed to assess atrial remodeling by two-dimensional speckle tracking LA reservoir strain (by AFI LA) and LA volume index (LAVI) in apical 2- and 4-chamber views in sinus rhythm at baseline and at 12 months’ follow-up by an investigator blinded to allocated arm. AF burden was monitored by an implantable loop recorder in all patients. Results: We included 104 patients (62 ±7 years old, 77% men, BMI 30 ± 4) of whom 50 were randomized to CPAP and 54 to standard care. PVI was performed in 83 patients six months after the baseline exam. There was no clear improvement in LA reservoir strain in either group from baseline to follow up (CPAP: 30 ± 8 % vs 32 ± 9 %, p = 0.15; standard care: 28 ± 7 % vs 30 ± 6 %, p = 0.11), and there was no difference between the groups (p=0.41). Similarly, LAVI did not change in the CPAP group (38±8 mL/m 2 vs 36± 10ml/m 2 , p= 0.16) or in the standard care group (39± 10 mL/m 2 vs 37 ± 12 ml/m 2 , p= 0.20) during follow-up, with no difference between the two groups (p =0.62). In patients who underwent PVI, the AF burden decreased in both treatment arms, with no between-group difference (p = 0.69). Conclusions: In AF patients with OSA, treatment with CPAP did not improve reverse left atrial remodeling within 12 months. Although the trial was relatively small, these results suggest that CPAP is unlikely to have a major impact on normalization of LA function after atrial fibrillation ablation in this patient group.

Aims Arrhythmic mitral valve syndrome is linked to life-threatening ventricular arrhythmias. The incidence, morphology and methods for risk stratification are not well known. This prospective study aimed to describe the incidence and the morphology of ventricular arrhythmia and propose risk stratification in patients with arrhythmic mitral valve syndrome. Methods Arrhythmic mitral valve syndrome patients were monitored for ventricular tachyarrhythmias by implantable loop recorders (ILR) and secondary preventive implantable cardioverter-defibrillators (ICD). Severe ventricular arrhythmias included ventricular fibrillation, appropriate or aborted ICD therapy, sustained ventricular tachycardia and non-sustained ventricular tachycardia with symptoms of hemodynamic instability. Results During 3.1 years of follow-up, severe ventricular arrhythmia was recorded in seven (12%) of 60 patients implanted with ILR [first event incidence rate 4% per person-year, 95% confidence interval (CI) 2-9] and in four (20%) of 20 patients with ICD (re-event incidence rate 8% per person-year, 95% CI 3-21). In the ILR group, severe ventricular arrhythmia was associated with frequent premature ventricular complexes, more non-sustained ventricular tachycardias, greater left ventricular diameter and greater posterolateral mitral annular disjunction distance (all P < 0.02). Conclusions The yearly incidence of ventricular arrhythmia was high in arrhythmic mitral valve syndrome patients without previous severe arrhythmias using continuous heart rhythm monitoring. The incidence was even higher in patients with secondary preventive ICD. Frequent premature ventricular complexes, non-sustained ventricular tachycardias, greater left ventricular diameter and greater posterolateral mitral annular disjunction distance were predictors of first severe arrhythmic event.

Abstract Aims Patients with mitral valve prolapse (MVP) have high risk of life-threatening ventricular arrhythmias (VAs). Data on the impact of exercise on arrhythmic risk in these patients are lacking. We explored whether lifetime exercise dose was associated with severe VA and with established risk factors in patients with MVP. Furthermore, we explored the circumstances at the VA event. Methods and results In this retrospective cohort study, we included patients with MVP and assessed lifetime exercise dose as metabolic equivalents of task (MET) hours/week. Severe VA was defined as sustained ventricular tachycardia or fibrillation, aborted cardiac arrest, and appropriate shock by a primary preventive implantable cardioverter defibrillator. We included 136 MVP patients (48 years [interquartile range (IQR) 35–59], 61% female), and 17 (13%) had previous severe VA. The lifetime exercise dose did not differ in patients with and without severe VA (17 MET h/week [IQR 9–27] vs. 14 MET h/week [IQR 6–31], P = 0.34). Lifetime exercise dose > 9.6 MET h/week was a borderline significant marker for severe VA (OR 3.38, 95% CI 0.92–12.40, P = 0.07), while not when adjusted for age (OR 2.63, 95% CI 0.66–10.56, P = 0.17). Ventricular arrhythmia events occurred most frequently during wakeful rest (53%), followed by exercise (29%) and sleep (12%). Conclusion We found no clear association between moderate lifetime exercise dose and severe VA in patients with MVP. We cannot exclude an upper threshold for safe levels of exercise. Further studies are needed to explore exercise and risk of severe VA.

Abstract Funding Acknowledgements Type of funding sources: Public hospital(s). Main funding source(s): Oslo University Hospital, Procardio Center for Innovation Introduction Arrhythmogenic cardiomyopathy (AC) is an inheritable heart disease caused by mutations in genes encoding the cardiac desmosomes, while exercise-induced AC (EiAC) has been proposed as an acquired similar phenotype in athletes. Little is known about the progression of EiAC compared to AC. Purpose To assess functional and structural disease progression in EiAC compared to desmosomal AC during long-term follow-up. Methods We consecutively included probands with definite AC diagnosis according to the 2010 Task Force Criteria and patients with EiAC in a longitudinal cohort study. EiAC was diagnosed in competitive endurance athletes (>24 MET-hours/week for >6 consecutive years) referred with symptomatic ventricular arrhythmias who had no family history, no genetic mutations associated with heart disease, and no other identified etiology after through clinical work-up. All patients in both groups were recommended to avoid high intensity exercise. Progression of the structural phenotype was assessed by regular repeated echocardiographic examinations during long-term follow-up. Right ventricular (RV) function and size were assessed by RV fractional area change (FAC), RV basal diameter (RVD) and RV outflow tract (RVOT) diameter. Disease progression was evaluated and compared using linear mixed model regression. Results Forty-one EiAC patients (15% women, age 45±13 years) and 84 AC probands (51% mutation positive, 35% women, age 43±15) were followed for 6.6 (IQR 3.7-10.5) and 7.9 (IQR 5.2-10.8) years, respectively. Key parameters from 570 echocardiographic examinations (184 EiAC and 386 AC) were assessed. There was no deterioration of RV function during follow-up in EiAC patients, in contrast to AC patients (FAC yearly progression rate: EiAC +0.02% [95% CI -0.27 to 0.31] vs AC -0.60% [95% CI -0.71 to -0.50] per year, p=0.001, Figure left panel). RV size did not increase in EiAC patients in contrast to AC patients (RVD: EiAC +0.01 mm [95% CI -0.00 to 0.03] vs AC +0.78 mm [95% CI 0.68 to 0.89] per year, p<0.001, Figure mid panel, and RVOT: EiAC +0.01 mm [95% CI -0.01 to 0.02] vs AC +0.47 mm [95% CI 0.38 to 0.56] per year, p<0.001, Figure right panel). Conclusion Patients with exercise-induced AC had no evidence of disease progression during long-term follow-up. These patients had a more benign disease trajectory than patients with desmosomal AC. Despite the limited sample size, these results seem reassuring for patients diagnosed with exercise-induced AC.

Abstract Background & aims Echocardiography has a central role in diagnosis and follow-up of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). It is however not included in the risk calculator for ventricular arrhythmia (VA) which was recently developed and validate, since traditional echo parameters were outperformed by RV ejection fraction (RVEF) measured by CMR. CMR is however not as widely available as echocardiography. We aimed to investigate whether echocardiographic RV deformation imaging could be used as a substitute for RVEF by CMR in arrhythmic risk prediction in ARVC. Methods & results From two referral centres, 150 consecutive patients with a definite ARVC diagnosis, no prior sustained VA and an echocardiogram suitable for deformation analysis were included (aged 41 ± 17 years, 50% female). During a median follow-up of 6.3 (IQR 3.1-9.8) years, 37 (25%) experienced a first-time sustained VA. The current ARVC risk calculator, including RVEF by CMR performed well in this cohort, with an optimism corrected C-statistic of 0.77 (95% CI 0.71 – 0.84). When RVEF was replaced by RV free wall longitudinal strain, the latter was an independent predictor (p = 0.005) and C-statistic changed to 0.80 (95% CI 0.74 – 0.86). The model including RV deformation imaging reduced the Akaike information criterion by >2. Conclusions This study showed that echocardiographic RV deformation imaging is able to replace RVEF by CMR in ARVC risk prediction without losing discriminative power. In clinical use, repeated yearly risk assessment using echocardiography could be alternated with a CMR every few years.

Our objective was to compare long-term outcomes in patients with non-ST-elevation myocardial infarction (NSTEMI) and ST-elevation myocardial infarction (STEMI) between two time periods in Southern Norway. There are limited contemporary data comparing long-term follow-up after revascularization in the last decades. This prospective follow-up study consecutively included both NSTEMI and STEMI patients during two time periods, 2014–2015 and 2004–2009. Patients were followed up for a period of 5 years. The primary outcome was all-cause mortality after 1 and 5 years. A total of 539 patients with acute myocardial infarction (AMI), 316 with NSTEMI (234 included in 2014 and 82 included in 2007) and 223 with STEMI (160 included in 2014 and 63 included in 2004). Mortality after NSTEMI was high and remained unchanged during the two time periods (mortality rate at 1 year: 3.5% versus 4.9%, p = 0.50; and 5 years: 11.4% versus 14.6%, p = 0.40). Among STEMI patients, all-cause mortality at 1 year was reduced in 2014 compared to 2004 (1.3% versus 11.1%, p < 0.001; and 5 years: 7.0% versus 22.2%, p = 0.004, respectively). Time to coronary angiography in NSTEMI patients remained unchanged between 2014 and 2007 (28.2 h [IQR 18.1–46.3] versus 30.3 h [IQR 18.0–48.3], p = 0.20), while time to coronary angiography in STEMI patients was improved in 2014 compared with 2004 (2.8 h [IQR 2.0–4.8] versus 21.7 h [IQR 5.4–27.1], p < 0.001), respectively. During one decade of AMI treatment, mortality in patients with NSTEMI remained unchanged while mortality in STEMI patients decreased, both at 1 and 5 years.

AbstractAimsCardiac disease progression prior to first ventricular arrhythmia (VA) in LMNA genotype–positive patients is not described.Methods and resultsWe performed a primary prevention cohort study, including consecutive LMNA genotype–positive patients from our centre. Patients underwent repeated clinical, electrocardiographic, and echocardiographic examinations. Electrocardiographic and echocardiographic disease progression as a predictor of first-time VA was evaluated by generalized estimation equation analyses. Threshold values at transition to an arrhythmic phenotype were assessed by threshold regression analyses. We included 94 LMNA genotype–positive patients without previous VA (age 38 ± 15 years, 32% probands, 53% females). Nineteen (20%) patients experienced VA during 4.6 (interquartile range 2.1–7.3) years follow up, at mean age 50 ± 11 years. We analysed 536 echocardiographic and 261 electrocardiogram examinations. Individual patient disease progression was associated with VA [left ventricular ejection fraction (LVEF) odds ratio (OR) 1.4, 95% confidence interval (CI) 1.2–1.6 per 5% reduction, left ventricular end-diastolic volume index (LVEDVi) OR 1.2 (95% CI 1.1–1.3) per 5 mL/m2 increase, PR interval OR 1.2 (95% CI 1.1–1.4) per 10 ms increase]. Threshold values for transition to an arrhythmic phenotype were LVEF 44%, LVEDVi 77 mL/m2, and PR interval 280 ms.ConclusionsIncidence of first-time VA was 20% during 4.6 years follow up in LMNA genotype–positive patients. Individual patient disease progression by ECG and echocardiography were strong predictors of VA, indicating that disease progression rate may have additional value to absolute measurements when considering primary preventive ICD. Threshold values of LVEF <44%, LVEDVi >77 mL/m2, and PR interval >280 ms indicated transition to a more arrhythmogenic phenotype.

Arrhythmic symptoms are prevalent and life-threatening ventricular arrhythmias (VA) are a feared complication in patients with arrhythmic mitral valve syndrome (AMVS) (1). There is no established medical therapy to suppress VA and relieve arrhythmic symptoms in these patients beyond conventional beta-blocker therapy, which is often unsuccessful and not evaluated in randomized controlled trials. Catheter ablation can suppress VA in some patients, but is limited due to the multifocal nature of premature ventricular complexes (PVC), papillary muscle origin, and VA recurrence despite initial successful procedures.

BACKGROUND LMNA genotype-positive patients have high risk of experiencing life-threatening ventricular tachyarrhythmias (VTAs). The LMNA-risk VTA calculator published in 2019 has not been exter-nally validated. OBJECTIVE The purpose of this study was to validate the LMNA-risk VTA calculator. METHODS We included LMNA genotype-positive patients without previous VTAs from 2 large Scandinavian centers. Patients under-went electrocardiography, 24-hour Holter monitoring, and echocar-diographic examinations at baseline and repeatedly during follow-up. Validation of the LMNA-risk VTA calculator was performed using Harrell's C-statistic derived from multivariable Cox regression anal-ysis. RESULTS We included 118 patients (age 37 years [IQR 27-49 years]; 39 [33%] probands; 65 [55%] women; 100 [85%] with non-missense LMNA variants). Twenty-three patients (19%) experi-enced VTA during 6.1 years (interquartile range 3.0-9.1 years) follow-up, resulting in 3.0% (95% confidence interval 2.0%- 4.5%) yearly incidence rate. Atrioventricular block and reduced left ventricular ejection fraction were independent predictors of VTAs, while nonsustained ventricular tachycardia, male sex, and non-missense LMNA variants were not. The LMNA-risk VTA calculator showed 83% sensitivity and 26% specificity for identifying patients with VTAs during the coming 5 years, and a Harrell's C-statistic of 0.85, when applying >= 7% predicted 5-year VTA risk as threshold. The sensitivity increased to 100% when reevaluating risk at the time of last consultation before VTA. The calculator overestimated arrhythmic risk in patients with mild and moderate phenotype, particularly in men. CONCLUSION Validation of the LMNA-risk VTA calculator showed high sensitivity for subsequent VTAs, but overestimated arrhythmic risk when using >= 7% predicted 5-year risk as threshold. Frequent reevaluation of risk was necessary to maintain the sensitivity of the model.

BACKGROUND Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibrofatty replacement of primarily the right ventricular myocardium, a substrate for life-threatening ventricular arrhythmias (VAs). Repeated cardiac imaging of at-risk relatives is important for early disease detection. However, it is not known whether screening should be age-tailored. OBJECTIVES The goal of this study was to assess the need for age-tailoring of follow-up protocols in early ARVC by evaluating myocardial disease progression in different age groups. METHODS We divided patients with early-stage ARVC and genotype-positive relatives without overt structural disease and VA at first evaluation into 3 groups: age <30 years, 30 to 50 years, and >= 50 years. Longitudinal biventricular deformation characteristics were used to monitor disease progression. To link deformation abnormalities to underlying myocardial disease substrates, Digital Twins were created using an imaging-based computational modeling framework. RESULTS We included 313 echocardiographic assessments from 82 subjects (57% female, age 39 +/- 17 years, 10% probands) during 6.7 +/- 3.3 years of follow-up. Left ventricular global longitudinal strain slightly deteriorated similarly in all age groups (0.1%-point per year [95% CI: 0.05-0.15]). Disease progression in all age groups was more pronounced in the right ventricular lateral wall, expressed by worsening in longitudinal strain (0.6%-point per year [95% CI: 0.46-0.70]) and local differences in myocardial contractility, compliance, and activation delay in the Digital Twin. Six patients experienced VA during follow-up. CONCLUSIONS Disease progression was similar in all age groups, and sustained VA also occurred in patients aged >50 years without overt ARVC phenotype at first evaluation. Unlike recommended by current guidelines, our study suggests that follow-up of ARVC patients and relatives should not stop at older age. (c) 2023 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).