Citations

Showing 10 of 107930 citations.

Abstract 4365908: A Novel Composite Artificial Intelligence-Electrocardiography Risk Score Is Independently Associated with Mortality in Chronic Tricuspid Regurgitation
Wood, Julia, Friedman, Paul A. , Thaden, Jeremy J. , Kane, Garvan C. , Anand, Vidhu , Ciobanu, A. , Naser, Jwan A. , Wood, Julia, Vinereanu, Dragoş , Nkomo, Vuyisile T. , Pislaru, Cristina , Lara-Breitinger, Kyla M , Pellikka, Patricia A. , Pislaru, Sorin V.

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4365908 Get It

Background: Tricuspid regurgitation (TR) is associated with significant morbidity, but individualized treatment and timing of intervention are yet to be defined. We previously proposed and validated artificial intelligence electrocardiography (AI-ECG) scores for various diseases. Whether incorporating AI-ECG information adds to risk stratification above and beyond classical clinical parameters is unknown. Objectives: To identify AI-ECG scores independently associated with all-cause mortality in chronic TR and to create a composite AI-ECG risk score. Methods: Patients with a first echocardiographic diagnosis of ≥moderate TR between 2005-2016 and an ECG within ±15 days were included. The Tricuspid Regurgitation Impact on Outcome (TRIO) score was calculated as previously described based on 8 simple parameters (age, sex, severe TR, heart failure, lung disease, heart rate, creatinine and AST). AI-ECG probabilities of low ejection fraction, aortic stenosis, amyloid, cirrhosis, atrial fibrillation and hypertrophic cardiomyopathy were estimated by existing algorithms; the first 4 were associated with all-cause mortality and a composite AI-ECG risk score was calculated (0-4; 1 point for each AI-ECG above specific threshold). Results: A total of 12,377 pts were included (age 72±13 yrs, 55% women). Over a median follow-up of 1.3 yrs (IQR 0.1 to 4.4), 7,121 pts (58%) died. Mortality risk increased significantly with higher composite AI-ECG risk score (Fig. 1A). On multivariable analysis (proportional hazards method), the composite AI-ECG risk score remained independently associated with death, even after adjusting for age, sex, high NT-proBNP levels (>2,500 pg/mL), right ventricular systolic pressure (RVSP), diuretic use and TRIO risk score category (Fig. 1B). Conclusions: Patients with ≥moderate TR show progressively worse survival with increasing composite AI-ECG risk score. Incorporating AI-derived information from a simple and inexpensive ECG may identify patients with the highest risk of mortality beyond clinical risk scores as TRIO or classical clinical, laboratory and echocardiographic parameters. Prospective validation of this approach needs to be tested in future trials.
Abstract 4361592: State-Level Syncope Driving Restrictions and Crash Risk in Older Adults: A National Comparative Analysis
Dreher, Luke , Mishra, Nandini, Shen, Win K., Sorajja, Dan , Mishra, Nandini

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4361592 Get It

Background: Syncope is a leading cause of transient loss of consciousness, especially in older adults. Post-syncopal driving restrictions are widely recommended, but the required duration varies by state. The impact of these policies on motor vehicle crash outcomes remains unclear. Methods: We conducted a cross-sectional analysis of 45 U.S. states, comparing crash and fatality data between states requiring < 6 months vs. ≥ 6 months of driving restriction following a syncopal episode. Data were obtained from Fatality Analysis Reporting System, National Highway Traffic Safety Administration, state Department of Motor Vehicles, and medical boards. Crash rates were normalized by licensed drivers and population. Statistical comparisons were made using Welch’s t-tests. Results: States with ≥ 6-month restrictions had significantly lower crash rates among older drivers (0.0171 vs. 0.0256, p = 0.0076). Relative crash ratios comparing older to younger drivers were also lower (0.220 vs. 0.306, p = 0.067). Overall crash rates trended lower in states with ≥ 6-month restrictions in both per licensed driver ( p = 0.054) and per population ( p = 0.099). No significant differences were observed in fatality rates. Conclusions: Longer post-syncope driving restriction durations (≥ 6 months) are associated with reduced crash risk among older drivers and a narrowing of age-related disparities in crash rates. These findings suggest potential public health value in adopting more uniform, evidence-based restriction policies following syncopal events for older drivers.
Abstract 4361539: Long-term Outcomes of Leadless versus Transvenous Single-chamber Pacemaker in Elderly Patients
Sin, Yuh Miin, Singh, Ravnit, Deshmukh, Abhishek , Sroubek, Jakub , Wazni, Oussama M. , Lee, Justin Z. , Tan, Min Choon , Vignarajah, Aravinthan , Sin, Yuh Miin, DeSimone, Christopher V. , Santangeli, Pasquale

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4361539 Get It

Background: Leadless pacemaker has emerged as a promising alternative to traditional transvenous pacemaker (PM) among elderly patients who are at high risk of complications. Objective: This study aims to assess and compare 5-year clinical outcomes of leadless PM versus transvenous single-chamber PM in elderly patients using a multi-national dataset. Methods: Using the TriNetX Analytics Research Network, we included patients aged ≥65 years who underwent leadless versus transvenous single-chamber PM implantation between 1/1/2014 and 1/1/2020. Propensity score matching (PSM) was performed by using patient’s demographic, LVEF, cardiac comorbidities, and medications. The study outcomes included all-cause mortality and adverse cardiovascular events over a 5-year follow-up period. Results: After PSM, 7,501 patients in each group were analyzed. Leadless PM was associated with a significantly lower odds of 5-year all-cause mortality (odds ratio [OR]: 0.534; 95% confidence interval [CI]: 0.497-0.575), heart failure exacerbation (OR: 0.779, 95% CI: 0.721-0.841), major bleeding (OR: 0.666, 95% CI: 0.604-0.734), cardiac arrest (OR: 0.747, 95% CI: 0.646-0.864) and stroke (OR: 0.825, 95% CI: 0.750-0.908) compared to transvenous single-chamber PM. No significant difference was observed between the two groups in all-cause hospitalization (OR: 1.006, 95%CI: 0.938-1.079), cardiac thrombus (OR: 1.077, 95%CI: 0.791-1.465) or venous thromboembolism (OR: 1.028, 95%CI: 0.916-1.153). Conclusion: Leadless PM was associated with significantly improved mortality and cardiovascular outcomes compared to transvenous single-chamber PM in elderly patients. These findings support the strong consideration of leadless pacing as a strategy to reduce mortality and improve clinical outcomes in this high-risk population.
Abstract 4370453: Incidence and Predictors of Shock in Patients with Noncompaction Cardiomyopathy and Implantable Cardioverter-Defibrillators: A Multicenter Retrospective Study.
Raslan, Mohamed , Youssef, Amal, Dreher, Luke , Abdalla, Hesham, Sahwan, Oudai , Raslan, M. Alaa, Nabi, Hussein Abdul , Youssef, Amal, Baqal, Omar J , Masry, Hicham El

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4370453 Get It

Background: Noncompaction cardiomyopathy (NCCM) is a distinct myocardial disorder associated with a high risk of ventricular arrhythmias and sudden cardiac death. Although implantable cardioverter-defibrillators (ICDs) are frequently used in this population, data on predictors of shock therapy remain limited. Aim: Evaluation of a large cohort of patients with NCCM and ICDs to identify incidence and clinical factors associated with ICD shock Methods: This retrospective cohort study included patients with confirmed NCCM between 2002 and 2023 at all Mayo Clinic sites. Baseline clinical data, genetic findings, cardiac imaging parameters, and device-related outcomes were collected through detailed chart review. Multivariable Logistic regression was used to identify independent predictors. Results: Among 145 patients with NCCM who received ICDs, the median age was 45 years (IQR: 34–56), with 75 (52%) female and 120 (83%) White. The median left ventricular ejection fraction (LVEF) was 35% (IQR: 22.5–47.5%), and the median follow-up from ICD implantation was 6.4 years (IQR: 2.9–11.0). Most ICDs (124, 85.5%) were implanted for primary prevention. Appropriate shocks were observed in 37 patients (25.5%), with 14 (9.8%) experiencing three or more appropriate shocks. Inappropriate shocks occurred in 15 patients (10.3%). Device-related complications were reported in 22 patients (15.2%), including infections in 4 (2.8%), lead fractures or dislodgement in 17 (11.9%), and pneumothorax in 1 (0.7%). Clinical predictors of shocks are illustrated in the figure. Conclusion: ICD therapy for NCCM patients prevented SCD in 25.5% of patients. Syncope, RBBB, troponin T >30 ng/L, and imaging-based apical trabeculations were independently associated with ICD shocks in patients with NCCM. These factors may aid in risk stratification and clinical decision-making.
Abstract 4369484: Evaluating the Impact of Lutetium-177 Therapy on Bioprosthetic Heart Valves in Carcinoid Heart Disease
Abdelfattah, Fatmaelzahraa , Ibrahim, Omar , Esfahani, Sogol Attaripour , Ali, Nima Baba , Sheashaa, Hesham, Razaghi, Mahshad , Arsanjani, Reza, Ayoub, Chadi , Scalia, Isabel G. , Farina, Juan M. , Awad, Kamal , Pereyra, Milagros , Abbas, Mohammed Tiseer , Arsanjani, Reza

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4369484 Get It

Background: High serotonin from neuroendocrine tumors (NETs) can result in carcinoid heart disease (CaHD) with severe valve dysfunction, often requiring valve replacement surgery. Lutetium-177 (Lu-177), a targeted radioactive isotope therapy, is increasingly utilized in the treatment of NETs; however, there is limited data on its impact on bioprosthetic heart valves (BHVs) in CaHD. Methods: A retrospective review was conducted on all patients with stage IV metastatic NET who underwent BHV replacement at three tertiary centers in the United States. Patients were stratified by Lu-177 therapy, administered after valve replacement. A propensity matched cohort of CaHD patients with BHV who did not received Lu-177 was curated including age, sex, and baseline left ventricular ejection fraction. Serial transthoracic echocardiograms (TTEs) following surgery were evaluated for progression of BHV degeneration; rise in mean gradient or increase in grade of prosthetic/periprosthetic regurgitation (grade increase of ≥1 out of 5), from first TTE following surgery (TTE1) to most recent TTE (TTE2). Analysis was performed at the level of individual valves, recognizing that some patients had more than one valve implanted. To account for changes in Lu-177 exposure over time, we constructed a time-varying Cox proportional hazards model. Accounting for non-independence of multiple valves within the same patient, a clustered Cox model was fitted using robust standard errors. Mortality was compared between patients exposed to Lu-177 therapy and those not exposed. Results: 184 patients were identified, mean age 60.4 ± 10.3 years, 53.3 % male. Of these, 16 patients had Lu-177 therapy, with 45 individual BHVs within this group. Patients in the control group had a total of 60 BHVs. Across a median of 2.09 years [0.77-5.01] between TTE1 and TTE2, BHVs exposed to Lu-177 therapy demonstrated an increased risk of BHV degeneration (HR=2.44, 95% CI 1.05–5.69, p=0.038), accounting for the time between TTE1 and administration of Lu-177 and the non-independence of multiple BHVs within the same patient. There was no significant difference in mortality between the two cohorts (p=0.440). Conclusions: BHVs in patients with CaHD exposed to Lu-177 therapy demonstrated an increased risk of degeneration. These findings suggest an association between Lu-177 treatment and accelerated prosthetic valvular deterioration, warranting further investigation and close monitoring in this patient population.
Abstract 4369439: Concurrent Quadruple Valve Replacement and Patent Foramen Ovale Closure for Carcinoid Heart Disease
Schmitz, Katlin , Elhadi, Muhammed

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4369439 Get It

Introduction: Carcinoid cancer is a rare neuroendocrine tumor (NET) secreting vasoactive substances. Case Presentation: A 66-year-old woman presented to cardiology clinic for evaluation of dyspnea with exertion and leg swelling. Comorbidities included chronic kidney disease and a history of jejunal neuroendocrine tumor (NET) with prior resection and recent recurrence complicated by peritoneal carcinomatosis. A transthoracic echocardiogram was obtained at time of recurrence (1 year prior to presentation) and showed mild mitral regurgitation (MR), mild bi-atrial enlargement, moderate tricuspid regurgitation (TR), and mild aortic regurgitation (AR) with preserved ejection fraction. In clinic, physical exam showed jugular venous distention with prominent V waves and a systolic and diastolic murmur. Transthoracic echocardiogram (TTE) demonstrated severe TR, moderate to severe AR, moderate MR, severe pulmonary valve regurgitation, and a right to left shunt with enlarged right ventricle and reduced function. Patient was diagnosed with left and right carcinoid heart disease involving all 4 cardiac valves in the setting of a patent foramen ovale (PFO). After multidisciplinary discussion, surgical intervention was recommended. She underwent successful cardiopulmonary bypass with mitral valve replacement, aortic valve, pulmonary valve replacement, and tricuspid valve replacement in addition to closure of PFO. She did well postoperatively and was stable for dismissal from the hospital after 12 days. Discussion: Rarely, carcinoid heart disease can involve the left side of the heart. Left sided involvement is typically seen in the presence of a right to left side cardiac shunt. In patients with severe valve dysfunction secondary to carcinoid heart syndrome, surgical intervention is the definitive treatment and has been found to improve quality of life and prolong survival
Abstract 4366386: The Impact of Race/Ethnicity, Education, and Income on Patient Engagement in Adult Congenital Heart Disease Care
Rivera, Andrea Sandoval, Valente, J. , Banala, Keerthana Reddy , Duan, Rong, Alano, Lindsay, Parang, Kim , Jaimes, Katia Bravo, Moons, Philip , Okumura, Megumi J. , Gurvitz, Michelle , Agarwal, Anushree , Rivera, Andrea Sandoval, Valente, Joseph, Norris, Mark, Reardon, Leigh Christopher

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4366386 Get It

Background: It is currently unknown whether patient engagement in adult congenital heart disease (CHD) care is influenced by social determinants of health (SDOH) such as race/ethnicity, education, and income. Adapting from the chronic care model, patient engagement, defined as the knowledge, skills, ability, and willingness to manage one’s health, can minimize lapses in care and improve CHD care outcomes. Methods: This cross-sectional study analyzes data from patients aged 18 and older with a self-reported diagnosis of CHD enrolled in a digital health research study called Empower My Congenital Heart. Patient engagement was measured using the Gothenburg Empowerment Scale (GES). We compared mean GES scores across demographic categories (race/ethnicity, education, and income), using independent samples t-tests or one-way ANOVA as appropriate. Additionally, we conducted a multivariate linear regression analysis to evaluate the impact of race/ethnicity, education, and income on GES scores, adjusting for age, gender, marital status, parental status, and employment status. Results: Of 323 adult CHD patients, the mean age was 45.6 ±15 years, 32.5% were males, 89.9% were non-Hispanic White, 29.7% had less than a Bachelor's degree, and 43.5% had less than $100,000 annual household income. The mean GES score was 61.8 ± 8, with significantly higher scores observed for non-Hispanic White identifying individuals (p=0.006) and those with a Bachelor's or higher level of education (p=0.005), while no difference across income levels (p = 0.394) (Figure 1). Linear regression analysis demonstrated that race and ethnicity remained significantly associated with GES scores (Table 1). Conclusions: Race/ethnicity and education level (in the bivariate analysis only), but not income, are the SDOH variables significantly associated with how empowered a CHD patient is in managing their health care. Future interventions that target enhancing patient engagement and self-management skills should take into consideration the influence of these demographic and social factors.
Abstract 4365058: Artificial Intelligence enabled Electrocardiograms (AI-ECG) and Pregnancy Related Cardiomyopathy: a Systematic Review and Diagnostic Meta-Analysis
Irorere, Osaivbie E, Tobah, Yvonne Butler , Adesanya, Oluwafolajimi Adetoye , Carter, Rickey E. , Young, Katie, Adedinsewo, Demilade

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4365058 Get It

Background: Cardiomyopathy occurring during pregnancy or in the postpartum period is a leading case of maternal death in the US. The most commonly described form of pregnancy related cardiomyopathy (PRCM) associated with left ventricular systolic dysfunction is peripartum cardiomyopathy, defined as left ventricular ejection fraction (LVEF) <45% on echocardiography. PRCM symptoms often overlap with those of normal pregnancy and the lack of validated screening tools limits effective recognition, contributing to diagnostic delays. Recently, several artificial intelligence enabled electrocardiogram (AI-ECG) models have been developed and evaluated for PRCM detection. Aim&Hypothesis: We sought to investigate the pooled diagnostic performance of AI-ECG models for detecting PRCM. We hypothesized that AI-ECG models would be effective for detecting PRCM. Methods: A systematic search of PubMed, Scopus, and Cochrane was performed by combining search terms “artificial intelligence”, “machine learning”, “deep learning”, “algorithm”, “ECG”, “EKG”, “electrocardiogram”, “peripartum cardiomyopathy”, “pregnancy”, “pregnancy related cardiomyopathy” , to identify studies investigating the use of AI-ECG models for PRCM detection. Studies included those utilizing standard 12-lead ECGs as well as single lead ECGs from various devices. For inclusion in the meta-analysis, studies had to provide complete confusion matrix data for the AI-ECG model. Inverse variance random effects model meta-analysis was performed. Pooled performance estimates with corresponding 95% CIs are presented in forest plots and a summary receiver operating characteristics (sROC) curve. Results: Our search identified a total of 201 studies (PubMed: 64, Embase: 117, Cochrane: 20) from which 6 studies involving 11 AI-ECG models and 3,006 patients were included in the meta-analysis (Fig. 1) . Following random effect bivariate meta-analysis, the AI-ECG models yielded a pooled sensitivity of 0.721 (95% CI 0.676–0.762); specificity of 0.931 (95% CI 0.932–0.938); diagnostic odds ratio (DOR) of 44.658 (95% CI 23.843–83.644); and AUC of 0.896 (95% CI 0.87–0.91), for PRCM detection (Fig. 2A-C, 3) . Conclusion: AI-ECG models appear to be effective tools for detecting PRCM and may represent a viable solution to reduce diagnostic delays consequently improving maternal outcomes. Additional studies are needed to evaluate its impact on clinical outcomes in pragmatic settings.
Abstract 4362826: Use of Stress Cardiac Magnetic Resonance to Evaluate Prognosis in Patients with Cardiac Implantable Electronic Devices
Jimenez, M. , Starrett, Lane, Topel, Çağdaş , Jaamour, Dania , Gill, Nathan , Hong, KyungPyo , Knight, Bradley P. , Knight, Bradley P , Jimenez, Maria Davo, Fan, Lexiaozi , Urban, Jacqueline Bexiga , Behnoush, Amir Hossein , Collins, Jeremy D. , Patel, Amit R. , Kim, Daniel , Lee, Daniel

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4362826 Get It

Background: The prevalence of cardiac implantable electronic devices (CIED) is increasing, with over 300,000 new implants annually in the U.S. Stress cardiac magnetic resonance (CMR) has proven as a powerful modality for myocardial characterization, enabling accurate diagnosis of different cardiac conditions. In patients without devices, CMR findings have also demonstrated prognostic value for major adverse cardiovascular events (MACE) and guide clinical decision-making. However, its use in CIED patients has historically been limited due to safety and image quality concerns. Hypothesis: We aimed to evaluate the prognostic value of CMR imaging using wideband sequences in CIED patients. Methods: CIED patients referred for clinical CMR were included prospectively and retrospectively between January 2020 and February 2025. All patients underwent a stress CMR protocol, which included cine, wideband late gadolinium enhancement (LGE), wideband T1 mapping, and wideband stress/rest perfusion imaging. Clinical follow-up is ongoing. The primary endpoint was the occurrence of MACE, defined as the composite of myocardial infarction, heart failure hospitalization, implantable cardioverter defibrillator (ICD) therapy, cardiac death, revascularization, or heart transplant. Results: A total of 89 patients were included (median age 69.0 [58.0–75.5] years; 62.9% male). Device types included pacemakers (33.7%), ICDs (48.3%), and cardiac resynchronization therapy devices (16.9%). Over a median of follow-up of 613 days [355-991], 21 patients experienced a MACE. In unadjusted models, higher left ventricular ejection fraction (LVEF) (per 5%) was significantly associated with a lower risk of MACE (odds ratio [OR] 0.80, 95% confidence interval [CI] 0.65–0.95; p=0.016), as was higher stress myocardial blood flow (MBF) (per 0.25 mL/g/min increase: OR 0.78, 95% CI 0.60–0.98; p=0.05). In contrast, greater LV myocardial mass (per 10 g) was significantly associated with a higher risk of MACE (OR 1.27, 95% CI 1.08–1.52; p = 0.006), figure 1. Conclusion: The use of wideband sequences enabled the assessment of prognostic imaging value in CIED patients. LVEF remains a strong and consistent predictor of MACE in this population and LV mass was significantly associated with higher rates of MACE reflecting the influence of underlying cardiomyopathy. While stress perfusion CMR imaging showed promising trends, further studies with longer follow-up are needed to clarify its prognostic value in this population.
Abstract 4361525: Influence of Cardiovascular Magnetic Resonance Imaging Phenotype and Variant Type on Ventricular Arrhythmia Risk in RBM20-Related Cardiomyopathy
Schlesinger, Reid, Witter, Taylor L. , Ackerman, Michael J. , Giudicessi, John R. , Schlesinger, Reid P , Castrichini, Matteo , Garmany, Ramin

Mayo Clinic

Circulation | November 3, 2025 | Volume: 152 | Issue: Suppl_3 | Pages: No page available

10.1161/circ.152.suppl_3.4361525 Get It

Background: Pathogenic/likely pathogenic (P/LP) variants in the RBM20-encoded RNA-binding protein motif 20 splicing factor result in a penetrant form of arrhythmogenic/dilated cardiomyopathy (ACM/DCM) characterized by ventricular arrhythmias (VA) and early-onset heart failure. The role of cardiac magnetic resonance (CMR) imaging in characterizing RBM20 cardiomyopathy and guiding risk stratification remains inadequately defined. Objectives: To characterize CMR phenotypes in patients with RBM20 cardiomyopathy and explore associations between variant type, CMR phenotype, and VA risk. Methods: Retrospective analysis of 1,061 genotype-positive ACM/DCM patients was used to identify individuals with P/LP arginine/serine-rich domain-localizing missense (RBM20msv) or truncating (RBM20tv) variants in RBM20. Clinical outcomes, including major VA (MVA; sudden cardiac arrest, sustained ventricular arrhythmia, or appropriate ICD therapy) events, were compared between RBM20msv and RBM20tv-positive patients. When available, CMR findings were also analyzed by variant type and correlated with MVA events. Results: Overall, 64 of 1,061 (6%) ACM/DCM patients possessed an RBM20msv or RBM20tv. Of those, 35 (mean age 38±16 years, 54% female, 94% Caucasian) underwent CMR. Late gadolinium enhancement (LGE) was present in 9/35 (26%), typically in a mid-myocardial septal or inferolateral pattern. Left ventricular (LV) noncompaction was noted in 9/35 (26%). Within the CMR subgroup, RBM20msv accounted for 29/35 (83%) of cases, while RBM20tv was observed in 6/35 (17%). No significant difference in LV ejection fraction (36±7% vs. 42±13%, p=0.358), LV end-diastolic volume index (100±32 mL/m2 vs. 118±44 mL/m2, p=0.342), or LGE prevalence (1/6 [17%] vs. 8/29 [28%], p=1.000) was observed between RBM20tv- and RBM20msv-positive patients. However, MVA events were more frequent among RBM20tv-positive patients in the CMR subset (3/6 [50%] vs. 2/29 [7%], p=0.026) and the overall cohort (7/15 [47%] vs. 6/49 [12%], p=0.008). Conclusions: RBM20tv-positive patients experienced a higher rate of MVA events despite similar rates of LGE and structural remodeling compared to those with RBM20msv. Future studies are needed to determine whether mechanisms independent of fibrosis/re-entry drive arrhythmogenesis in RBM20tv-positive patients and to establish if RBM20tv represents an independent risk factor for MVA events that merits incorporation into genotype-specific risk stratification strategies.