Citations

Background: Ventricular tachycardia (VT) is a potentially life-threatening arrhythmia frequently observed in structural heart diseases, including cardiac laminopathy, a genetic cardiomyopathy associated with a high risk of sudden cardiac death (SCD) and progressive systolic dysfunction caused by pathogenic variants in LMNA -encoded lamin A/C. While catheter ablation is a key therapeutic strategy for VT management, its efficacy in cardiac laminopathy remains poorly defined. Objective: This systematic review and single-arm meta-analysis aims to synthesize the available evidence in the literature on the role of VT ablation for patients with cardiac laminopathy. Methods: A single-arm meta-analysis was conducted following PRISMA guidelines, systematically searching PubMed, Embase, and Cochrane databases for studies investigating VT ablation outcomes in patients with cardiac laminopathy. We applied an inverse variance random-effects model for a meta-analysis of proportions. Results: We included 6 cohort studies and 1 abstract comprising 62 patients with LMNA-related cardiomyopathy (mean age at 53.2 ± 9.7 years, 85% males, and 88% with an ICD). The predominant ablation strategy was endocardial/endo-epicardial in 93% of cases. Acute success rate following VT ablation was 37% (95% CI: 14 to 63%) and 28% of the patients required multiple procedures. Over a median follow-up of 26 (9-35) months, VT recurrence rate after ablation was 91% (95% CI: 76 to 100%; Figure A ). The all-cause mortality rate was 54% (95% CI: 39% to 70%; Figure B ), primarily driven by cardiac causes 51% (95% CI: 25 to 76%; Figure C ). There was an elevated rate of heart transplantation 14% (95% CI: 1 to 34%), highlighting overall poor outcomes in this patient population. Conclusion: Catheter ablation in cardiac laminopathy is associated with limited long-term efficacy, with high rates of VT recurrence, cardiovascular mortality and heart transplantation. These findings underscore the importance of ICD implantation for primary prevention and the pressing need to develop alternative treatment strategies. Further research is necessary to determine whether specific LMNA variants influence ablation outcomes and to explore emerging therapies targeting the underlying molecular mechanisms of cardiac laminopathy.

Background: Ventricular tachycardia (VT) is a life-threatening arrhythmia frequently observed in structural heart diseases, including LMNA-related cardiomyopathy, a genetic disorder associated with high risk of sudden cardiac death and progressive systolic dysfunction. While catheter ablation is an established therapeutic option for VT, its efficacy in cardiac laminopathy remains poorly defined. Objectives: To synthesize available evidence on the role and outcomes of VT ablation in patients with cardiac laminopathy. Methods: A systematic review and single-arm meta-analysis was performed in accordance with PRISMA guidelines. PubMed, Embase, and Cochrane databases were searched for studies reporting VT ablation outcomes in LMNA-related cardiomyopathy. An inverse variance random-effects model was applied for meta-analysis of proportions. Results: Seven studies (six cohorts, one abstract) comprising 62 patients (mean age 53.2 ± 9.7 years; 85% male; 88% with implantable cardioverter-defibrillator) were included. An endocardial/endo-epicardial approach was used in 93% of cases. Acute procedural success was achieved in 37% (95% CI: 14–63%; I 2 = 27.4%), and 28% required multiple procedures. Over a median follow-up of 26 (9–35) months, VT recurrence occurred in 91% (95% CI: 76–100%; I 2 = 37.2%). All-cause mortality was 54% (95% CI: 39–70%; I 2 = 28%), predominantly from cardiac causes (51%, 95% CI: 25–76%; I 2 = 53%), and 14% (95% CI: 1–34%; I 2 = 42%) underwent heart transplantation. Conclusions: In LMNA-related cardiomyopathy, catheter ablation is associated with high VT recurrence, limited long-term success, and substantial cardiovascular mortality and transplantation rates, underscoring the importance of ICD implantation and the need for alternative strategies, including variant-specific and molecularly targeted therapies.

Background: Arrhythmogenic cardiomyopathy (ACM) is a genetic heart disease characterized by proclivity for ventricular arrhythmias and systolic dysfunction. Rarely, sudden cardiac arrest (SCA) is the presenting event in ACM. These individuals may have concealed disease with no appreciable structural manifestations on imaging. Aims: To determine the prevalence, clinical features, and outcomes of patients with genotype-positive ACM who presented with a sentinel SCA. Methods: In this retrospective study, 445 genotype-positive ACM patients evaluated from 1/2015-4/2024 were analyzed to identify those whose initial presentation was SCA. Pertinent demographic, clinical, genetic, and outcome data were extracted from the electronic medical record. Structural manifestations were defined by having one of the following on baseline TTE: EF < 50%, LVEDV >56 mL (male) or >51 mL (female), or regional RV akinesia, dyskinesia, or aneurysm. Categorical variables are presented as number (%), and continuous variables as median (IQR). Statistical analysis was done using Fisher’s exact test (categorical), t-test (continuous), and Logrank test (survival). Patients were censored at time of transplant, death, or last followup. Results: Overall, 14/445 (3%) ACM patients presented with sentinel SCA [71% male; median age at SCA 39 (IQR 30-46)]. The most common genetic substrates were DSP -encoded desmoplakin (n=4; 29%), LMNA -encoded lamin A/C (n=3; 21%), and PKP2 -encoded plakophilin-2 (n=3; 21%). During a median followup of 8.4 years, 12 (86%) experienced appropriate ICD shocks, 8 (57%) required catheter ablation for ventricular arrhythmias, 6 (42%) underwent transplant, and 2 (14%) died of cardiac causes. Notably, 5 (35%) patients did not have evidence of structural disease during baseline evaluation. Compared to those with structural disease, this group was younger [median age 28 (IQR 21-29) vs 45 (IQR 41-48), p = 0.002] and less likely to undergo transplant [0 (0%) vs 6 (42%), p = 0.03]. Conclusions: Sentinel SCA is a rare presentation in ACM and may precede overt structural cardiac changes in a subset of younger patients. This population remains at increased risk for recurrent ventricular arrhythmias and often require advanced therapies.

AbstractIntroductionLittle is known regarding the prevalence and prognostic implications of non‐sustained ventricular tachycardia (NSVT) in patients with wild‐type transthyretin cardiac amyloidosis (ATTRwt‐CA). We aimed to investigate the prevalence of NSVT in patients with ATTRwr‐CA, and the association of NSVT with sustained ventricular arrhythmias (VA) and all‐cause mortality.MethodsIn a cohort of ATTRwt‐CA patients from 2011 to 2022 without prior sustained VA, we ascertained the presence and characteristics of NSVT during clinically indicated ambulatory Holter monitoring. Patients were stratified based on the presence of NSVT at baseline. The primary and secondary endpoints of sustained VA and all‐cause mortality, respectively, were assessed during follow‐up.ResultsThe cohort included 217 patients with ATTRwt‐CA (95% males, median age 75 years; median NYHA class 2). Baseline Holter monitoring demonstrated NSVT in 116 (53%) patients. During a median follow‐up of 27 months (IQR 16–45) after the index Holter monitor, 11 (5.1%) patients reached the primary endpoint of sustained VA (incidence 1.8 per 100 person‐years, all monomorphic VT), and 46 (21%) patients died. In univariable Cox proportional hazard model, NYHA class 3 or 4 heart failure (p = .048), the presence of NSVT (p = .04), the duration of longest NSVT run (p = .029), and the percentage of ventricular ectopy (p < .001) were associated with the primary outcome. When adjusting for age and NYHA class 3 or 4 heart failure, the presence of NSVT remained associated with the primary outcome (p = .03). All‐cause mortality was not significantly different between patients with and without NSVT. A cutoff for ventricular ectopy burden of 0.5%, the median for all patients in this study, was associated with increased risk in sustained VA (log‐rank p = .004) and mortality (log‐rank p = .02).ConclusionNSVT is highly prevalent among ATTRwt‐CA patients undergoing clinically indicated Holter monitoring and may confer an increased risk of incident sustained VA.

Abstract Background Sarcoidosis is a disease characterized by non-caseating granulomas and may affect any organ system. Cardiac involvement may lead to conduction abnormalities, heart failure, or malignant ventricular arrhythmias. As sarcoidosis may present with heterogeneous manifestations, a detailed past medical history may provide clues that help guide further workup. We present a rare case of a patient with undiagnosed oral sarcoidosis who subsequently experienced cardiac arrest from cardiac involvement. Case summary A 43-year-old male with a history of palpitations and periodontitis consistent with oral sarcoidosis presents after experiencing sudden cardiac arrest. He was subsequently diagnosed with cardiac and pulmonary sarcoidosis. With contemporary management (both immunosuppression and antiarrhythmics), he has not experienced any recurrent arrhythmias. Discussion In the setting of cardiac arrest and non-ischaemic cardiomyopathy, a careful clinical history and targeted cardiac testing may help clinicians determine when to consider cardiac sarcoidosis as a diagnosis. While oral sarcoidosis is a very rare condition, this case highlights how infrequent manifestations of sarcoidosis may be encountered in the clinical setting.

Introduction: Advanced imaging with cardiac magnetic resonance (CMR) or computed tomography (CCT) can be helpful for planning of ventricular tachycardia (VT) ablation procedures by defining the arrhythmogenic substrate. The frequency with which these modalities are utilized in the pre-ablation setting and the associated outcomes remain poorly studied. Objectives: (1) Determine the utilization of advanced cardiac imaging prior to structural VT ablation; (2) establish factors associated with improved VT ablation outcomes Methods: We retrospectively evaluated all VT ablations performed in patients with structural heart disease at a tertiary referral institution during a 2.5 year period (1/2020 - 7/2022). We documented pre-ablation use of cardiac imaging modalities and investigated their association with acute and long-term ablation outcomes. The primary outcome was time to VT recurrence, cardiac transplant, or death. Results: Our dataset included n = 129 subjects. Median post-ablation follow-up was 17 months. Recent echocardiogram, CMR, or CCT (<3 months pre-ablation) had been performed in 113 (88%), 48 (37%), and 31 (24%) patients, respectively. CMR was not associated with procedural success, post-ablation VT recurrence (p = 1), or the composite primary outcome (p = 0.72). Similarly, recent CCT was not associated with procedural success (p = 0.5), VT recurrence (p = 0.3), or the composite primary outcome (p = 0.31). When adjusted for other variables, EF (p = 0.048), recent echo (p = 0.02), and procedural success (p = 0.013) were associated with the primary outcome, but recent CCT (p = 0.11) and CMR (p = 0.4) were not. These results did not differ significantly in patients with ischemic and non-ischemic cardiomyopathy. Conclusions: In this large tertiary care ablation center, CMR and CCT were utilized in only a fraction of patients prior to VT ablations. In multivariate analysis, CMR or CCT were not associated with improved acute or long-term ablation outcomes.