Frans Serpa

BACKGROUND:Advanced atrial remodeling, often associated with atrial fibrosis, may be accompanied by severe atrial electrical dysfunction and a range of electrocardiographic and arrhythmic manifestations. Data on the clinical features of this process are limited. CASE REPORT:We report 3 patients with end-stage atrial myopathy and longstanding persistent or permanent atrial fibrillation (AF). One patient with extensive biatrial scar developed recurrent low-amplitude atrial tachycardias after AF ablation. Two patients demonstrated progression from AF to atrial standstill with junctional rhythms. In both patients, serial ECGs showed loss of fibrillatory activity, and device interrogation demonstrated absent atrial sensing and capture. One patient underwent pacemaker implantation without atrial lead placement, due to the absence of atrial sensing or capture on electrophysiology mapping. Important coexisting substrates for atrial remodeling included hypertrophic cardiomyopathy, mitral valve disease, and prior cardiac surgeries. CONCLUSIONS:Patients with longstanding persistent or permanent AF may demonstrate distinct findings suggestive of advanced atrial myopathy, including loss of fibrillatory waves on ECG, low-amplitude atrial tachycardias, atrial standstill, junctional rhythms, absent atrial sensing or capture on device interrogation, and extensive atrial scarring on electrophysiology mapping. Recognition of these clinical manifestations may provide insight into advanced atrial remodeling and potentially inform AF ablation and atrial pacing strategies. Further studies are needed to better define the mechanisms, prevalence, and clinical significance of these findings.

Background: Although the availability of electrophysiology technologies has expanded in the United States, little is known about how sociodemographic factors influence their use. This national study of the Medicare population aims to (1) examine trends in the use of contemporary electrophysiology procedures and (2) assess associations between sociodemographic factors and population‐level use of these procedures. Methods: We identified Medicare beneficiaries who underwent pulmonary vein isolation, left atrial appendage occlusion, leadless pacemaker implantation, or subcutaneous implantable cardioverter‐defibrillator implantation from 2018 to 2021 using Current Procedural Terminology codes. Procedural rates were calculated per 100 000 person‐years among all eligible Medicare beneficiaries. We compared the rates of each procedure across sociodemographic characteristics (including sex, race, census region, and social vulnerability index) by reporting rate ratios adjusted for age and comorbidities. Results: A total of 425 242 electrophysiology procedures performed between 2018 and 2021 were included. During the study period, use rates of pulmonary vein isolation, left atrial appendage occlusion, and leadless pacemaker increased steadily, whereas subcutaneous implantable cardioverter‐defibrillator use declined. In adjusted models, individuals identifying as Black or categorized as other race were less likely to undergo a pulmonary vein isolation, left atrial appendage occlusion, and leadless pacemaker procedure but were more likely to receive a subcutaneous implantable cardioverter‐defibrillator. Beneficiaries in less socially vulnerable areas were more likely to receive pulmonary vein isolation, left atrial appendage occlusion, and leadless pacemaker but less likely to undergo subcutaneous implantable cardioverter‐defibrillator implantation. Regional variation showed higher use rates in the South and West of the United States. Conclusions: Significant sociodemographic differences in the use of contemporary electrophysiology procedures persist across communities in the United States. Targeted efforts are needed to ensure that use aligns with clinical need.

Aims: Catheter ablation is the standard treatment for symptomatic atrial fibrillation (AF) and can be performed under general anaesthesia (GA) or varying levels of sedation to optimize patient comfort and lesion formation. However, the effect of different anaesthesia strategies on AF recurrence rates remains uncertain. Methods and results: We systematically searched PubMed, Embase, Cochrane, and ClinicalTrials.gov for randomized controlled trials (RCTs) and observational studies comparing outcomes of catheter ablation under GA vs. sedation (including deep, moderate, and conscious sedation). We pooled risk ratios (RR) with 95% confidence intervals (CI) with a random effects model. R version 4.4.1 was used for statistical analyses. Our systematic review and meta-analysis included 6 RCTs and 17 observational studies, corresponding to 12 302 patients assigned to either sedation (n = 8952) or GA (n = 3350). There was no difference in recurrence of atrial tachyarrhythmias (ATAs) between groups (RR 1.15; 95% CI 0.97–1.36; P = 0.10; 95% prediction interval 0.66–2.01). There was no significant subgroup interaction in the recurrence of AF according to sedation type (conscious vs. mild vs. moderate sedation vs. deep sedation) (P = 0.20) or AF type (persistent AF vs. non-persistent) (P = 0.20). Conclusion: In patients undergoing catheter ablation for AF, there was no significant difference in recurrence of ATA between GA and sedation.

Background Cardiac myosin inhibitors (CMI) have emerged as the first disease-specific, noninvasive therapy with promising results in patients with hypertrophic cardiomyopathy. However, its role in obstructive hypertrophic cardiomyopathy (oHCM) remains uncertain, especially in secondary endpoints of randomized controlled trials (RCTs). Methods We systematically searched PubMed, Embase, Web of Science, and Clinicaltrials.gov from inception to June 2024 for RCTs comparing CMI versus placebo in patients with oHCM. We applied a random-effects model to evaluate efficacy and safety outcomes and primary or secondary outcomes of RCTs. Results We included five RCTs comprising 767 patients, of whom 402 (52.5%) were randomized to CMI. Relative to placebo, CMI were associated with a higher rate of improvement of at least one New York Heart Association (NYHA) functional class [risk ratio (RR) 2.33; 95% confidence interval (CI) 1.92–2.82]. In addition, CMI reduced resting left ventricular outflow tract (LVOT) [mean difference (MD) − 42.51 mmHg; 95% CI − 59.27 to − 25.75] and the provoked LVOT gradients (MD − 46.12 mmHg; 95% CI − 55.70 to − 36.54). However, CMI significantly increased the risk of reaching a left ventricular ejection fraction below 50% (RR 4.80; 95% CI 1.42–16.20), affecting 8% of patients during long-term follow-up of up to 120 weeks. There was no significant interaction across subgroups of class representatives, pointing to a class effect. The benefit-risk analysis indicated a larger benefit for NYHA class improvement than risk for systolic dysfunction. Conclusion In patients with oHCM, mavacamten and aficamten as a class improve clinical and hemodynamic endpoints compared with placebo, albeit with a higher incidence of a reduction in left ventricular ejection fraction. Registration PROSPERO CRD42023468079.

Inherited cardiomyopathies are genetic diseases that can lead to heart failure and sudden cardiac death. These conditions tend to run in families, following an autosomal dominant pattern where first-degree relatives have a 50% chance of carrying the pathogenic variant. Despite significant advancements and increased accessibility of genetic testing, accurately predicting the phenotypic expression of these conditions remains challenging due to the inherent variability in their clinical manifestations and the incomplete penetrance observed. This poses challenges in providing patient care and effectively communicating the potential risk of future disease to patients and their families. To address these challenges, this review aims to synthesize the available evidence on penetrance, expressivity, and factors influencing disease expression to improve communication and risk assessment for patients with inherited cardiomyopathies and their family members.

Background: The efficacy and safety of adjunctive low‐voltage area (LVA) ablation on outcomes of catheter ablation (CA) for atrial fibrillation (AF) remains uncertain.Methods: PubMed, Embase, Cochrane Library, and ClinicalTrials.gov were searched for randomized controlled trials (RCTs) comparing CA with versus without LVA ablation for patients with AF. Risk ratios (RR) with 95% confidence intervals (CI) were pooled with a random‐effects model. Our primary endpoint was recurrence of atrial tachyarrhythmia (ATA), including AF, atrial flutter, or atrial tachycardia. We used R version 4.3.1 for all statistical analyses.Results: Our meta‐analysis included 10 RCTs encompassing 1780 patients, of whom 890 (50%) were randomized to LVA ablation. Adjunctive LVA ablation significantly reduced recurrence of ATA (RR 0.76; 95% CI 0.67−0.88; p < .01) and reduced the number of redo ablation procedures (RR 0.54; 95% CI 0.35−0.85; p < .01), as compared with conventional ablation. Among 691 (43%) patients with documented LVAs on baseline substrate mapping, adjunctive LVA ablation substantially reduced ATA recurrences (RR 0.57; 95% CI 0.38−0.86; p < .01). There was no significant difference between groups in terms of periprocedural adverse events (RR 0.78; 95% CI 0.39−1.56; p = .49).Conclusions: Adjunctive LVA ablation is an effective and safe strategy for reducing recurrences of ATA among patients who undergo CA for AF.