Heart rhythm最新文献

The aortic root overlies the left ventricular ostium and the interatrial septum. Muscle sleeves supporting the aortic sinuses and extensions to the inter-leaflet triangle are sources for outflow tract arrhythmias. The sinuses of Valsalva, in addition, provide access to the inter-atrial septum and left ventricular infra-aortic regions where substrates for arrhythmias reside in both idiopathic arrhythmias and structural heart disease. This review summarizes the anatomical relationship of the aortic root to the various arrhythmic substrates and discusses approaches to ablation.

Background: Filamin-C (FLNC) gene variants are associated with cardiac and skeletal muscle diseases including a clear role of loss-of-function variants in dilated cardiomyopathy.

Objective: To assess the contribution of rare FLNC variants to hypertrophic/restrictive cardiomyopathy (HCM/RCM).

Methods: Family-based studies in two specialist services, and statistical modelling of rare FLNC missense variants, using a cohort of 3,289 sarcomere-negative HCM cases and 122,348 genome aggregation database controls.

Results: Clinical evaluation of patients with HCM/RCM and a rare FLNC variant identified a distinct ECG repolarisation phenotype in 37% (19/51 individuals, from 12 families) which was observed in only 1.0% (2/197) of a control HCM cohort. FLNC variant carriers with the characteristic ECG had smaller LV cavity size, lower contractility, more severe diastolic dysfunction, and were more likely to have a restrictive phenotype. Heart failure death, transplant or cardiac arrest occurred in at least one individual in seven of the 12 families (58%) in the 'ECG positive' group, and musculoskeletal abnormalities were present in four families (33%). Five of 12 variants (41.7%) in the 'ECG positive' group co-segregated, and two were apparently de novo. Eleven variants were missense, one splice site. Rare FLNC missense variant burden indicated a low case excess amongst all HCM cases (etiological fraction 0.45, 95% CI [0.36-0.54]), but in 'ECG positive' cases the etiological fraction was substantially higher (0.98, 95% CI [0.97-0.99]).

Conclusion: Pathogenic FLNC variants in patients with HCM/RCM are non-truncating and cause a discrete phenotype comprising a characteristic ECG, hypertrophic and restrictive features without hypercontractility, and extra-cardiac abnormalities.

Background: Catheter ablation for atrial fibrillation (AF) can improve atrial function; however, little is known about chamber-specific remodeling between the left (LA) and right (RA) atria.

Objective: We investigated the differences in bi-atrial strain and its predictive value for atrial tachyarrhythmias (AF/AT) recurrence.

Methods: We retrospectively analyzed the patients undergoing initial AF ablation who underwent pre- and post-procedural echocardiography. Atrial strain was quantified by two-dimensional speckle-tracking echocardiography.

Results: Eighty-one patients were analyzed. Both the mean LA (LASr: 15.3 ± 10.0 to 19.9 ± 8.2%) and RA (RASr: 18.3 ± 10.8 to 27.3 ± 9.3%) reservoir strain improved significantly after AF ablation (P<0.001 for both), with a greater improvement in RASr than LASr (ΔRASr: 9.0 ± 10.7% vs. ΔLASr: 4.5 ± 8.4%, P<0.001). Moreover, whereas LA conduit strain did not change significantly (-9.3 ± 5.6 to -10.3 ± 4.4%, P=0.071), RA conduit strain improved significantly (-10.3 ± 6.3 to -14.1 ± 6.2%, P<0.001). Post-procedural LASr (HR 0.92, 95%CI: 0.85-1.0, P=0.049) and RASr (HR 0.94, 95%CI:0.89-0.99, P=0.016) were independent predictors of AF/AT recurrence. ROC analysis yielded similar discriminative ability for LASr (area under the curve (AUC): 0.75, cut-off value: 20.0%) and RASr (AUC: 0.69, cut-off value: 27.0%) (P=0.367).

Conclusion: AF ablation led to significant bi-atrial functional recovery, with RA improvement exceeding that of LA. Both post-procedural LASr and RASr independently predicted AF/AT recurrence, supporting chamber-specific atrial strain as a sensitive marker of reverse remodeling and procedural outcome.

Background: Sinus node dysfunction (SND) is a known complication after surgery for congenital heart disease (CHD), however its post-operative course is not well characterized.

Objective: Describe the incidence of post-operative SND and its impact on post-operative outcomes.

Methods: Retrospective study of children 0-18 years old after cardiac surgery 2013-2017. SND defined as bradycardia for age, junctional escape rhythm, or use of post-operative atrial pacing for bradycardia. SND sub-categorized as either Absolute SND (sinus bradycardia for age or junctional rhythm < 100 bpm) or Relative SND (normal sinus rate with hemodynamic need for atrial pacing). Post-operative outcomes compared in surgeries with no post-operative SND, Absolute SND, and Relative SND.

Results: Of 4571 surgeries (age 1 [IQR 0.2, 4.5] year), SND occurred in 786 (17.2%) surgeries (Absolute 695, Relative 91). Absolute SND most common following surgery involving pulmonary veins (31%), mitral valve (27%), atrial baffle creation (27%), Glenn (26%), and Fontan palliation (26%). Relative SND most common after Stage 1 palliation (10%). Most surgeries (89%) recovered from SND before discharge, median recovery time 31.9 hours. Surgeries with SND had longer hospital and ICU length of stay, time to extubation, and higher incidence of major adverse cardiac events.

Conclusion: SND occurs in over 25% of surgeries involving pulmonary veins, mitral valve, atrial baffle, Glenn, and Fontan palliation. Stage 1 palliation frequently requires pacing for hemodynamic need. SND persists at discharge in 11% and is associated with worse post-operative outcomes. Permanent atrial pacing need was found in 2% of all surgeries with post-operative SND.

Background: Biventricular (Bi-V) pacing improves left ventricular (LV) systolic function by correcting dyssynchrony, but potential proarrhythmic effects have been reported. The relationship between Bi-V pacing and ventricular tachycardia (VT) recurrence after catheter ablation (CA) remains unclear.

Objective: To assess the impact of Bi-V pacing on VT recurrence after CA in patients with structural heart disease, considering VT origin.

Methods: Out of 795 consecutive patients who underwent CA for VT, 384 met inclusion criteria of ≥90% Bi-V pacing (Bi-V pacing group) or intrinsic AV conduction with ≤10% ventricular pacing (Intrinsic Rhythm group). VT origin was classified as septal, lateral, and anterior/inferior. Baseline characteristics were balanced by propensity score matching (1:1) .

Results: 146 patients were included per group (median follow-up 473 days). All-cause mortality (26.0% vs. 24.7%, log-rank p=0.49) and heart failure hospitalization (21.9% vs. 21.9%, log-rank p=0.63) did not differ significantly between groups. VT/VF recurrence was higher in the Bi-V pacing group (37.0% vs. 19.2%, log-rank p<0.01) than in the intrinsic rhythm group and was largely due to greater VT recurrence in patients with lateral wall VT origins and Bi-V pacing (55.9% vs. 21.9% log-rank p<0.01). In the absence of Bi-V pacing a lateral wall VT origin was not associated with greater VT recurrences. On multivariable Cox regression, an LV lateral wall VT with Bi-V pacing was an independent predictor of VT recurrence (HR 3.04, 95% CI 1.65-5.60; p<0.01).

Conclusions: Bi-V pacing with an LV lateral wall substrate was associated with increased VT/VF recurrence after CA.

Background: Desmoglein-2 (DSG2)-associated cardiomyopathy represents a distinct subset of arrhythmogenic cardiomyopathy (ACM). A founder variant, NM_001943.5 (DSG2): c.T1592G (p.Phe531Cys), was identified with high frequency in China.

Objective: The study aimed to describe clinical features and outcomes of this founder variant.

Methods: Individuals with DSG2 c.T1592G (p.Phe531Cys) variants were recruited from 9 centers across China and categorized as single heterozygous, compound heterozygous (single variant plus rare variants of uncertain significance; abbreviated as compound) and homozygous. Clinical features and risk factors for malignant ventricular arrhythmias (MVA), end-stage heart failure (ESHF), and composite events of heart transplantation or cardiac death were analyzed.

Results: Ninety-one subjects were included: 21 (23.1%) single heterozygous, 21 (23.1%) compound, and 49 (53.8%) homozygous. Most of subjects (74.7%) showed right ventricular dilatation and nearly half (49.5%) had biventricular involvement. In patients with contrast-enhanced magnetic resonance imaging, 75.9% exhibited biventricular involvement. Compared with single heterozygous, compound and homozygous had younger age at onset, more T wave inversion, epsilon waves, and biventricular involvement (all pairwise P<0.05). Homozygous experienced significantly earlier MVA than compound (P=0.013), and single heterozygous (P<0.001), with a trend toward earlier MVA in compound compared with single heterozygous (P=0.089). Compound and homozygous exhibited significantly higher incidences of ESHF and composite events while single heterozygous remains event-free (all P<0.05).

Conclusion: DSG2 c.T1592G (p.Phe531Cys) founder variant defines a distinct ACM subset with high prevalence of biventricular involvement. Single heterozygous variant carriers held less severe phenotype and relatively favorable prognosis, while compound and homozygous held advanced phenotype and poorer prognosis.

Background: Left atrial low-voltage areas (LVAs), indicative of atrial fibrosis and structural remodeling, are present in a subset of patients with paroxysmal atrial fibrillation (AF).

Objective: This study aimed to develop and validate a novel predictive model for identifying LVAs in paroxysmal AF patients.

Methods: Paroxysmal AF patients receiving their initial radiofrequency ablation in the Department of Cardiology at Shanghai East Hospital were enrolled. LVAs were defined as regions with a bipolar voltage of less than 0.5 mV during left atrial voltage mapping. Logistic regression analysis was used to identify independent predictors and construct the prediction model. An independent prospective cohort and a multicenter cohort of paroxysmal AF patients were used for validation.

Results: A total of 383 patients with paroxysmal AF were enrolled respectively, among whom 104 patients (27.2%) had left atrial LVAs. Multivariate logistic regression analysis identified female, prior stroke, left atrial diameter, PR interval, hemoglobin level, and serum creatinine level were independent predictors of LVAs. The HeSLeF-PC score was developed based on these factors, and could predict the presence of left atrial LVAs in paroxysmal AF patients (AUC = 0.810, 95% CI: 0.762-0.859), which was further validated in the prospective cohort (AUC = 0.826, 95% CI: 0.757-0.896) and the multicenter cohort (AUC = 0.767, 95% CI 0.678-0.857). Decision curve analysis confirmed its clinical utility.

Conclusions: The HeSLeF-PC score could effectively predict the presence of left atrial LVAs in paroxysmal AF patients and may assist in preprocedural risk stratification and ablation planning.

Background: Among patients with persistent atrial fibrillation (PerAF), linear ablation lesions beyond pulmonary vein isolation (PVI) are often performed with variable efficacy and inconsistent added value. New catheter designs and energy sources have the potential to improve outcomes.

Objective: To examine: 1) the performance of a novel dual-energy (pulsed field [PF] /radiofrequency [RF]) lattice-tip mapping and ablation system for linear ablation, and 2) the impact of the ablation strategy on outcomes in the randomized SPHERE Per-AF trial.

Methods: Linear lesion characteristics, safety, and effectiveness were assessed for a conventional RF ablation system in the control arm vs. the dual-energy lattice-tip system in the investigational arm.

Results: In the investigational arm, 203 (95.8%) received left atrial roof and/or posterior wall isolation (LAPWI), 117 (55.2%) cavotricuspid isthmus (CTI) line, and 76 (35.8%) mitral line. In the control arm, 102 (68.9%) patients received LAPWI, 76 (51.4%) CTI, and 19 (12.8%) mitral line ablation. Transpired ablation and energy application times were shorter for each lesion type using the investigational vs. control catheter (all p<0.0001). Overall, there was a trend towards numerically higher 12-month effectiveness of a "PVI + all linear lesions combined" (n=25) approach compared to a PVI-only (n=56) ablation strategy (p=0.07), and the effect was more pronounced in the investigational arm, but sub-groups were small.

Conclusion: There was a trend towards numerically higher effectiveness with linear lesions vs. PVI-alone. These findings are exploratory and hypothesis-generating and should prompt future randomized controlled trials of additional lesion sets vs. PVI with this new technology.

Clinical trial registration: NCT05120193- Treatment of Persistent Atrial Fibrillation with Sphere-9 and Affera Mapping and Ablation System (SPHERE Per-AF).

Background: Catheter ablation (CA) is a curative therapy for atrioventricular reentrant tachycardia (AVRT) caused by accessory pathways (APs), but precise AP localization with conventional mapping can be challenging. While omnipolar technology near-field (OTNF) provides novel frequency-based analysis, its clinical utility for AP ablation remains unproven.

Objective: We aimed to characterize the spatial relationship between high-frequency areas identified by OTNF-derived peak frequency maps and successful AP ablation sites.

Methods: This multicenter retrospective study analyzed OTNF data from 33 patients undergoing successful CA for AVRT. Offline peak frequency analysis was performed on acquired open-window mapping data. We measured: (1) the peak frequency (PF) at the successful ablation site, (2) the highest PF in the immediate vicinity, (3) the spatial distance, and (4) the directional displacement between these two points.

Results: While successful ablation sites were located in areas of high frequency (mean 332 ± 206 Hz), the absolute highest PF was consistently found in the immediate vicinity (mean 435 ± 56 Hz; p < 0.01). Critically, the site of the highest PF was spatially displaced from the successful ablation site by a median distance of 4 mm (IQR: 0-6 mm). Directionally, when viewed from the annulus, the highest frequency site was co-located with the successful site in 21 cases (64%), shifted counter-clockwise in 9 cases (27%), and clockwise in 3 cases (9%).

Conclusion: High-frequency areas on OTNF maps are consistently located adjacent to, but spatially distinct from, successful AP ablation sites. This quantifiable spatial dissociation represents a novel electrophysiological target that may improve the precision and efficiency of AP catheter ablation.