BACKGROUND: Atrioventricular block (AVB) is common in the elderly and therefore considered to be a degenerative disease of the cardiac conduction system. However, there exist other etiologies contributing to AVB in young patients. This study aimed to determine the etiologies in patients aged before 60 years receiving their first pacemaker implantation for AVB in China.
RESULTS: Medical records and diagnostic tests of AVB patients were reviewed to identify the etiologies between 2010 and 2021 at Guangdong Provincial People\'s Hospital. Eight hundred and twenty-six patients (median age 47 years; 47.9% males) were included. The etiologies were identified in 336 (40.7%) cases, including complications to cardiac surgery (n = 190 [23.0%]), myocarditis (n = 57 [6.9%]), myocardial infarction (n = 25 [3.0%]), complications to catheter-based interventional procedures (n = 21 [2.5%]) and others (n = 43 [5.2%]). AVB caused by myocardial infarction was more common in men (5.8% vs. 0.5%, p < .001), while women received pacing treatment earlier (48 vs. 46 years, p = .019). Men were more likely to suffer from dilated cardiomyopathy (6.6% vs. 2.1%, p = .001) and atrial fibrillation/flutter (23.0% vs. 12.8%, p < .001). The number of first pacemaker implantation increased with age especially among patients with unclear etiologies.
CONCLUSIONS: The etiology of AVB was only determined in approximately 40% of patients receiving their first pacemaker implantation aged before 60 years. The predominance of AVB with unknown etiology and potential gender differences warrants further studies.

Risperidone is a second-generation antipsychotic for treating schizophrenia and bipolar disorder. It can potently inhibit IKr, but is classified into conditional risk for torsade de pointes (TdP) by CredibleMeds®. Our previous studies using chronic atrioventricular block dogs showed that risperidone alone did not induce TdP, and that dl-sotalol (β-adrenoceptor blockade plus IKr inhibition) induced TdP three times more frequently than d-sotalol (IKr inhibition alone). Since risperidone can block α1-adrenoceptor and decrease blood pressure, the resulting reflex-mediated increase of sympathetic tone on β-adrenoceptor might protect the heart from its IKr inhibition-associated TdP. To validate this hypothesis, risperidone was administered to chronic atrioventricular block dogs after β-adrenoceptor blocker atenolol infusion with monitoring J-Tpeak and Tpeak-Tend, which are proarrhythmic surrogate markers of \"substrate\" and \"trigger\" toward TdP, respectively. Atenolol alone induced TdP in 1 out of 5 dogs; moreover, an additional infusion of risperidone induced TdP in 3 out of 4 dogs. Risperidone prolonged QT interval, J-Tpeak and Tpeak-Tend in animals that induced TdP. These findings indicate that β-adrenoceptor blockade can diminish repolarization reserve to augment risperidone\'s torsadogenic potential, thus advising caution when using β-adrenoceptor blockers in patients with IKr inhibition-linked labile repolarization.

UNASSIGNED: LAMA2-related muscular dystrophy (LAMA2-MD) and SELENON-related myopathy (SELENON-RM) are two rare neuromuscular diseases characterized by proximal and axial muscle weakness, scoliosis, spinal rigidity, low bone quality and respiratory impairment. Cardiac involvement has previously been described in retrospective studies and case reports, but large case series and prospective studies in unselected cohorts are lacking.
UNASSIGNED: The objective of this study is to conduct prevalence estimations, perform cardiac phenotyping, and provide recommendations for clinical care.
UNASSIGNED: In this case series including two time points, we conducted comprehensive assessments with electrocardiography (ECG) and transthoracic echocardiography (TTE). ECGs were systematically assessed for a large subset of variables. TTE included left and right ventricular ejection fraction (LVEF/RVEF) and left ventricular global longitudinal strain (GLS), the latter being a more early and sensitive marker of left ventricular dysfunction.
UNASSIGNED: 21 LAMA2-MD (M = 5; 20±14 years) and 10 SELENON-RM patients (M = 7; 18±12 years) were included. In most patients, QRS fragmentation and Q waves, markers of heterogeneous ventricular activation, were present both at baseline and at follow-up. GLS was abnormal (age specific in children, > -18% in adults) in 33% of LAMA2-MD and 43% of SELENON-RM patients at baseline. Reduced LVEF (<52% in males, <54% in females and <55% in pediatric population) was observed in three LAMA2-MD patients at baseline and in none of the SELENON-RM patients. GLS and LVEF did not change between baseline and follow-up. RVEF was normal in all patients.
UNASSIGNED: ECG abnormalities and abnormal GLS are prevalent in LAMA2-MD and SELENON-RM, yet abnormal LVEF was only seen in LAMA2-MD patients. One LAMA2-MD patient had a clinically relevant deterioration in LVEF during 1.5-year follow-up. We advise routine screening of all patients with LAMA2-MD or SELENON-RM with ECG and echocardiography at diagnosis, minimally every two years from second decade of life and if new cardiac signs arise.

Cleidocranial dysplasia (CCD) is a rare genetic disorder characterized by skeletal abnormalities, including hypoplastic or absent clavicles, delayed closure of cranial sutures, and dental anomalies. We present a case of a 72-year-old female with a history of breast cancer treated with mastectomy and radio chemotherapy with the port-a catheter still in place in the left subclavian region. She presented to the emergency room with syncope related to a complete atrioventricular (AV) block. The patient underwent temporary pacing via femoral access while awaiting definitive pacemaker implantation. The absence of the right clavicle, first observed during prepuncture fluoroscopy and later confirmed on postprocedure imaging, significantly influenced the approach to pacemaker implantation. Venography played a crucial role in visualizing the venous trajectory and guiding the puncture, ensuring successful lead placement. The procedural challenges encountered due to the patient\'s skeletal anomalies highlight the importance of individualized approach and careful consideration of anatomical variations in interventional cardiology procedures.

BACKGROUND: Emerging evidence suggests a central role for inflammation in cardiac conduction disorder (CCD). It is unknown whether habitual physical activity could modulate the inflammation-associated risks of incident CCD in the general population.
RESULTS: This population-based cohort was derived from the China Kailuan study, including a total of 97 192 participants without prior CCD. The end points included incident CCD and its subcategories (atrioventricular block and bundle-branch block). Systemic inflammation was indicated by the monocyte-to-lymphocyte ratio (MLR). Over a median 10.91-year follow-up, 3747 cases of CCD occurred, with 1062 cases of atrioventricular block and 2697 cases of bundle-branch block. An overall linear dose-dependent relationship was observed between MLR and each study end point (all P-nonlinearity≥0.05). Both higher MLR and physical inactivity were significantly associated with higher risks of conduction block. The MLR-associated risks of developing study end points were higher in the physically inactive individuals than in those being physically active, with significant interactions between MLR levels and physical activity for developing CCD (P-interaction=0.07) and bundle-branch block (P-interaction<0.05) found. Compared with those in MLR quartile 2 and being physically active, those in the highest MLR quartile and being physically inactive had significantly higher risks for all study end points (1.42 [95% CI, 1.24-1.63], 1.62 [95% CI, 1.25-2.10], and 1.33 [95% CI, 1.13-1.56], respectively, for incident CCD, atrioventricular block, and bundle-branch block).
CONCLUSIONS: MLR should be a biomarker for the risk assessment of incident CCD. Adherence to habitual physical activity is favorable for reducing the MLR-associated risks of CCD.

BACKGROUND: Variations in the aortomitral positional anatomy, including aortic root rotation appear to be related to variations in the location of the conduction system, including the bundle of His. However, little is known about their clinical significance.
RESULTS: This study included 147 patients with normal ECGs who underwent mitral valve surgery. The aortomitral anatomy was classified using preoperative 3-dimensional transesophageal echocardiography, and postoperative conduction disorders, including atrioventricular block and bundle branch block, were analyzed. Variations classified as aortomitral appearance were designated as having a center appearance (85.7%, n=126/147) or lateral appearance (14.3%, n=21/147) on the basis of whether the aortic root was located at the center or was shifted to the left fibrous trigone side. Subsequently, those with a center appearance, aortic root rotation was classified as having a center rotation (83.3% [n=105/126]), in which the commissure of the left and noncoronary aortic leaflet was located at the center, lateral rotation (14.3% [n=18/126]), rotated to the left trigone side, or medial rotation (2.4% [n=3/126]), rotated to the right. The incidence of 3-month persistent new-onset conduction disorder was higher in the lateral appearance than the center appearance group (21.1% versus 5.0%; P=0.031) and higher in the lateral rotation than in the center or medial rotation groups (29.4% versus 1.0% versus 0.0%, respectively; P<0.001).
CONCLUSIONS: Aortomitral variations can be classified using 3-dimensional transesophageal echocardiography. Lateral appearance and lateral rotation are risk factors for conduction disorders in mitral valve surgery.

Cardioneuroablation (CNA) is a novel interventional procedure for the treatment of recurrent vasovagal syncope (VVS) and advanced atrioventricular block secondary to hyperactivation of vagal tone in young patients. By damaging the cardiac parasympathetic ganglia, CNA seems to be able to mitigate and/or abolish the excessive vagal activity and improve patients\' outcome. This review is intended to give a detailed and comprehensive overview of the current evidences regarding (1) the clinical applications of CNA (2) the identification of ablation targets and procedural endpoints (3) the medium-long term effect of the procedure and its future perspectives. However, clinical data are still limited, and expert consensus or recommendations in the guidelines regarding this technique are still lacking.

UNASSIGNED: Injury to the cardiac conduction system requiring a permanent pacemaker (PPM) implantation is a known adverse outcome of transcatheter aortic valve replacement (TAVR). Temporary-permanent pacemakers (TPPM) have been used as a bridge to PPM implantation in patients with systemic infection; however, there are only a few reports of its routine use in patients undergoing TAVR. This study aimed to assess the utility of routine use of TPPM in patients undergoing TAVR with a high risk of needing a PPM or those who develop high-grade conduction abnormalities during/after TAVR.
UNASSIGNED: Between April 2015 and December 2021, 978 patients underwent TAVR at our institution, of whom 111 patients had TPPM placed before or during/after TAVR during the study period. In total, 89 patients were included in the final analysis.
UNASSIGNED: The median age was 78 years (IQR, 71-84 years); 52 (58.4%) patients with preexisting native conduction disease were considered high risk for advanced heart block and had TPPM placed before TAVR. In addition, 37 (41.6%) patients had TPPM placed during/after TAVR. Of the 89 patients who received TPPM, 51 (57.3%) were treated with a balloon-expandable valve and 38 (42.7%) with a self-expandable valve. Of the patients who underwent TPPM placement, only 49 (55.1%) required a PPM, and TPPM was removed in 40 (44.9%) patients. TPPM was in place for a median of 6 days (IQR, 2-11 days). Only 1 of the 89 patients (1.1%) who received a TPPM had lead dislodgment. No other complications were noted. Median length of stay was 3 days (IQR, 2-4 days).
UNASSIGNED: In patients with high-risk baseline conduction abnormalities before TAVR and those who develop new high-grade conduction abnormalities during/after TAVR, TPPM provides a feasible and safe method for pacing that could allow early ambulation, facilitate early discharge, and prevent unnecessary PPM implantations in some patients.

BACKGROUND: Remifentanil, an ultra-short-acting µ-opioid receptor agonist, is commonly used for anesthetic management due to excellent adjustability. Remifentanil is known to cause sinus bradycardia, however, because it has a direct negative chronotropic effect on the cardiac conduction system and there is an indirect negative chronotropic effect via the parasympathetic nervous system.
METHODS: An 8-year-old Japanese boy was diagnosed with acute hydrocephalus due to a brain tumor in the fourth ventricle and underwent emergency surgery. Imaging examination showed brainstem compression. Endoscopic third ventriculostomy and ventriculoperitoneal shunt surgery were scheduled. Remifentanil was started during induction of general anesthesia, but electrocardiogram showed sinus bradycardia, then Wenckebach-type atrioventricular block, and then complete atrioventricular block. Remifentanil was immediately discontinued, and we administered atropine sulfate. Complete atrioventricular block was restored to sinus rhythm. When remifentanil was restarted, however, the electrocardiogram again showed sinus bradycardia, Wenckebach-type atrioventricular block, and then complete atrioventricular block. Remifentanil was again immediately discontinued, we administered adrenaline, and then complete atrioventricular block was restored to sinus rhythm. Fentanyl was used instead of remifentanil with continuous infusion of dopamine. There has since been no further occurrence of complete atrioventricular block.
CONCLUSIONS: This is the first known case of complete atrioventricular block in a pediatric patient with increased intracranial pressure seemingly caused by administration of remifentanil.

OBJECTIVE: Physiological activation of the heart using algorithms to minimize right ventricular pacing (RVPm) may be an effective strategy to reduce adverse events in patients requiring anti-bradycardia therapies. This systematic review and meta-analysis aimed to evaluate current evidence on clinical outcomes for patients treated with RVPm algorithms compared to dual-chamber pacing (DDD).
RESULTS: We conducted a systematic search of the PubMed database. The predefined endpoints were the occurrence of persistent/permanent atrial fibrillation (PerAF), cardiovascular (CV) hospitalization, all-cause death, and adverse symptoms. We also aimed to explore the differential effects of algorithms in studies enrolling a high percentage of atrioventricular block (AVB) patients. Eight studies (7229 patients) were included in the analysis. Compared to DDD pacing, patients using RVPm algorithms showed a lower risk of PerAF [odds ratio (OR) 0.74, 95% confidence interval (CI) 0.57-0.97] and CV hospitalization (OR 0.77, 95% CI 0.61-0.97). No significant difference was found for all-cause death (OR 1.01, 95% CI 0.78-1.30) or adverse symptoms (OR 1.03, 95% CI 0.81-1.29). No significant interaction was found between the use of the RVPm strategy and studies enrolling a high percentage of AVB patients. The pooled mean RVP percentage for RVPm algorithms was 7.96% (95% CI 3.13-20.25), as compared with 45.11% (95% CI 26.64-76.38) of DDD pacing.
CONCLUSIONS: Algorithms for RVPm may be effective in reducing the risk of PerAF and CV hospitalization in patients requiring anti-bradycardia therapies, without an increased risk of adverse symptoms. These results are also consistent for studies enrolling a high percentage of AVB patients.