This study aimed to evaluate the progression of electrophysiological phenomena in a cohort of patients with paroxysmal atrial fibrillation (PAF) and persistent atrial fibrillation (PsAF).
Electrical ...remodeling has been conjectured to determine atrial fibrillation (AF) progression.
High-density electroanatomic maps during sinus rhythm of 20 patients with AF (10 PAF, 10 PsAF) were compared with 5 healthy control subjects (subjects undergoing ablation of a left-sided accessory pathway). A computational postprocessing of electroanatomic maps was performed to identify specific electrophysiological phenomena: slow conductions corridors, defined as discrete areas of conduction velocity <50 cm/s, and pivot points, defined as sites showing high wave-front curvature documented by a curl module >2.5 1/s.
A progressive decrease of mean conduction velocity was recorded across the groups (111.6 ± 55.5 cm/s control subjects, 97.1 ± 56.3 cm/s PAF, and 84.7 ± 55.7 cm/s PsAF). The number and density of slow conduction corridors increase in parallel with the progression of AF (8.6 ± 2.2 control subjects, 13.3 ± 3.2 PAF, and 20.5 ± 4.5 PsAF). In PsAF the atrial substrate is characterized by a higher curvature of wave-front propagation (0.86 ± 0.71 1/s PsAF vs 0.74 ± 0.63 1/s PAF; P = 0.003) and higher number of pivot points (25.1 ± 13.8 PsAF vs 9.5 ± 6.7 PAF; P < 0.0001). Slow conductions: corridors were mostly associated with pivot sites tending to cluster around pulmonary veins antra.
The electrical remodeling hinges mainly on corridors of slow conduction and higher curvature of wave-front propagation. Pivot points associated to SC corridors may be the major determinants for functional localized re-entrant circuits creating the substrate for maintenance of AF.
Display omitted
Brugada syndrome (BrS) is an inherited autosomal dominant genetic disorder responsible for sudden cardiac death from malignant ventricular arrhythmia. The term “channelopathy” is nowadays used to ...classify BrS as a purely electrical disease, mainly occurring secondarily to loss-of-function mutations in the α subunit of the cardiac sodium channel protein Nav1.5. In this setting, arrhythmic manifestations of the disease have been reported in the absence of any apparent structural heart disease or cardiomyopathy. Over the last few years, however, a consistent amount of evidence has grown in support of myocardial structural and functional abnormalities in patients with BrS. In detail, abnormal ventricular dimensions, either systolic or diastolic dysfunctions, regional wall motion abnormalities, myocardial fibrosis, and active inflammatory foci have been frequently described, pointing to alternative mechanisms of arrhythmogenesis which challenge the definition of channelopathy. The present review aims to depict the status of the art of concealed arrhythmogenic substrates in BrS, often resulting from an advanced and multimodal diagnostic workup, to foster future preclinical and clinical research in support of the cardiomyopathic nature of the disease.
Non-invasive prenatal testing (NIPT) using cell-free DNA can detect fetal chromosomal anomalies with high clinical sensitivity and specificity. In approximately 0.1% of clinical cases, the NIPT ...result and a subsequent diagnostic karyotype are discordant. Here we report a case of a 32-year-old pregnant patient with a 44.1 Mb duplication on the short arm of chromosome 4 detected by NIPT at 12 weeks' gestation. Amniocentesis was carried out at 18 weeks' gestation, followed by conventional and molecular cytogenetic analysis on cells from the amniotic fluid. SNP array analysis found a
deletion of 1.2 Mb at chromosome 4, and this deletion was found to be near the critical region of the Wolf-Hirschhorn syndrome. A normal 46,XY karyotype was identified by G-banding analysis. The patient underwent an elective termination and molecular investigations on tissues from the fetus, and the placenta confirmed the presence of type VI true fetal mosaicism. It is important that a patient receives counselling following a high-risk call on NIPT, with appropriate diagnostic analysis advised before any decisions regarding the pregnancy are taken. This case highlights the importance of genetic counselling following a high-risk call on NIPT, especially in light of the increasing capabilities of NIPT detection of sub-chromosomal deletions and duplications.
ECG-documented AF recurrences were most frequent in Group 4 patients, with a significant correlation between BMI behavior and the time interval between subsequent recurrences (every 2.6 months Group ...4, 5.5 months Group 3, 13.7 months Group 2, and 15.2 months Group 1). ...we can confirm that BMI reduction has a preventive effect against AF recurrence and that the LEGACY study results can be applied also to Mediterranean patients.
The NeoChord procedure is an echo-guided trans-ventricular beating-heart mitral valve repair technique to treat degenerative mitral regurgitation (MR) due to prolapse and/or flail. The aim of this ...study is to analyze echocardiographic images to find pre-operative parameters to predict procedural success (≤moderate MR) at 3-year follow-up. Seventy-two consecutive patients with severe MR underwent the NeoChord procedure between 2015 and 2021. MV pre-operative morphological parameters were assessed using 3D transesophageal echocardiography with dedicated software (QLAB, Philips). Three patients died during their hospitalization. The remaining 69 patients were retrospectively analyzed. At follow-up, MR > moderate was found in 17 patients (24.6%). In the univariate analysis, end-systolic annulus area (12.5 ± 2.5 vs. 14.1 ± 2.6 cm
;
= 0.038), end-systolic annulus circumference (13.2 ± 1.2 vs. 14 ± 1.3 cm;
= 0.042), indexed left atrial volume (59 ± 17 vs. 76 ± 7 mL/m
;
= 0.041), and AF (25% vs. 53%;
= 0.042) were lower in the 52 patients with ≤ MR compared to those with > moderate MR. Annular dysfunction parameters were the best predictors of procedural success: 3D early-systolic annulus area (AUC 0.74;
= 0.004), 3D early-systolic annulus circumference (AUC 0.75;
= 0.003), and 3D annulus area fractional change (AUC 0.73;
= 0.035). Patient selection relying on 3D dynamic and static MA dimensions may improve the maintenance of procedural success at follow-up.
Arrhythmogenic cardiomyopathy (ACM) is an inherited heart disease characterized by fibro-fatty replacement of myocardium. Limited data is available concerning cardioembolic stroke. This study sought ...to determine the occurrence of cardioembolic ischemic events (CIEs) in ACM patients and to identify clinical and imaging predictors of CIEs.
Every consecutive ACM patient was enrolled. ECG, Holter monitoring or implantable cardiac devices were used to detect atrial arrhythmias (AAs). CIEs were defined according to TOAST classification.
In our cohort of 111 patients, CIEs were observed in eleven (10%) over a 12.9-year median follow-up, with an incidence of 7.9 event/1000 patient-year (HR 4.12 compared to general population). Mean age at the event was 42 ± 9 years. Female sex (p = 0.03), T-wave inversion (p = 0.03), RVOT dilatation (p = 0.006) and lower LVEF (p = 0.006) were associated with CIEs. Among patients with AAs (23/111, 20.7%), 5 (21.7%) experienced CIEs. CHA2DS2-VASc did not prove useful to define patients at higher risk of CIEs (p = 0.098). 60% of stroke suffering patients had a pre-event score between 0 and 1 (if female).
In ACM patients, CIEs are much more common than in general population and present a high burden at younger age. AAs relate to less than half of these events. In AAs patients, CHA2DS2-VASc is not useful to stratify those requiring oral anticoagulation. As a hypothesis-generating study, our research proposes the role of atrial myopathy, irrespective of AAs, as a pivotal factor in thrombogenesis risk, pointing out a definite unmet need in ACM therapeutic algorithm.
•In ACM patients, cardioembolic cerebral events are more frequent than expected and present a high burden even at younger age•Atrial fibrillation (AF) is correlated to less than half of these events•In ACM patients with AF, the CHA2DS2-VASc score is not useful to stratify those requiring oral anticoagulation
Every year, 80,000–100,000 ablation procedures take place in the United States and approximately 1% of these involve paediatric patients. As the paediatric population undergoing catheter ablation to ...treat dysrhythmia is constantly growing, involvement of anaesthesiologists in the cardiac electrophysiology laboratory is simultaneously increasing. Compared with the adult population, paediatric patients need deeper sedation or general anaesthesia (GA) to guarantee motionlessness and preserve comfort. As a result, the anaesthesiologist working in this setting should keep in mind heart physiopathology as well as possible interactions between anaesthetic drugs and arrhythmia. In fact, drug-induced suppression of accessory pathways (APs) conduction capacity is a major concern for completing a successful electrophysiology study (EPS). Nevertheless, the literature on this topic is scarce and the optimal type of anaesthesia in EPS and ablation procedures in children is still controversial. Thus, the main goal of the present review is to collect the literature published so far on the effects on cardiac conduction tissue of the drugs commonly employed for sedation/GA in the cath lab for EPS and ablation procedures to treat supraventricular tachycardia in patients aged <18 years.
•Anaesthetics can influence procedural outcomes by potentially affecting arrhythmia inducibility.•Tailoring the anaesthetic plan to each patient is crucial to achieving successful arrhythmia ablation.•Children exhibit unique characteristics that cannot be directly extrapolated from the adult population.•While general anaesthesia is largely adopted in elethrophisiology procedures it may affect procedural outcomes.•Future studies should compare general anaesthesia vs. sedation in terms of successful arrhythmia ablation and safety.
We report a case of patient with a single-chamber Hisian pacemaker who developed complete atrioventricular block and significant deterioration of the ventricular threshold and sensing after ...transcatheter aortic valve replacement. Revision of the implant was required, with ventricular lead extraction and replacement. (Level of Difficulty: Advanced.)
Display omitted
Abstract
Background
Arrhythmogenic cardiomyopathy (ACM) is an inherited heart muscle disease characterized by fibro-fatty replacement of predominantly right, left or biventricular myocardium. The ...clinical course of ACM is characterized by high risk of abrupt arrhythmic ventricular events, potentially leading to sudden cardiac death (SCD), and by progressive impairment of biventricular systolic function, which may lead to heart failure (HF)-related death, with an estimated overall yearly mortality ranging from 0.08% to 3.6% among studies.
Aim
This study sought to assess the long-term follow-up of a cohort of ACM patients from the Piedmont region of Italy, specifically evaluating the cumulative incidence of SCD, HF-related death and heart transplantat.
Methods
Patients who received a definite ACM diagnosis according to the latest available International Task Force Criteria (ITFC) at three different institutions in Piedmont (“Cardinal Massaia” Hospital of Asti, “Azienda Ospedaliera Ordine Mauriziano” of Turin and “Città della Salute e della Scienza” Hospital of Turin) between 1982 and March 2022 were prospectively enrolled. Clinical, electrocardiographic and imaging data were collected at baseline and during follow-up. The occurrence of two coprimary composite endpoints was evaluated, namely the arrhythmic composite endpoint of SCD or aborted SCD and the HF-related composite endpoint, including heart transplant for end-stage HF or cardiac death due to pump failure. Independent predictors of the coprimary composite endpoints were also assessed by means of multivariable analysis.
Results
The overall population included 97 patients; mean age at diagnosis was 38.2 ± 14.3 years; 66 (68%) patients were male. Median follow-up was 12.9 years (interquartile range 0-38 years). The composite arrhythmic outcome occurred in 10 (10.3%) patients and the composite HF outcome occurred in 13 (13.5%) patients. Overall, 19 (19.6%) patients died: seven (37%) patients died from SCD, four (21%) patients died of end-stage HF and eight (42%) patients died from non-cardiac causes. The cumulative incidence of cardiac death was 1.65% per year. Aborted SCD occurred in 3 (3%) patients, sustained ventricular tachycardias in 51 (52%), HF in 17 (18%) and atrial fibrillation in 21 (22%); forty-seven (48%) underwent an implantable cardioverter defibrillator implantation (ICD) and 8 (8%) patients underwent heart transplant. Mean interval between ACM diagnosis and heart transplant was 130.4 ± 75.3 months. At univariable analysis, the presence at baseline of terminal activation duration (TAD) ≥ 55 ms on surface electrocardiogram (p value 0.037), right atrium dilatation at transthoracic echocardiography (p value 0.021) and cardiac magnetic resonance imaging (p value 0.031) and palpitations as main clinical presentation (p value 0.017) were associated with the occurrence of the arrhythmic endpoint. At multivariable analysis, baseline TAD ≥ 55 msec was the only predictor of the composite arrhythmic endpoint (odds ratio 12.5, 95% confidence interval 1.29 to 121.26, p value 0.029). At univariable analysis, the presence of epsilon waves (p value 0.017), TAD ≥ 55 ms (p value 0.020) and T wave inversion in leads V1-V3 (p value 0.022) on surface electrocardiogram, HF (p value 0.018) or atrial arrhythmias (p value < 0.001) at presentation and New York Heart Association (NYHA) class III to IV at diagnosis (p value 0.002) were significantly associated with the composite HF-related endpoint. At multivariable analysis, the presence of atrial arrhythmias at presentation was the only predictor of the HF-related composite endpoint (odds ratio 10.8, 95% confidence interval 1.29 to 90.32, p value 0.028).
Conclusions
In this multicenter cohort of 97 ACM patients, the cumulative incidence of cardiac death was 1.65% per year at long-term follow-up. Arrhythmic death accounted for the majority of cardiac fatal events (64%), confirming the peculiar predisposition to SCD of ACM patients. Evolution towards end-stage HF and cardiac death due to pump failure were common.
PDF
