Lifestyle physical activity and rapid-rate non-sustained ventricular tachycardia in arrhythmogenic cardiomyopathy.

OBJECTIVE: To investigate the association of accelerometer-measured lifestyle physical activity with rapid-rate non-sustained ventricular tachycardias (RR-NSVTs) in patients with arrhythmogenic cardiomyopathy (AC). METHODS: This multicentre, observational study enrolled 72 patients with AC, including right, left and biventricular forms of the disease, with underlying desmosomal and non-desmosomal mutations. Lifestyle physical activity, objectively monitored with accelerometers (ie, movement sensors) and RR-NSVT, identified as >188 bpm and >18 beats from a textile Holter ECG for 30 days. RESULTS: Sixty-three patients with AC (38±17.6 years, 57% men) were included. A total of 17 patients experienced ≥1 RR-NSVTs, and a total of 35 events were recorded. The odds of occurrence of ≥1 RR-NSVT during the recording did not increase as a function of either total physical activity (OR 0.95, 95% CI (CI95%) 0.68 to 1.30 for 60 min increase) or moderate-to-vigorous activities (OR 0.89, CI95% 0.71 to 1.08 for 5 min increase). Participants presenting RR-NSVTs during the recording (n=17) did not present greater odds of RR-NSVT in the days with more time either in total physical activity (OR 1.05, CI95% 0.84 to 1.29 for additional 60 min) or moderate-to-vigorous activities (OR 1.05, CI95% 0.97 to 1.12 for additional 5 min). Physical activity levels were neither different between the patients with and without RR-NSVTs during the recording period nor in the days of occurrence of RR-NSVT compared with the rest of the days. Finally, 4 of the 35 RR-NSVTs recorded in the 30 days occurred during physical activity (3 during moderate-to-vigorous intensity and 1 during light-intensity activities). CONCLUSIONS: These findings suggest that lifestyle physical activity is not associated with RR-NSVTs in patients with AC.

Feasibility and safety of zero-fluoroscopy left bundle branch pacing: An initial experience.

INTRODUCTION: Left bundle branch pacing (LBBP) has emerged in recent years as a new pacing modality, providing patients with a narrower paced QRS than conventional pacing and stable pacing parameters. At the same time, there is a growing concern about the use of fluoroscopy in pacemaker implantations, given its harmful effects on both patients and operators. However, there are no prior experiences of zero-fluoroscopy in LBBP procedure. METHODS: We conducted an observational prospective study recruiting consecutive patients that underwent zero-fluoroscopy LBBP pacemaker implantation. A 6-month follow-up visit was programmed for every patient. The main goal of our study was to assess the efficacy, feasibility, and safety of the procedure. RESULTS: From January 2021 to February 2022, we included 10 patients, 8 males. The average age was 63 ± 4 years. The procedure was successful in all patients. We observed a significant reduction in paced QRS width compared with basal QRS width (149 ± 31.9 vs. 116 ± 15.6 ms, p = .02). All device parameters remained stable at 6-month follow-up: no significant differences in mean impedance (700.5 ± 136.4 vs. 494 ± 72.7 Ohm, p = .09), capture threshold (0.67 ± 0.2 vs. 0.83 ± 0.2 V @ 0.4 ms, p = .27) or endocardial V-wave amplitude (10.6 ± 5.2 vs. 13.9 ± 6.3 mV, p = .19). No complications were reported in any case. CONCLUSION: Zero-fluoroscopy LBBP is feasible and safe, and it may be considered in cases where radiation exposure is contraindicated or especially undesirable. Future randomized clinical trials are needed for the widespread use of this new technique.

Local impedance and contact force guidance to predict successful cavotricuspid isthmus ablation with a zero-fluoroscopy approach.

Introduction: A new technology capable of monitoring local impedance (LI) and contact force (CF) has recently been developed. At the same time, there is growing concern regarding catheter ablation performed under fluoroscopy guidance, due to its harmful effects for both patients and practitioners. The aim of this study was to assess the safety and effectiveness of zero-fluoroscopy cavotricuspid isthmus (CTI) ablation monitoring LI drop and CF as well as to elucidate if these parameters can predict successful radiofrequency (RF) applications in CTI ablation. Methods: We conducted a prospective observational study recruiting 50 consecutive patients who underwent CTI ablation. A zero-fluoroscopy approach guided by the combination of LI drop and CF was performed. In each RF application, CF and LI drop were monitored. A 6-month follow-up visit was scheduled to assess recurrences. Results: A total of 767 first-pass RF applications were evaluated in 50 patients. First-pass effective RF applications were associated with greater LI drops: absolute LI drops (30.05 ± 6.23 Ω vs. 25.01 ± 5.95 Ω), p = 0.004) and relative LI drops (-23.3 ± 4.9% vs. -18.3 ± 5.6%, p = 0.0005). RF applications with a CF between 5 and 15 grams achieved a higher LI drop compared to those with a CF below 5 grams (29.4 ± 8.76 Ω vs. 24.8 ± 8.18 Ω, p < 0.0003). However, there were no significant differences in LI drop between RF applications with a CF between 5 and 15 grams and those with a CF beyond 15 grams (29.4 ± 8.76 Ω vs. 31.2 ± 9.81 Ω, p = 0.19). CF by itself, without considering LI drop, did not predict effective RF applications (12.3 ± 7.54 g vs. 11.18 ± 5.18 g, p = 0.545). Successful CTI ablation guided by a zero-fluoroscopy approach was achieved in all patients. Only one patient experienced a recurrence during the 6-month follow-up. Conclusions: LI drop (absolute and relative values) appears to be a good predictor of successful RF applications to achieve CTI conduction block. The optimal CF to achieve a good LI drop is between 5 and 15 g. A zero-fluoroscopy approach guided by LI and CF was feasible, effective, and safe.

Magnetic resonance imaging in patients with cardiac implantable electronic devices: A prospective study.

BACKGROUND: The number of patients with cardiac implantable electronic devices in whom magnetic resonance imaging (MRI) is indicated is constantly increasing. The potential risk of electromagnetic interference has limited its use and it is still contraindicated by the Food and Drug Administration in some cases. The aim of this study is to evaluate the safety and efficacy of MRI in these patients. METHODS: A prospective registry comprising patients with a pacemaker (PM) or implantable cardioverter-defibrillator (ICD), MRI-conditional or not, who were candidates for MRI (at 1.5 T) with no suitable alternative diagnostic technique. All devices were programmed before the procedure and patients were monitored throughout the test. Clinical, electrical, and technical parameters were evaluated before and after MRI. RESULTS: 147 MRI examinations (132 PM and 15 ICD) were performed. There were no clinical events or significant differences in the electrical parameters of the leads after MRI. A variation in the impedance of the ventricular leads was detected, although the difference was not clinically relevant. In one patient with a PM, a failure in release of the safety impulse was detected in the auto-threshold test, although the threshold was correctly determined. In 11 of the 17 thoracic MRIs, image artifacts were detected, preventing the diagnosis in two of them. CONCLUSIONS: In patients with cardiac implantable electronic devices, MRIs performed under a specific protocol has been shown to be safe in the short term even in the thoracic region, as well as interpretable in most cases.

Improved procedural workflow for catheter ablation of paroxysmal AF with high-density mapping system and advanced technology: Rationale and study design of a multicenter international study.

BACKGROUND: The antral region of pulmonary veins (PV)s seems to play a key role in a strategy aimed at preventing atrial fibrillation (AF) recurrence. Particularly, low-voltage activity in tissue such as the PV antra and residual potential within the antral scar likely represent vulnerabilities in antral lesion sets, and ablation of these targets seems to improve freedom from AF. The aim of this study is to validate a structured application of an approach that includes the complete abolition of any antral potential achieving electrical quiescence in antral regions. METHODS: The improveD procEdural workfLow for cathETEr ablation of paroxysmal AF with high density mapping system and advanced technology (DELETE AF) study is a prospective, single-arm, international post-market cohort study designed to demonstrate a low rate of clinical atrial arrhythmias recurrence with an improved procedural workflow for catheter ablation of paroxysmal AF, using the most advanced point-by-point RF ablation technology in a multicenter setting. About 300 consecutive patients with standard indications for AF ablation will be enrolled in this study. Post-ablation, all patients will be monitored with ambulatory event monitoring, starting within 30 days post-ablation to proactively detect and manage any recurrences within the 90-day blanking period, as well as Holter monitoring at 3, 6, 9, and 12 months post-ablation. Healthcare resource utilization, clinical data, complications, patients' medical complaints related to the ablation procedure and patient's reported outcome measures will be prospectively traced and evaluated. DISCUSSION: The DELETE AF trial will provide additional knowledge on long-term outcome following a structured ablation workflow, with high density mapping, advanced algorithms and local impedance technology, in an international multicentric fashion. DELETE AF is registered at ClinicalTrials.gov (NCT05005143).

Results of catheter ablation with zero or near zero fluoroscopy in pediatric patients with supraventricular tachyarrhythmias.

INTRODUCTION AND OBJECTIVES: Ionizing radiation exposure in catheter ablation procedures carries health risks, especially in pediatric patients. Our aim was to compare the safety and efficacy of catheter ablation guided by a nonfluoroscopic intracardiac navigation system (NFINS) with those of an exclusively fluoroscopy-guided approach in pediatric patients. METHODS: We analyzed catheter ablation results in pediatric patients with high-risk accessory pathways or supraventricular tachycardia referred to our center during a 6-year period. We compared fluoroscopy-guided procedures (group A) with NFINS guided procedures (group B). RESULTS: We analyzed 120 catheter ablation procedures in 110 pediatric patients (11±3.2 years, 70% male); there were 62 procedures in group A and 58 in group B. We found no significant differences between the 2 groups in procedure success (95% group A vs 93.5% group B; P=.53), complications (1.7% vs 1.6%; P=.23), or recurrences (7.3% vs 6.9%; P = .61). However, fluoroscopy time (median 1.1minutes vs 12minutes; P <.0005) and ablation time (median 96.5seconds vs 133.5seconds; P=.03) were lower in group B. The presence of structural heart disease was independently associated with recurrence (P=.03). CONCLUSIONS: The use of NFINS to guide catheter ablation procedures in pediatric patients reduces radiation exposure time. Its widespread use in pediatric ablations could decrease the risk of ionizing radiation.

Predictores de fibrilación auricular en pacientes con ictus embólico de origen indeterminado / Atrial fibrillation predictors in patients with embolic stroke of undetermined source

Antecedentes y objetivos: En el accidente cerebrovascular embólico de origen indeterminado (ESUS) la detección de fibrilación auricular (FA) conlleva un cambio de tratamiento y una reducción drástica en la incidencia de nuevos ictus. Es necesario determinar qué pacientes se benefician en mayor medida de una monitorización electrocardiográfica prolongada. Nuestro objetivo fue la búsqueda de predictores electrocardiográficos y ecocardiográficos de FA en pacientes con ESUS.Materiales y métodosSe diseñó un estudio observacional de cohortes en el que se incluyeron 95 pacientes consecutivos que ingresaron por ESUS en un hospital terciario. A todos se les realizó un electrocardiograma (ECG), un Holter electrocardiograma (Holter-ECG) de 24h y un ecocardiograma durante el ingreso. Se realizó un seguimiento presencial durante 2años mediante Holter-ECG de 24h, trimestral durante el primer año y semestral durante el segundo.ResultadosDurante el seguimiento se detectó FA en 11 pacientes (11,6%), siendo la tasa detección del 3,2% a los 6meses, del 7,4% a los 12meses y del 11,6% a los 18 y a los 24meses. Las variables que se relacionaron de forma independiente con el desarrollo de FA fueron la dilatación en grado moderado o severo de la aurícula izquierda (AI) (p=0,02), el bloqueo interauricular avanzado (BIA-A) (p=0,04) y la presencia de más de 1.000 extrasístoles auriculares (EA) en Holter-ECG de 24h (p=0,01).ConclusionesLa dilatación en un grado moderado o severo de AI, el BIA-A y la presencia de más de 1.000 EA en Holter-ECG de 24h se comportan como predictores independientes de FA en pacientes con ESUS. (AU)

Background and objectives: Atrial fibrillation (AF) detection in patients with embolic stroke of underdetermined source (ESUS) entails a change of medical treatment and a significant decrease in the incidence of new strokes. It is necessary to determine which patients would benefit more from prolonged electrocardiographic monitoring. Our aim was to find electrocardiographic and echocardiographic AF predictors in patients with ESUS.MethodsWe performed a cohort study that included 95 consecutive patients admitted to the hospital because of an ESUS. An electrocardiogram, each subject in the study underwent a 24-hour Holter-electrocardiogram (Holter-ECG) and an echocardiogram. A 2-year follow up was also conducted, with a 24-hour Holter-ECG every 3months for the first year, and every 6months during the second one.ResultsDuring the follow-up, AF was detected in 11 patients (11.6%), with a detection rate of 3.2% at 6months, 7.4% at 12months, and 11.6% at 18months as well as at 24months. The variables that were independently related to AF detection included moderate or severe left atrium dilation (P=.02), interatrial advanced block (P=.04) and more than 1000 premature atrial beats on 24-hour Holter-ECG (P=.01).ConclusionsModerate or severe atrial dilation, interatrial advanced block, and the presence of more than 1000 premature atrial beats on 24-hour Holter-ECG behave as AF predictors in patients with ESUS. (AU)

His-Bundle Pacing in a Patient With Tricuspid and Mitral Prosthetic Valves Without Suitable Coronary Veins for Lead Placement.

Atrioventricular block in patients with a prosthetic tricuspid valve and a pacemaker with a dysfunctional epicardial lead is not uncommon. In such instances, coronary sinus lead placement is the preferred option, but it has a failure rate of 10%-15%. An atrial transseptal left ventricular lead placement has been proposed as an alternative, but this approach is not feasible in patients with a prosthetic mitral valve. This analysis represents the first reported case of His-bundle pacing from the atria in a patient with prosthetic tricuspid and mitral valves, with no suitable coronary veins for lead placement.

Le bloc auriculo-ventriculaire n'est pas rare chez les patients ayant reçu une valve tricuspide prothétique et porteurs d'un stimulateur cardiaque dont la sonde épicardique est dysfonctionnelle. Dans de tels cas, le positionnement de la sonde sur le sinus coronaire est l'option à privilégier, mais son taux d'échec varie entre 10 et 15 %. L'implantation de la sonde sur le ventricule gauche par la voie transsetale a été proposée à titre de solution de rechange, mais cette approche n'est pas envisageable chez les patients ayant reçu une valve mitrale prothétique. La présente analyse constitue le premier cas de stimulation du faisceau de His à partir des oreillettes chez un patient ayant reçu des valves tricuspides et mitrales prothétiques, en l'absence de veines coronaires se prêtant à l'implantation de la sonde.

[Atrial fibrillation predictors in patients with embolic stroke of undetermined source]. / Predictores de fibrilación auricular en pacientes con ictus embólico de origen indeterminado.

BACKGROUND AND OBJECTIVES: Atrial fibrillation (AF) detection in patients with embolic stroke of underdetermined source (ESUS) entails a change of medical treatment and a significant decrease in the incidence of new strokes. It is necessary to determine which patients would benefit more from prolonged electrocardiographic monitoring. Our aim was to find electrocardiographic and echocardiographic AF predictors in patients with ESUS. METHODS: We performed a cohort study that included 95 consecutive patients admitted to the hospital because of an ESUS. An electrocardiogram, each subject in the study underwent a 24-hour Holter-electrocardiogram (Holter-ECG) and an echocardiogram. A 2-year follow up was also conducted, with a 24-hour Holter-ECG every 3months for the first year, and every 6months during the second one. RESULTS: During the follow-up, AF was detected in 11 patients (11.6%), with a detection rate of 3.2% at 6months, 7.4% at 12months, and 11.6% at 18months as well as at 24months. The variables that were independently related to AF detection included moderate or severe left atrium dilation (P=.02), interatrial advanced block (P=.04) and more than 1000 premature atrial beats on 24-hour Holter-ECG (P=.01). CONCLUSIONS: Moderate or severe atrial dilation, interatrial advanced block, and the presence of more than 1000 premature atrial beats on 24-hour Holter-ECG behave as AF predictors in patients with ESUS.

Prognostic impact of misdiagnosis of cardiac channelopathies as epilepsy.

INTRODUCTION: Cardiac channelopathies are a frequent cause of sudden cardiac death (SCD) and often manifest with convulsive syncope, leading to a misdiagnosis of epilepsy. We aim to evaluate the clinical impact of epilepsy misdiagnosis in a cohort of patients with cardiac channelopathies. METHODS: Fifty probands/families with a cardiac channelopathy were included. We retrospectively collected information from medical records to identify all patients who presented with convulsive syncope and were diagnosed with epilepsy after neurological evaluation. Clinical data and outcome were compared with those of patients without a previous epilepsy diagnosis. RESULTS: Eight patients had a previous diagnosis of epilepsy. At first episode, 3 of them presented a positive family history of SCD and 5 showed a pathological electrocardiogram; half presented with sudden cardiac arrest (SCA) and the rest with recurrent syncope despite treatment with 1 or more anti-epileptic drugs. Five patients had long QT syndrome, 2 had catecholaminergic polymorphic ventricular tachycardia, and 1 had Brugada syndrome. Epilepsy misdiagnosis was associated with an increased risk of SCA/SCD (OR 6.92, P = .04), a delay of 12 years (P = .047) in correct diagnosis, and a delay from first symptom to channelopathy diagnosis of 18.45 years (P < .0001). CONCLUSION: Cardiac channelopathy patients can be misdiagnosed with epilepsy. This involves a delayed diagnosis, a delay from the first symptom to a correct diagnosis, and an increased risk of SCA/SCD.

Formin Homology 2 Domain Containing 3 (FHOD3) Is a Genetic Basis for Hypertrophic Cardiomyopathy.

BACKGROUND: The genetic cause of hypertrophic cardiomyopathy remains unexplained in a substantial proportion of cases. Formin homology 2 domain containing 3 (FHOD3) may have a role in the pathogenesis of cardiac hypertrophy but has not been implicated in hypertrophic cardiomyopathy. OBJECTIVES: This study sought to investigate the relation between FHOD3 mutations and the development of hypertrophic cardiomyopathy. METHODS: FHOD3 was sequenced by massive parallel sequencing in 3,189 hypertrophic cardiomyopathy unrelated probands and 2,777 patients with no evidence of cardiomyopathy (disease control subjects). The authors evaluated protein-altering candidate variants in FHOD3 for cosegregation, clinical characteristics, and outcomes. RESULTS: The authors identified 94 candidate variants in 132 probands. The variants' frequencies were significantly higher in patients with hypertrophic cardiomyopathy (74 of 3,189 [2.32%]) than in disease control subjects (18 of 2,777 [0.65%]; p < 0.001) or in the gnomAD database (1,049 of 138,606 [0.76%]; p < 0.001). FHOD3 mutations cosegregated with hypertrophic cardiomyopathy in 17 families, with a combined logarithm of the odds score of 7.92, indicative of very strong segregation. One-half of the disease-causing variants were clustered in a small conserved coiled-coil domain (amino acids 622 to 655); odds ratio for hypertrophic cardiomyopathy was 21.8 versus disease control subjects (95% confidence interval: 1.3 to 37.9; p < 0.001) and 14.1 against gnomAD (95% confidence interval: 6.9 to 28.7; p < 0.001). Hypertrophic cardiomyopathy patients carrying (likely) pathogenic mutations in FHOD3 (n = 70) were diagnosed after age 30 years (mean 46.1 ± 18.7 years), and two-thirds (66%) were males. Of the patients, 82% had asymmetric septal hypertrophy (mean 18.8 ± 5 mm); left ventricular ejection fraction <50% was present in 14% and hypertrabeculation in 16%. Events were rare before age 30 years, with an annual cardiovascular death incidence of 1% during follow-up. CONCLUSIONS: FHOD3 is a novel disease gene in hypertrophic cardiomyopathy, accounting for approximately 1% to 2% of cases. The phenotype and the rate of cardiovascular events are similar to those reported in unselected cohorts. The FHOD3 gene should be routinely included in hypertrophic cardiomyopathy genetic testing panels.