Management of Inherited Arrhythmia Syndromes: A HiRO Consensus Handbook on Process of Care.

Inherited arrhythmia syndromes are rare genetic conditions that predispose seemingly healthy individuals to sudden cardiac arrest and death. The Hearts in Rhythm Organization is a multidisciplinary Canadian network of clinicians, researchers, patients, and families that aims to improve care for patients and families with inherited cardiac conditions, focused on those that confer predisposition to arrhythmia and sudden cardiac arrest and/or death. The field is rapidly evolving as research discoveries increase. A streamlined, practical guide for providers to diagnose and follow pediatric and adult patients with inherited cardiac conditions represents a useful tool to improve health system utilization, clinical management, and research related to these conditions. This review provides consensus care pathways for 7 conditions, including the 4 most common inherited cardiac conditions that confer predisposition to arrhythmia, with scenarios to guide investigation, diagnosis, risk stratification, and management. These conditions include Brugada syndrome, long QT syndrome, arrhythmogenic right ventricular cardiomyopathy and related arrhythmogenic cardiomyopathies, and catecholaminergic polymorphic ventricular tachycardia. In addition, an approach to investigating and managing sudden cardiac arrest, sudden unexpected death, and first-degree family members of affected individuals is provided. Referral to specialized cardiogenetic clinics should be considered in most cases. The intention of this review is to offer a framework for the process of care that is useful for both experts and nonexperts, and related allied disciplines such as hospital management, diagnostic services, coroners, and pathologists, in order to provide high-quality, multidisciplinary, standardized care.

Les syndromes d'arythmie héréditaires sont des troubles génétiques rares qui prédisposent des personnes en apparence en bonne santé à un arrêt cardiaque soudain et à la mort. L'organisation Hearts in Rhythm Organization est un réseau multidisciplinaire canadien qui regroupe des cliniciens, des chercheurs ainsi que des patients et leurs proches dans le but d'améliorer les soins prodigués aux patients atteints de maladies cardiaques héréditaires et à leur famille, en particulier dans le cas des maladies qui entraînent une prédisposition à l'arythmie et à un arrêt cardiaque soudain et/ou à la mort. Puisque ce champ de recherche évolue rapidement, la mise au point d'un guide pratique et simple à l'intention des professionnels de la santé pour le diagnostic et le suivi des patients enfants et adultes présentant une maladie cardiaque héréditaire serait donc un outil intéressant pour améliorer l'utilisation du système de santé et la prise en charge clinique de ces maladies tout en orientant la recherche à ce propos. La présente synthèse expose les trajectoires de soins faisant l'objet d'un consensus pour sept maladies, dont les quatre maladies cardiaques héréditaires les plus courantes qui prédisposent à l'arythmie. Elle présente aussi des scénarios pour orienter les examens, le diagnostic, la stratification du risque et la prise en charge des patients. Ces maladies sont le syndrome de Brugada, le syndrome du QT long, la cardiomyopathie arythmogénique du ventricule droit et les cardiomyopathies arythmogènes associées, et la tachycardie ventriculaire polymorphe catécholaminergique. En outre, une approche pour la prise en charge de l'arrêt cardiaque soudain, de mort subite inattendue et des membres de la famille immédiate de la personne touchée est proposée. L'orientation vers des cliniques spécialisées en cardiogénétique doit être envisagée dans la plupart des cas. L'objectif est d'établir un cadre de soins qui soit utile pour les experts et les non-experts ainsi que pour les professionnels des domaines connexes, par exemple le personnel de l'administration hospitalière et des services diagnostiques, les coroners et les pathologistes, en vue d'offrir des soins multidisciplinaires normalisés de grande qualité.

Premature Ventricular Contractions Are Presaged by a Mechanically Abnormal Sinus Beat.

BACKGROUND: Frequent premature ventricular contractions (PVCs) can lead to cardiomyopathy; it is unclear if there are abnormal myocardial mechanics operative in the PVC and non-PVC beats. OBJECTIVES: The aim of this study was to investigate regional and global myocardial mechanics, including dyssynchrony, in patients with frequent PVCs. METHODS: Fifty-six consecutive patients referred for PVC ablation were prospectively studied. During sinus rhythm (SR) and PVC beats, left ventricular (LV) global longitudinal strain (GLS), LV dyssynchrony (measured as the SD of time to peak GLS), and dyssynergy (measured as maximum regional strain minus minimum regional strain at aortic valve closure) were quantified using 2-dimensional strain echocardiography. GLS, dyssynchrony, and dyssynergy were compared in remote SR, pre-PVC SR, PVC, and post-PVC SR beats. RESULTS: In SR beats remote from the PVC, GLS was -17.3% ± 4%, dyssynchrony was 49 ± 14 ms, and dyssynergy was 22% ± 9%. Myocardial mechanics were significantly abnormal during PVCs compared with remote SR beats (GLS -7.7% ± 3% [P < 0.001], dyssynchrony 115 ± 37 milliseconds [P < 0.001], and dyssynergy 26% ± 10% [P < 0.001]). There were significant mechanical abnormalities in the SR beat preceding the PVC, which demonstrated significantly lower LV strain (pre-PVC SR, -13% ± 4%; P < 0.001) and more dyssynchrony (pre-PVC SR, 63 ± 19 milliseconds; P < 0.001) compared with remote SR beats. Dyssynergy was significantly higher for pre-PVC SR and PVC beats compared with remote SR (pre-PVC SR, 25% ± 8% [P < 0.001]; PVC, 26% ± 10% [P < 0.001]). CONCLUSIONS: In patients with frequent PVCs, the SR beat preceding the PVC demonstrates significant mechanical abnormalities. This finding suggests that perturbations in cellular physiological processes such as excitation-contraction coupling may underlie the generation of frequent PVCs.

US hospital webpages on atrial fibrillation ablation: A potential source for misinformation.

INTRODUCTION: Atrial fibrillation (AF) ablation is performed worldwide. To attract patients, hospitals frequently have webpages that tout the success of the procedure. The information disseminated to the public via these webpages has not been systematically reviewed. Our objective was to assess accuracy of information delivered to the public on hospital websites in regard to atrial AF ablation. METHODS: From July 2019 to January 2020, we performed a Google search for all US hospitals registered with Medicare to see if they had a webpage describing AF ablation. Resulting hospital webpages were abstracted for data on AF ablation success rates and risks. Success rates over 86%, the highest success rate in the medical literature, were deemed exaggerated. RESULTS: Among 4805 hospitals, 487 had webpages describing AF ablation and 33 discussed success rates of AF ablation. Twelve percentage reported exaggerated success rates, 3% referred to ablation as a cure, and 2.8% referred to ablation as a tool to eliminate AF. Less than 10% of webpages describing AF ablation noted the potential need for a second ablation to achieve the stated success rate and merely 16% mentioned risks of the procedure. One percentage of webpages directly suggested AF ablation could reduce risk of stroke while others indirectly suggested it by discussing cessation of anticoagulation. Two webpages mentioned reduced mortality. CONCLUSION: US hospital webpages rarely discuss AF ablation. When discussed, there were concerning unsubstantiated claims regarding mortality, stroke prevention, and need for medical therapy. This could lead to some patients undergoing AF ablation based on faulty understanding.

Bi-atrial characterization of the electrical substrate in patients with atrial fibrillation.

BACKGROUND: Little is known regarding the characterization of electrical substrate in both atria in patients with atrial fibrillation (AF). METHODS: Eight consecutive patients undergoing AF ablation (five paroxysmal, three persistent) underwent electrical substrate characterization during sinus rhythm. Mapping of the left (LA) and right atrium (RA) was performed with the use of the HD Grid catheter (Abbott). Bipolar voltage maps were analyzed to search for low voltage areas (LVA), the following electrophysiological phenomena were assessed: (1) slow conduction corridors, and (2) lines of block. EGMs were characterized to search for fractionation. Electrical characteristics were compared between atria and between paroxysmal versus persistent AF patients. RESULTS: In the RA, LVAs were present in 60% of patients with paroxysmal AF and 100% of patients with persistent AF. In the LA, LVAs were present in 40% of patients with paroxysmal AF and 66% of patients with persistent AF. The areas of LVA in the RA and LA were 4.8±7.3 cm2 and 7.8±13.6 cm2 in patients with paroxysmal AF versus 11.7±3.0 cm2 and 2.1±1.8 cm2 in patients with persistent AF. In the RA, slow conduction corridors were present in 40.0% (paroxysmal AF) versus 66.7% (persistent AF) whereas in the LA, slow conduction corridors occurred in 20.0% versus 33.3% respectively (p = ns). EGM analysis showed more fractionation in persistent AF patients than paroxysmal (RA: persistent AF 10.8 vs. paroxysmal AF 4.7%, p = .036, LA: 10.3 vs. 4.1%, p = .108). CONCLUSION: Bi-atrial involvement is present in patients with paroxysmal and persistent AF. This is expressed by low voltage areas and slow conduction corridors whose extension progresses as the arrhythmia becomes persistent. This electrophysiological substrate demonstrates the important interplay with the pulmonary vein triggers to constitute the substrate for persistent arrhythmia.

Landing on the spot: Approaches to outflow tract PVCs; from ECG to EGMs to intracardiac echocardiography.

Premature ventricular complexes (PVCs) are increasingly recognized, as the use of ECG wearables becomes more widespread. In particular, PVCs arising from both the right ventricular outflow tract (RVOT) and left ventricular outflow tract (LVOT) comprise the majority of these arrhythmias and form a significant component of an electrophysiology practice. A keen understanding of the correlative anatomy of the outflow tracts, in addition to recognizing key ECG indices illustrating PVC sites of origin, are fundamental in preparing for a successful ablation. Patient selection, incorporating symptomatology, structural disease, and PVC burden can pose a challenge, though tools such as the ABC-VT risk score may help identify those patients with a higher risk of clinical deterioration. Utilizing intracardiac echocardiography to highlight salient anatomic features not visible with fluoroscopy allows for a more precise and safer ablation. Interpretation of intracardiac EGMs, and the careful examination for low amplitude highly fractionated pre-potentials, enhanced by the advent of new developed mapping/ablation catheters, remains crucial. Utilizing these tools will guide the electrophysiologist to an efficient and effective outflow tract PVC ablation.

Characterization of cardiac electrogram signals in atrial arrhythmias.

Despite significant advancements in 3D cardiac mapping systems utilized in daily electrophysiology practices, the characterization of atrial substrate remains crucial for the comprehension of supraventricular arrhythmias. During mapping, intracardiac electrograms (EGM) provide specific information that the cardiac electrophysiologist is required to rapidly interpret during the course of a procedure in order to perform an effective ablation. In this review, EGM characteristics collected during sinus rhythm (SR) in patients with paroxysmal atrial fibrillation (pAF) are analyzed, focusing on amplitude, duration and fractionation. Additionally, EGMs recorded during atrial fibrillation (AF), including complex fractionated atrial EGMs (CFAE), may also provide precious information. A complete understanding of their significance remains lacking, and as such, we aimed to further explore the role of CFAE in strategies for ablation of persistent AF. Considering focal atrial tachycardias (AT), current cardiac mapping systems provide excellent tools that can guide the operator to the site of earliest activation. However, only careful analysis of the EGM, distinguishing low amplitude high frequency signals, can reliably identify the absolute best site for RF. Evaluating macro-reentrant atrial tachycardia circuits, specific EGM signatures correspond to particular electrophysiological phenomena: the careful recognition of these EGM patterns may in fact reveal the best site of ablation. In the near future, mathematical models, integrating patient-specific data, such as cardiac geometry and electrical conduction properties, may further characterize the substrate and predict future (potential) reentrant circuits.

Complete Electroanatomic Imaging of the Diastolic Pathway Is Associated With Improved Freedom From Ventricular Tachycardia Recurrence.

BACKGROUND: The development of multielectrode mapping catheters has expanded the spectrum of mappable ventricular tachycardias (VTs). Full diastolic pathway recording has been associated with a high rate of VT termination during radiofrequency ablation as well as noninducibility at study end. However, the role of diastolic pathway mapping on VT recurrence has yet to be clearly elucidated. We aimed to explore the role of complete diastolic pathway activation mapping on VT recurrence. METHODS: Eighty-five consecutive patients who underwent VT ablation guided by high-density mapping were enrolled. During activation mapping, the presence of electrical activity in all segments of diastole defined the evidence of having had recorded the whole diastolic interval. Patients were categorized as having recorded the full diastolic pathway, partial diastolic pathway, or no diastolic pathway map performed. Recurrences of VT were defined as appropriate implantable cardioverter defibrillator therapies or on the basis of ECG-documented arrhythmia. RESULTS: Eighty-five patients were included. Complete recording of the diastolic pathway was achieved in 36/85 (42.4%) patients. Partial recording of the diastolic pathway of the clinical VT was achieved in 24/85 (28.2%) patients. No recording of the diastolic pathway of the clinical VT was feasible in 25/85 patients (29.4%). At a mean of 12.8 months, freedom from VT recurrence was 67% in the overall cohort. At a mean of 12.8 months, freedom from VT recurrence was 88%, 50%, and 55% in patients who had full diastolic activity recorded, partial diastolic activity recorded, or underwent substrate modification, respectively; the observed differences were statistically significant (P=0.02). CONCLUSIONS: Mapping of the entire diastolic pathway was associated with a higher freedom from VT recurrence as compared with partial diastolic pathway recording and substrate modification. The use of multielectrode mapping catheters in recording diastolic activity may help predict those VTs employing intramural circuits and further optimize ablation strategies.

Outer loop and isthmus in ventricular tachycardia circuits: Characteristics and implications.

BACKGROUND: The isthmus of ventricular tachycardia (VT) circuits has been extensively characterized. Few data exist regarding the contribution of the outer loop (OL) to the VT circuit. OBJECTIVE: The purpose of this study was to characterize the electrophysiological properties of the OL. METHODS: Complete substrate activation mapping during sinus rhythm (SR) and full activation mapping of the VT circuit with high-density mapping were performed. Maps were analyzed mathematically to reconstruct conduction velocities (CVs) within the circuit. CV >100 cm/s was defined as normal and <50 cm/s as slow. Electrograms along the entire circuit were analyzed for fractionation, duration, and amplitude. RESULTS: Six postmyocardial infarction patients were enrolled. The VT circuit was a figure-of-eight reentrant circuit in 4 patients and a single-loop circuit in 2 patients. The OL exhibited a mean of 1.9 ± 0.9 and 1.6 ± 0.5 corridors of slow conduction (SC) during VT and SR, respectively. SC in the OL were longer and faster than SC in the isthmus during SR. At the OL, SC sites showed local abnormal ventricular activity in 92%, and a bipolar voltage <0.5 mV was identified in 80.7%. Of the double-loop circuits, only 1 patient had fixed lines of block as isthmus boundaries, whereas in 3 patients the circuits were at least partially functional. CONCLUSION: In ischemic reentrant VT circuits, the OL contributes significantly to reentry with multiple corridors of SC. These corridors can result from structural or functional phenomena. Isthmus boundaries may correspond to functional or fixed lines of block.

Noninvasive programmed stimulation in the setting of ventricular tachycardia catheter ablation.

In the setting of catheter ablation of ventricular tachycardia (VT), invasive programmed ventricular stimulation (PVS) is considered an important tool to assess the (residual) inducibility of ventricular arrhythmias and determine the acute success of the procedure. In patients with cardiovascular implantable electronic devices, noninvasive programmed stimulation via implantable cardioverter-defibrillator (ICD) leads can be an alternative to the invasive PVS with intracardiac catheters. The advantages of noninvasive programmed stimulation include preprocedure planning of the electrophysiology procedure to ensure optimal conditions for successful catheter ablation of VT. Following the procedure, noninvasive programmed stimulation has been shown to be used as a guide for repeat early ablation, to offer better programming of ICD, to offer prognostic value regarding the VT recurrence, and to guide antiarrhythmic drug therapy. The noninvasive nature of noninvasive programmed stimulation makes it an attractive alternative to PVS in patients with ICD who have not undergone catheter ablation of VT to obtain prognostic value regarding the occurrence of VT.

Predictors of adverse outcome in patients with frequent premature ventricular complexes: The ABC-VT risk score.

BACKGROUND: No independently validated score currently exists for risk stratification of patients with frequent premature ventricular complexes (PVCs). OBJECTIVES: The purpose of this study was to develop a risk score to predict adverse events in patients with frequent PVCs. METHODS: We analyzed consecutive patients between 2012 and 2017 undergoing 14-day continuous monitoring with frequent PVCs (>5%) and concurrent echocardiography. We performed binary logistic regression to determine multivariate predictors of adverse left ventricular remodeling (left ventricular ejection fraction [LVEF] <45% or left ventricular end-diastolic volume index >75 mL/m2). A risk score was created using the log(odds ratio (OR)) of these predictors and validated prospectively to determine the risk of future adverse events in those with baseline LVEF >45%. An adverse event was defined as LVEF decline by 10%, heart failure hospitalization, or cardiovascular mortality. Two validation cohorts were used: follow-up from the original derivation cohort (cohort 1) and an independent Korean PVC registry (cohort 2). RESULTS: The derivation cohort comprised 206 patients with a mean PVC burden of 11.6% ± 6.2% and considerable daily fluctuation (minimum burden 7.3% ± 6.2% vs maximum 17.9% ± 8.0%). Independent predictors of adverse remodeling were as follows: superiorly directed PVC axis (OR 2.7; 1 point), PVC burden 10%-20% (OR 3.5; 2 points) and >20% (OR 4.4; 3 points), PVC coupling interval >500 ms (OR 4.7; 4 points), nonsustained ventricular tachycardia (OR 5.3; 4 points), which form the ABC-VT risk score. This score predicted future adverse events in both validation cohorts: cohort 1, hazard ratio 1.43; 95% confidence interval 1.19-1.73; P < .001 and cohort 2, hazard ratio 1.22; 95% confidence interval 1.05-1.42; P = .01. CONCLUSION: The ABC-VT score is a simple tool that predicts adverse left ventricular remodeling and future clinical deterioration in patients with frequent PVCs.

Predicting response to cardiac resynchronization therapy: Use of strict left bundle branch block criteria.

BACKGROUND: Cardiac resynchronization therapy (CRT) reduces morbidity and mortality in heart failure with reduced ejection fraction (HFrEF). CRT efficacy is greater in left bundle branch block (LBBB). This study aimed to determine if strict LBBB criteria predict an improved QRS duration and left ventricular ejection fraction (LVEF) response after CRT. METHODS: HFrEF patients who received a CRT device at a single quaternary center were included. Patients were divided into three groups based on baseline QRS morphology. Group 1 consisted of patients with strict LBBB. Group 2 had conventional LBBB, and group 3 had non-LBBB morphology. Outcomes assessed included change in QRS duration after CRT, change in LVEF, and all-cause mortality. RESULTS: In 231 patients, 56% of patients were in group 1, 29% were in group 2, and 15% were in group 3. Patients with strict LBBB had a significant reduction in QRS duration (-20.9 ± 12.4 ms) compared to conventional LBBB (6.7 ± 19.4 ms; P < 0.0001) and non-LBBB (3.9 ± 29.3 ms; P < 0.0001). Patients with strict LBBB had a significant increase in LVEF (19.5 ± 10.2) compared to conventional LBBB (5.3 ± 12.6; P < 0.0001) and non-LBBB (-1.3 ± 10.9; P < 0.0001). There was moderate negative correlation between changes in QRS duration and LVEF (correlation coefficient = -0.63, P < 0.0001). Strict LBBB criteria were associated with a significant reduction in mortality compared to conventional LBBB (odds ratio 0.49, 95% confidence interval 0.24 to 0.99; P = 0.046). CONCLUSIONS: Strict LBBB predicted a reduction in QRS duration and an increase in LVEF compared to conventional LBBB and non-LBBB morphology in patients with HFrEF who received CRT.

Implantation of cardiac resynchronization therapy devices using three leads by cephalic vein dissection approach.

AIMS: Percutaneous subclavian, axillary, and cephalic vein access are all used in conjunction for atrial and ventricular lead implantation, though no standard approach for cardiac resynchronization therapy (CRT) device implantation has been established. We describe an effective and a safe technique for implanting three leads via cephalic vein for CRT pacemaker and/or defibrillator implantations. METHODS AND RESULTS: A total of 171 consecutive patients undergoing de novo implantation of CRT pacemaker or defibrillator were included. Cephalic vein access was achieved by dissection and direct visualization. If the cephalic vein was inadequate, alternate means of access was determined after outset of the procedure. Procedural success rates and complications were recorded. Of the 171 de novo CRT implant attempts, 169 (98.8%) patients had successful implantation of all 3 leads on the first attempt. Of the 171 procedural attempts, 150 (87.7%) patients had all 3 leads placed via cephalic vein. Overall, complications occurred in 6 of 171 patients (3.5%) including initial and repeat procedures. These complications included seven lead dislodgements, two cases of diaphragmatic stimulation requiring lead revision, and one coronary sinus dissection without pericardial effusion. There were no cases of pneumothorax, pocket haematoma requiring evacuation, or infection. CONCLUSION: The triple lead via cephalic vein technique is safe and effective when used as a first approach for CRT device implantation.

A Systematic Review on the Progression of Paroxysmal to Persistent Atrial Fibrillation: Shedding New Light on the Effects of Catheter Ablation.

The progression from paroxysmal atrial fibrillation (AF) to persistent or long-term persistent forms has recently gained increasing attention. A growing amount of data has shown a significant morbidity and mortality associated with the transition. The aim of our systematic review was to assess the evidence regarding AF progression rates with different management approaches. Electronic databases were searched by using text words and relevant indexing to capture data on AF progression. Studies that considered progression from paroxysmal AF to a persistent or permanent form were included. The papers collected were divided into 2 groups: 1) general population studies (with almost exclusively medical therapy); and 2) studies that consider progression of AF subsequent to AF ablation. Twenty-one studies were included in the first group and 8 in the second group. In the first group, percentage of AF progression at 1 year ranged from 10% to 20%. Studies that included a longer follow-up detected a higher percentage of progression (from 50% to 77% after 12 years). In patients treated with catheter ablation, the percentage of progression was significantly lower (from 2.4% to 2.7% at 5 years' follow-up). The percentage of progression after catheter ablation did not change according to duration of follow-up. AF ablation is associated with significantly reduced progression to persistent forms compared with studies in the general population. Prevention of long-term AF progression may be a clinically relevant outcome after AF ablation. Further research is required to determine whether delaying progression of AF by catheter ablation reduces morbidity and mortality.