Arrhythmia-Induced Cardiomyopathy: Key Points
American College of Cardiology
JUNE 3, 2024
The following are key points to remember from a state-of-the-art review on arrhythmia-induced cardiomyopathy (AiCM).
Abstract 4138334: Identification and Immunological Characterization of Arrhythmia-Related Molecular Clusters in Ischemic Cardiomyopathy
Circulation
NOVEMBER 11, 2024
Ischemic cardiomyopathy, a severe cardiac condition resulting from prolonged myocardial ischemia, is characterized by ventricular dilation, dysfunction, and an increased risk of life-threatening arrhythmias. Arrhythmia, a common complication in ischemic cardiomyopathy, is associated with poor clinical outcomes.
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PO-06-075 SAFETY AND OUTCOMES OF CATHETER ABLATION PROCEDURES FOR VENTRICULAR ARRHYTHMIAS IN PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY
HeartRhythm
APRIL 30, 2024
Arrhythmias are common in patients with hypertrophic cardiomyopathy (HCM) and are associated with increased morbidity and mortality. Ventricular arrhythmias (VA) are less common compared to supraventricular arrhythmias yet are associated with worse outcomes in this patient population.
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Circulating microRNAs as biomarkers of Chagas cardiomyopathy
Frontiers in Cardiovascular Medicine
DECEMBER 18, 2023
Background Chagas cardiomyopathy (CHCM) is the most important clinical manifestation of Chagas disease. The analysis of cardiac miRNAs may contribute to predicting the progression to CHCM in Chagas indeterminate phase and/or to the differential diagnosis for cardiomyopathy.
Voltage mapping and right ventriculography to guide ablation for arrhythmogenic right ventricular cardiomyopathy ventricular tachycardia: a case report
Journal of Cardiothoracic Surgery
SEPTEMBER 22, 2024
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a family inherited cardiomyopathy associated with ventricular arrhythmias. With the development of molecular biology, histology, imaging, and other dia.
PO-01-091 MAVACAMTEN IS ASSOCIATED WITH LOWER VENTRICULAR ARRHYTHMIA BURDEN IN OBSTRUCTIVE HYPERTROPHIC CARDIOMYOPATHY PATIENTS
HeartRhythm
APRIL 30, 2024
It has been approved for use in patients with severe obstructive hypertrophic cardiomyopathy (oHCM). Currently, there has been little data on ventricular arrhythmias in patients treated with mavacamten.
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Phenotypes in Arrhythmogenic Cardiomyopathy
All About Cardiovascular System and Disorders
FEBRUARY 24, 2024
Arrhythmogenic Cardiomyopathy was better known as Arrhythmogenic Right Ventricular Dysplasia or ARVD and sometimes as Arrhythmogenic Right Ventricular Cardiomyopathy or ARVC earlier. A study published in JACC has compared the phenotypic expression and clinical outcomes in patients with arrhythmogenic cardiomyopathy [2].
PO-01-122 LONG-TERM FOLLOW-UP OF CARDIAC SYMPATHETIC DENERVATION IN GENETIC CARDIOMYOPATHY PATIENTS WITH REFRACTORY VENTRICULAR ARRHYTHMIA
HeartRhythm
APRIL 30, 2024
Cardiac Sympathetic Denervation (CSD) has proven efficacy in Long-QTS syndrome (LQTS) patients with refractory ventricular arrhythmia (VA).1 1 However, owing to the limited prevalence of this procedure, outcomes in patients with other genetic cardiomyopathies remain less understood.
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Risk of Atrial Arrhythmias in Patients with Ventricular Tachycardia in Arrhythmogenic Right Ventricular Cardiomyopathy
HeartRhythm
NOVEMBER 10, 2023
In arrhythmogenic right ventricular cardiomyopathy (ARVC), risk of atrial arrhythmias (AA) persists after ventricular tachycardia (VT) ablation.
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Extended monitoring detects more arrhythmias in hypertrophic cardiomyopathy, researchers find
Medical Xpress - ECG
APRIL 17, 2023
Thirty day electrocardiogram (ECG) monitoring in patients with hypertrophic cardiomyopathy (HCM) detects more arrhythmias than the standard 24 to 48 hours, according to late breaking science presented at EHRA 2023, a scientific congress of the European Society of Cardiology (ESC).
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PO-05-043 VENTRICULAR TACHYCARDIA AS AN EARLY MANIFESTATION OF A HERITABLE CARDIOMYOPATHY: THE EVOLVING SCOPE OF GENETIC PANELS IN THE WORKUP OF VENTRICULAR ARRHYTHMIAS
HeartRhythm
APRIL 30, 2024
Decisions as to the scope of workup for pathologic cardiac substrates in patients with ventricular arrhythmias can be challenging. Recent data suggests that in this scenario, disease specific genetic arrhythmia panels may miss heritable diagnoses over arrhythmic plus cardiomyopathy panels.(1)
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Abstract 4143083: Cardiomyopathy in Pregnancy Complicated by Loeys-Dietz Syndrome
Circulation
NOVEMBER 11, 2024
She underwent a cesarean section at 32 weeks given ongoing arrhythmia and heart failure symptoms. Her arrhythmia burden continued to lessen. Cardiomyopathy and arrhythmia have been described in a small number of cases. Initiation of guideline-directed medical therapy was limited by hypotension and pregnancy.
c-Src Is Responsible for Mitochondria-Mediated Arrhythmic Risk in Ischemic Cardiomyopathy
Circulation: Arrhythmia and Electrophysiology
AUGUST 30, 2024
Circulation: Arrhythmia and Electrophysiology, Ahead of Print. BACKGROUND:Increased mitochondrial Ca2+uptake has been implicated in the QT prolongation and lethal arrhythmias associated with nonischemic cardiomyopathy. In a heterologous expression system, c-Src could bind MCU and phosphorylate MCU tyrosines.
Krüpple-like factors in cardiomyopathy: emerging player and therapeutic opportunities
Frontiers in Cardiovascular Medicine
MARCH 6, 2024
Cardiomyopathy, a heterogeneous pathological condition characterized by changes in cardiac structure or function, represents a significant risk factor for the prevalence and mortality of cardiovascular disease (CVD). Research conducted over the years has led to the modification of definition and classification of cardiomyopathy.
Arrhythmias in children undergoing orthotopic heart transplantation
Frontiers in Cardiovascular Medicine
JANUARY 10, 2024
Introduction Heart transplantation (HT) is the only treatment option in children with heart failure secondary to cardiomyopathies and non-reparable congenital heart diseases. 24 patients (88.8%) were diagnosed with dilated cardiomyopathy, 2 (7.4%) with restrictive cardiomyopathy, and 1 (3.7%) with hypertrophic cardiomyopathy.
PO-04-038 EXERCISE IS NOT ASSOCIATED WITH CLINICAL ONSET OF CARDIOMYOPATHY OR INCREASED RISK OF SUSTAINED VENTRICULAR ARRHYTHMIAS IN PATIENTS WITH PATHOGENIC DESMOPLAKIN (DSP) VARIANTS
HeartRhythm
APRIL 30, 2024
Introduction: Pathogenic desmoplakin (DSP) variants are associated with left or biventricular arrhythmogenic cardiomyopathy. Exercise promotes penetrance and sustained ventricular arrhythmias (VA) in classic ARVC. It is unknown if exercise affects risk in DSP variant carriers.
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Challenging Arrhythmias — MIS-C Case Report (9-16.1-2024) - NOT_YET_FINISHED
Ken Grauer, MD
SEPTEMBER 15, 2024
For full discussion of the case — CLICK HERE — ECG Rhythm Overview: A 12-year-old boy was admitted to our hospital with severe myocardial dysfunction and chaotic rhythm with tachy- and bradycardic arrhythmias. Hope this helps — :) ECG-3 — I see sinus bradycardia and arrhythmia.
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The “arrhythmic” presentation of peripartum cardiomyopathy: case series and critical review of the literature
Frontiers in Cardiovascular Medicine
MARCH 13, 2024
Peripartum Cardiomyopathy (PPCM) is a polymorphic myocardial disease occurring late during pregnancy or early after delivery. The issue is relevant since some arrhythmias are associated to sudden cardiac death occurring in young patients, and the overall risk does not cease during the early postpartum period.
PO-05-189 CMR RADIOMIC SIGNATURES OF VENTRICULAR ARRHYTHMIA IN NON-ISCHEMIC CARDIOMYOPATHY
HeartRhythm
APRIL 30, 2024
Risk stratification in patients with non-ischemic cardiomyopathy (NICM) remains challenging. Although late gadolinium enhancement (LGE) CMR is recognized as a major risk factor for VT/VF, the prognostic value of LGE radiomics is unknown.
Imagenetics for Precision Medicine in Dilated Cardiomyopathy
Circulation: Genomic and Precision Medicine
FEBRUARY 28, 2024
Dilated cardiomyopathy (DCM) is a common heart muscle disorder of nonischemic etiology associated with heart failure development and the risk of malignant ventricular arrhythmias and sudden cardiac death. Circulation: Genomic and Precision Medicine, Ahead of Print.
Improved diagnosis of arrhythmogenic right ventricular cardiomyopathy using electrocardiographic deep-learning
HeartRhythm
AUGUST 18, 2024
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetic heart disease associated with life-threatening ventricular arrhythmias. Diagnosis of ARVC is based on the 2010 Task Force Criteria (TFC), application of which often requires clinical expertise at specialized centers.
Cardiac magnetic resonance for ventricular arrhythmias: a systematic review and meta-analysis
Heart BMJ
AUGUST 26, 2024
Background Cardiac magnetic resonance (CMR) allows comprehensive myocardial tissue characterisation, revealing areas of myocardial inflammation or fibrosis that may predispose to ventricular arrhythmias (VAs). A change in diagnosis after use of CMR ranged from 21% to 66% with a pooled average of 35% (29%–41%). to 2.42).
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Case Report: Multiple types of arrhythmias in a late-confirmed Danon disease
Frontiers in Cardiovascular Medicine
MARCH 27, 2024
Introduction Danon disease is an X-linked disorder caused by pathogenic variants in lysosome-associated membrane protein 2 ( LAMP2 ) gene, typically characterized by the triad of hypertrophic cardiomyopathy, myopathy, and intellectual disability. However, many patients may not present the typical presentation, especially in the early stage.
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Abstract 4134590: Procedural Related Major Adverse Events of Cardiac Catheterization in Pediatric Patients with Cardiomyopathy: Insights from IMPACT Registry
Circulation
NOVEMBER 11, 2024
Background:Despite the role of cardiac catheterization for hemodynamic assessment and endomyocardial biopsy (EMB) in children with cardiomyopathy, data on procedure-related major adverse events (MAE) in this population is lacking. We aim to describe the rate of MAE in children with cardiomyopathy undergoing cardiac catheterization.
Catheter ablation in patients with atrial fibrillation and dilated cardiomyopathy
Frontiers in Cardiovascular Medicine
JANUARY 16, 2024
Nevertheless, studies on the long-term outcomes of catheter ablation in patients with dilated cardiomyopathy are limited. The recurrence rate of atrial arrhythmia was 30.4% ( n = 14) after an average follow-up duration of 7.7 Introduction Catheter ablation is an effective and safe strategy for treating atrial fibrillation patients.
Efficacy and Clinical Outcomes of Catheter Ablation for Atrial Arrhythmia in Cardiac Amyloidosis
HeartRhythm
AUGUST 13, 2024
Cardiac amyloidosis (CA) involves the abnormal accumulation of misfolded proteins in the heart muscle, leading to restrictive cardiomyopathy.1 Atrial arrhythmias (AA), particularly atrial fibrillation (AF), are prevalent in CA and may impact long-term cardiovascular outcomes, prompting an investigation into early rhythm management strategies.2
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40 ATRIAL TACHYARRHYTHMIAS IN ARRHYTHMOGENIC CARDIOMYOPATHY
HeartRhythm
NOVEMBER 27, 2023
Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by replacement of ventricular myocardial by fibrofatty scar tissue which underlies morpho-functional ventricular abnormalities and life-threatening ventricular arrhythmias potentially responsible of sudden cardiac death (SCD), mostly in young people and athletes 1,2.
An overview of the treatments for hypertrophic cardiomyopathy
Frontiers in Cardiovascular Medicine
JUNE 3, 2024
Hypertrophic cardiomyopathy (HCM) is a very prevalent inherited disease with a wide global distribution and a prevalence rate of approximately 0.2% in the general population. Left ventricular hypertrophy (LVH) caused by sarcomere mutation is the primary reason of HCM.
Genetic Atrial Cardiomyopathies: Common Features, Specific Differences, and Broader Relevance to Understanding Atrial Cardiomyopathy
Circulation: Arrhythmia and Electrophysiology
NOVEMBER 29, 2023
Circulation: Arrhythmia and Electrophysiology, Ahead of Print. Atrial cardiomyopathy is a condition that causes electrical and contractile dysfunction of the atria, often along with structural and functional changes.
Association of epicardial and intramyocardial fat with ventricular arrhythmias
HeartRhythm
AUGUST 25, 2023
Among patients with ischemic cardiomyopathy (ICM) and nonischemic cardiomyopathy (NICM), myocardial fibrosis is associated with an increased risk for ventricular arrhythmia (VA). Growing evidence suggests that myocardial fat contributes to ventricular arrhythmogenesis.
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PO-01-042 ARRHYTHMIC PROFILE AND PREDICTORS IN PATIENTS WITH DUCHENNE MUSCULAR DYSTROPHY CARDIOMYOPATHY
HeartRhythm
APRIL 30, 2024
Duchenne muscular dystrophy (DMD) is an X-linked inherited disorder characterized by progressive muscle wasting and dilated cardiomyopathy. With improved multi-disciplinary care patients with left ventricular (LV) dysfunction surviving to older ages may be at risk for arrhythmias.
DH-482888-005 MULTIMODAL EXPLAINABLE ARTIFICIAL INTELLIGENCE IDENTIFIES PATIENTS WITH NON-ISCHAEMIC CARDIOMYOPATHY AT RISK OF LETHAL VENTRICULAR ARRHYTHMIAS
HeartRhythm
APRIL 30, 2024
The efficacy of implantable cardioverter-defibrillators (ICD) in patients with a non-ischaemic cardiomyopathy (NICM) is being debated. Deep Learning models enable feature extraction from high-dimensional data, such as cardiac MRI (CMR) and ECG.
AAV-Mediated Delivery of Plakophilin-2a Arrests Progression of Arrhythmogenic Right Ventricular Cardiomyopathy in Murine Hearts: Preclinical Evidence Supporting Gene Therapy in Humans
Circulation: Genomic and Precision Medicine
JANUARY 30, 2024
BACKGROUND:Pathogenic variants in PKP2 (plakophilin-2) cause arrhythmogenic right ventricular cardiomyopathy, a disease characterized by life-threatening arrhythmias and progressive cardiomyopathy leading to heart failure. Circulation: Genomic and Precision Medicine, Ahead of Print. In contrast, AAVrh.74-PKP2a–mediated
ECG-only Explainable Deep Learning Algorithm Predicts the Risk for Malignant Ventricular Arrhythmia in Phospholamban Cardiomyopathy
HeartRhythm
FEBRUARY 22, 2024
Arg14del) variant carriers are at risk of developing malignant ventricular arrhythmias (MVA). Phospholamban (PLN) p.(Arg14del) Accurate risk stratification allows for timely implantation of intracardiac defibrillators (ICD) and is currently performed using a multimodality prediction model.
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Sinus arrest in a p.Arg160X-DSP-positive patient without evidence of desmoplakin-mediated cardiomyopathy: a case report
Frontiers in Cardiovascular Medicine
DECEMBER 18, 2023
Background Pathogenic/Likely pathogenic variants in DSP -encoded desmoplakin are strongly associated with arrhythmogenic cardiomyopathy (ACM). Workup including echocardiography, cardiac magnetic resonance imaging, and Holter monitor did not show evidence of ACM or significant arrhythmias.
Author’s Reply to Regarding the risk of atrial arrhythmias in arrhythmogenic right ventricular cardiomyopathy patients undergoing ventricular tachycardia ablation
HeartRhythm
APRIL 2, 2024
Cheng and Zhang to our paper1, we note that despite ARVC being a relatively uncommon cardiomyopathy, we uniquely report on a large clinical experience with very long-term follow-up after VT ablation and confirm a high incidence of atrial flutter (AFL) and the effectiveness and low risk of catheter ablation of AFL. In response to Drs.
Pitfalls in the diagnosis of arrhythmogenic right ventricular cardiomyopathy
HeartRhythm
SEPTEMBER 14, 2023
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic cardiomyopathy resulting from disordered function of the intercalated discs with mainly arrhythmic manifestation, which can be difficult to diagnose because of its variable presentation. The incidence of ARVC is reported to be 1:2000 to 1:5000.
PO-02-097 SCAR BURDEN DETERMINED BY MRI IS ASSOCIATED WITH EXERCISE RELATED CHANGES IN ACTIVATION PATTERN IN HYPERTROPHIC CARDIOMYOPATHY
HeartRhythm
APRIL 30, 2024
Hypertrophic cardiomyopathy (HCM) is associated with a predisposition to lethal ventricular arrhythmias. LGE scar burden on MRI is thought to contribute to this risk, but its impact on electrophysiological substrate is not well understood.
Role of arrhythmic phenotype in prognostic stratification and management of dilated cardiomyopathy
European Journal of Heart Failure
FEBRUARY 26, 2024
Dilated cardiomyopathy with arrhythmic phenotype. Abstract Aims Dilated cardiomyopathy (DCM) with arrhythmic phenotype combines phenotypical aspects of DCM and predisposition to ventricular arrhythmias, typical of arrhythmogenic cardiomyopathy. During a median follow-up of 6 years (interquartile range 1.6–12.1),
Restoration of calcium release synchrony: A novel target for heart failure and ventricular arrhythmia
HeartRhythm
SEPTEMBER 4, 2023
An abnormal myocardial Ca2+ transient is linked to heart failure and ventricular arrhythmias. This narrative review evaluates the role of aberrant Ca2+ release synchrony in the pathophysiology of cardiomyopathies and ventricular arrhythmias.
Hypertrophic Cardiomyopathy
All About Cardiovascular System and Disorders
APRIL 30, 2024
Hypertrophic cardiomyopathy is a genetic disorder with a guarded prognosis which occurs in about 1:500 individuals. The most common symptom of hypertrophic cardiomyopathy is dyspnoea which occurs in 90% of cases and is due to elevated left ventricular diastolic pressures as a consequence of the diastolic dysfunction.
PO-01-174 NUCLEOLAR STRESS AND TRANSCRIPTIONAL REPROGRAMMING IN ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY
HeartRhythm
APRIL 30, 2024
Arrhythmogenic right ventricular cardiomyopathy (ARVC), often caused by mutations in Plakophilin-2 (PKP2) is characterized by high propensity to life-threatening arrhythmias and loss of myocytes.
Clinical presentations leading to arrhythmogenic left ventricular cardiomyopathy
Open Heart
APRIL 20, 2022
Objectives To describe a cohort of patients with arrhythmogenic left ventricular cardiomyopathy (ALVC), focusing on the spectrum of the clinical presentations. Twenty-one (41%) had normal echocardiogram, 13 (25%) a hypokinetic non-dilated cardiomyopathy (HNDC) and 17 (33%) a dilated cardiomyopathy (DCM).
Management of arrhythmogenic right ventricular cardiomyopathy
Heart BMJ
JANUARY 10, 2024
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease characterised by fibrofatty replacement of the ventricular myocardium due to specific mutations, leading to ventricular arrhythmias and sudden cardiac death. channels, Connexin 43 and Wnt signalling, potentially modifying myocardial fibrosis.
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