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Antiarrítmicos , Aleteo Atrial , Electrocardiografía , Taquicardia Ventricular , Verapamilo , Humanos , Antiarrítmicos/uso terapéutico , Aleteo Atrial/complicaciones , Aleteo Atrial/tratamiento farmacológico , Aleteo Atrial/fisiopatología , Informes de Casos como Asunto , Ablación por Catéter , Taquicardia Ventricular/complicaciones , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Ventricular/fisiopatología , Verapamilo/uso terapéuticoAsunto(s)
Aleteo Atrial , Taquicardia Ventricular , Verapamilo , Humanos , Aleteo Atrial/tratamiento farmacológico , Aleteo Atrial/fisiopatología , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Ventricular/fisiopatología , Verapamilo/uso terapéutico , Antiarrítmicos/uso terapéutico , ElectrocardiografíaRESUMEN
Heart Failure with preserved ejection fraction (HFpEF) has a high rate of sudden cardiac death (SCD) and empirical treatment is ineffective. We developed a novel preclinical model of metabolic HFpEF that presents with stress-induced ventricular tachycardia (VT). Mechanistically, we discovered arrhythmogenic changes in intracellular Ca2+ handling distinct from the changes pathognomonic for heart failure with reduced ejection fraction. We further show that dantrolene, a stabilizer of the ryanodine receptor Ca2+ channel, attenuates HFpEF-associated arrhythmogenic Ca2+ handling in vitro and suppresses stress-induced VT in vivo. We propose ryanodine receptor stabilization as a mechanistic approach to mitigation of malignant VT in metabolic HFpEF.
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Arritmias Cardíacas , Calcio , Dantroleno , Modelos Animales de Enfermedad , Insuficiencia Cardíaca , Canal Liberador de Calcio Receptor de Rianodina , Canal Liberador de Calcio Receptor de Rianodina/metabolismo , Animales , Calcio/metabolismo , Insuficiencia Cardíaca/metabolismo , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/fisiopatología , Arritmias Cardíacas/metabolismo , Arritmias Cardíacas/tratamiento farmacológico , Dantroleno/farmacología , Volumen Sistólico/efectos de los fármacos , Miocitos Cardíacos/metabolismo , Miocitos Cardíacos/efectos de los fármacos , Humanos , Taquicardia Ventricular/metabolismo , Taquicardia Ventricular/tratamiento farmacológico , Ratones , Masculino , Señalización del Calcio/efectos de los fármacosRESUMEN
Left ventricular assist devices (LVADs) are widely used as end-stage therapy in patients with advanced heart failure, whereas implantation increases the risks of development of sustained ventricular tachycardia at the later postimplantation stage. Therefore, this study aimed to evaluate the clinical efficacy of orally administered amiodarone and propranolol in 3 patients with ventricular tachycardia (VT) after LVAD implantation who were resistant to initial anti-antiarrhythmic drugs. This retrospective cohort study consisted of the initial evaluation of the clinical data of 14 adult patients who underwent implantation of LVAD between January 2019 and March 2021. A total of 3 patients with resistant VT were finally included. In all cases, the patients were initially administered amiodarone in the different doses intravenously to stabilize the critical condition, whereas its oral form along with that of propranolol was used as maintenance therapy in the first 2 cases. In the third case, amiodarone was withdrawn because of the risk of development of hyperthyroidism, while oral propranolol was used in the treatment. The assessment in the 16-month follow-up period after discharge did not show presence of non-sustained and sustained VT in all 3 cases. In the ventricular arrhythmia-free group, the total mortality rate within the follow-up period was 11.1â ±â 7.78 months in the 3 patients. We suggest that maintenance oral therapy of propranolol and amiodarone can significantly decrease the risks of complications in patients with VT after implantation of ventricular assist device in the long term.
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Amiodarona , Antiarrítmicos , Propranolol , Taquicardia Ventricular , Humanos , Amiodarona/administración & dosificación , Amiodarona/efectos adversos , Propranolol/administración & dosificación , Propranolol/uso terapéutico , Masculino , Antiarrítmicos/administración & dosificación , Estudios Retrospectivos , Administración Oral , Persona de Mediana Edad , Taquicardia Ventricular/tratamiento farmacológico , Femenino , Adulto , Insuficiencia Cardíaca/tratamiento farmacológico , AncianoRESUMEN
Cardiac arrhythmias cause a significant proportion of hospitalizations and physician contacts worldwide. By using antiarrhythmic drugs, cardiac arrhythmias can be effectively treated and the frequency of recurrences reduced. Atrial fibrillation and heart failure represent diseases in which antiarrhythmic drugs are more often used on a long-term basis. The aim of this article is to provide an overview of the most common antiarrhythmic drugs and their uses as well as to provide recommendations for adequate handling and use, especially in the outpatient setting. In addition to long-term use, some antiarrhythmic drugs are also administered for the acute management of supraventricular or ventricular tachycardia. Relevant contraindications, side effects and interactions must be considered, meaning that patients should be followed up when using these potent drugs. This article shows in detail what to consider when using antiarrhythmic drugs in order to ensure not only effective but also safe treatment.
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Antiarrítmicos , Arritmias Cardíacas , Humanos , Antiarrítmicos/uso terapéutico , Antiarrítmicos/efectos adversos , Arritmias Cardíacas/tratamiento farmacológico , Arritmias Cardíacas/inducido químicamente , Fibrilación Atrial/tratamiento farmacológico , Insuficiencia Cardíaca/tratamiento farmacológico , Taquicardia Ventricular/tratamiento farmacológico , Interacciones Farmacológicas , Taquicardia Supraventricular/tratamiento farmacológicoRESUMEN
Nonsustained ventricular tachycardia (NSVT) is a common arrhythmia associated with heart failure, cardiomyopathy, coronary artery disease, electrolyte imbalances, and congenital heart disorders (Foth et al., 2023). NSVT is often asymptomatic depending on its burden percentage. However, typical NSVT presentation in the emergency department (ED) includes palpitations, near-syncope, dizziness, skipped beats, chest pain, and/or dyspnea (Katritsis et al., 2012). In some instances, NSVT can present with elevated or slightly elevated troponin from demand ischemia. A definite diagnosis of NSVT is not of high complexity; nevertheless, it is not always identified on electrocardiogram (ECG) by the time the patient arrives to the ED. Identification of NSVT usually requires prolonged cardiac monitoring, mobile cardiac telemetry (MCT), and in some instances internal loop recorder placement. The purpose of this case is to discuss the typical presentation and pharmacological approach of patients with stable NSVT.
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Electrocardiografía , Taquicardia Ventricular , Humanos , Antiarrítmicos/uso terapéutico , Servicio de Urgencia en Hospital , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Ventricular/diagnósticoAsunto(s)
Benzazepinas , Ivabradina , Ivabradina/uso terapéutico , Ivabradina/farmacología , Humanos , Benzazepinas/uso terapéutico , Benzazepinas/farmacología , Arritmias Cardíacas/tratamiento farmacológico , Antiarrítmicos/uso terapéutico , Antiarrítmicos/farmacología , Taquicardia Ventricular/tratamiento farmacológicoRESUMEN
BACKGROUND: Dexmedetomidine (DEX), a highly selective α2-adrenoceptor agonist, can decrease the incidence of arrhythmias, such as catecholaminergic polymorphic ventricular tachycardia (CPVT). However, the underlying mechanisms by which DEX affects cardiac electrophysiological function remain unclear. METHODS: Ryanodine receptor (RyR2) heterozygous R2474S mice were used as a model for CPVT. WT and RyR2R2474S/+ mice were treated with isoproterenol (ISO) and DEX, and electrocardiograms were continuously monitored during both in vivo and ex vivo experiments. Dual-dye optical mapping was used to explore the anti-arrhythmic mechanism of DEX. RESULTS: DEX significantly reduced the occurrence and duration of ISO-induced of VT/VF in RyR2R2474S/+ mice in vivo and ex vivo. DEX remarkably prolonged action potential duration (APD80) and calcium transient duration (CaTD80) in both RyR2R2474S/+ and WT hearts, whereas it reduced APD heterogeneity and CaT alternans in RyR2R2474S/+ hearts. DEX inhibited ectopy and reentry formation, and stabilized voltage-calcium latency. CONCLUSION: DEX exhibited an antiarrhythmic effect through stabilizing membrane voltage and intracellular Ca2+. DEX can be used as a beneficial perioperative anesthetic for patients with CPVT or other tachy-arrhythmias.
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Arritmias Cardíacas , Calcio , Dexmedetomidina , Canal Liberador de Calcio Receptor de Rianodina , Animales , Dexmedetomidina/farmacología , Canal Liberador de Calcio Receptor de Rianodina/metabolismo , Canal Liberador de Calcio Receptor de Rianodina/genética , Calcio/metabolismo , Ratones , Arritmias Cardíacas/metabolismo , Arritmias Cardíacas/tratamiento farmacológico , Arritmias Cardíacas/genética , Potenciales de la Membrana/efectos de los fármacos , Isoproterenol/farmacología , Taquicardia Ventricular/metabolismo , Taquicardia Ventricular/genética , Taquicardia Ventricular/tratamiento farmacológico , Antiarrítmicos/farmacología , Masculino , Potenciales de Acción/efectos de los fármacos , Ratones Endogámicos C57BLRESUMEN
Ryanodine receptor 2 (RyR2) is a large Ca2+-release channel in the sarcoplasmic reticulum (SR) of cardiac muscle cells. It serves to release Ca2+ from the SR into the cytosol to initiate muscle contraction. RyR2 overactivation is associated with arrhythmogenic cardiac disease, but few specific inhibitors have been reported so far. Here, we identified an RyR2-selective inhibitor 1 from the chemical compound library and synthesized it from glycolic acid. Synthesis of various derivatives to investigate the structure-activity relationship of each substructure afforded another two RyR2-selective inhibitors 6 and 7, among which 6 was the most potent. Notably, compound 6 also inhibited Ca2+ release in cells expressing the RyR2 mutants R2474S, R4497C and K4750Q, which are associated with cardiac arrhythmias such as catecholaminergic polymorphic ventricular tachycardia (CPVT). This inhibitor is expected to be a useful tool for research on the structure and dynamics of RyR2, as well as a lead compound for the development of drug candidates to treat RyR2-related cardiac disease.
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Bloqueadores de los Canales de Calcio , Canal Liberador de Calcio Receptor de Rianodina , Humanos , Calcio/metabolismo , Relación Dosis-Respuesta a Droga , Descubrimiento de Drogas , Células HEK293 , Estructura Molecular , Canal Liberador de Calcio Receptor de Rianodina/efectos de los fármacos , Canal Liberador de Calcio Receptor de Rianodina/genética , Canal Liberador de Calcio Receptor de Rianodina/metabolismo , Relación Estructura-Actividad , Bloqueadores de los Canales de Calcio/química , Bloqueadores de los Canales de Calcio/farmacología , Antiarrítmicos/química , Antiarrítmicos/farmacología , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Ventricular/genéticaAsunto(s)
Anticoagulantes , Ablación por Catéter , Inhibidores de Agregación Plaquetaria , Taquicardia Ventricular , Humanos , Taquicardia Ventricular/cirugía , Taquicardia Ventricular/tratamiento farmacológico , Anticoagulantes/uso terapéutico , Anticoagulantes/efectos adversos , Inhibidores de Agregación Plaquetaria/uso terapéutico , Inhibidores de Agregación Plaquetaria/efectos adversos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Coagulación Sanguínea/efectos de los fármacosRESUMEN
BACKGROUND: Sacubitril/valsartan (Sac/Val) is superior to angiotensin-converting enzyme inhibitors in reducing the risk of heart failure hospitalization and cardiovascular death, but its mechanistic data on myocardial scar after myocardial infarction (MI) are lacking. The objective of this work was to assess the effects of Sac/Val on inflammation, fibrosis, electrophysiological properties, and ventricular tachycardia inducibility in post-MI scar remodeling in swine. METHODS: After MI, 22 pigs were randomized to receive ß-blocker (BB; control, n=8) or BB+Sac/Val (Sac/Val, n=9). The systemic immune response was monitored. Cardiac magnetic resonance data were acquired at 2-day and 29-day post MI to assess ventricular remodeling. Programmed electrical stimulation and high-density mapping were performed at 30-day post MI to assess ventricular tachycardia inducibility. Myocardial samples were collected for histological analysis. RESULTS: Compared with BB, BB+Sac/Val reduced acute circulating leukocytes (P=0.009) and interleukin-12 levels (P=0.024) at 2-day post MI, decreased C-C chemokine receptor type 2 expression in monocytes (P=0.047) at 15-day post MI, and reduced scar mass (P=0.046) and border zone mass (P=0.043). It also lowered the number and mass of border zone corridors (P=0.009 and P=0.026, respectively), scar collagen I content (P=0.049), and collagen I/III ratio (P=0.040). Sac/Val reduced ventricular tachycardia inducibility (P=0.034) and the number of deceleration zones (P=0.016). CONCLUSIONS: After MI, compared with BB, BB+Sac/Val was associated with reduced acute systemic inflammatory markers, reduced total scar and border zone mass on late gadolinium-enhanced magnetic resonance imaging, and lower ventricular tachycardia inducibility.
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Aminobutiratos , Compuestos de Bifenilo , Cicatriz , Modelos Animales de Enfermedad , Combinación de Medicamentos , Infarto del Miocardio , Miocardio , Taquicardia Ventricular , Valsartán , Remodelación Ventricular , Animales , Valsartán/farmacología , Aminobutiratos/farmacología , Infarto del Miocardio/tratamiento farmacológico , Infarto del Miocardio/fisiopatología , Infarto del Miocardio/complicaciones , Infarto del Miocardio/patología , Cicatriz/fisiopatología , Cicatriz/etiología , Cicatriz/patología , Taquicardia Ventricular/fisiopatología , Taquicardia Ventricular/etiología , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Ventricular/prevención & control , Taquicardia Ventricular/metabolismo , Remodelación Ventricular/efectos de los fármacos , Compuestos de Bifenilo/farmacología , Miocardio/patología , Miocardio/metabolismo , Antiinflamatorios/farmacología , Tetrazoles/farmacología , Fibrosis , Porcinos , Antiarrítmicos/farmacología , Femenino , Masculino , Factores de Tiempo , Imagen por Resonancia Cinemagnética , Frecuencia Cardíaca/efectos de los fármacosRESUMEN
In this case report, we describe a 14-year-old patient with a novel RyR2 gene mutation (c.6577G > T/p.Val2193Leu), identified through a comprehensive review of medical history, examination findings, and follow-up data. The pathogenic potential of this mutation, which results in the loss of some interatomic forces and compromises the closure of the RyR2 protein pore leading to calcium leakage, was analyzed using the I-TASSER Suite to predict the structural changes in the protein. This mutation manifested clinically as co-morbid catecholaminergic polymorphic ventricular tachycardia (CPVT) and benign epilepsy with centrotemporal spikes (BECTS), a combination not previously documented in the same patient. While seizures were successfully managed with levetiracetam, the patient's exercise-induced syncope episodes could not be controlled with metoprolol, highlighting the complexity and challenge in managing CPVT associated with this novel RyR2 variation.
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Mutación , Canal Liberador de Calcio Receptor de Rianodina , Taquicardia Ventricular , Humanos , Canal Liberador de Calcio Receptor de Rianodina/genética , Taquicardia Ventricular/genética , Taquicardia Ventricular/tratamiento farmacológico , Adolescente , Masculino , Epilepsia Rolándica/genética , Epilepsia Rolándica/tratamiento farmacológico , ElectrocardiografíaRESUMEN
INTRODUCTION/OBJECTIVE: Studies on the use of amiodarone or sotalol are limited in dogs. Therefore, this study aimed to provide data on the efficacy and safety of these drugs in dogs with ventricular tachyarrhythmia (VT) and/or supraventricular tachyarrhythmia (SvT). ANIMALS, MATERIALS, AND METHODS: Dogs with VT and/or SvT treated with amiodarone or sotalol as a first-line therapy were retrospectively evaluated. Signalment, clinical, diagnostic, therapeutic, and outcome data were retrieved. For VT, efficacy was demonstrated through a decrease of the Lown-Wolf grade to less than five or a reduction of at least 85% in the number of ventricular premature complexes observed on Holter monitoring. For SvT, efficacy was represented by cardioversion or a reduction in the mean heart rate on Holter monitoring ≤140 beats/min. Treatment-related side effects (TRSEs) were classified as clinically relevant and irrelevant. Statistical analysis was performed to compare data before and after antiarrhythmic prescription. RESULTS: Sixty-four dogs were included. Amiodarone and sotalol were efficacious in treating both VT (85.7% and 90.0% of cases, respectively) and SvT (75% and 71.4% of cases, respectively). No significant differences were found when comparing their efficacy rates in dogs with VT and SvT (P=0.531 and 0.483, respectively). Clinically relevant TRSEs were rare with both amiodarone and sotalol (8.3% and 5% of cases, respectively), while clinically irrelevant TRSEs occurred more frequently with amiodarone (29.2%) than with sotalol (10%). DISCUSSION: In dogs with tachyarrhythmias, amiodarone and sotalol are generally efficacious and safe, as clinically relevant TRSEs seem rare. CONCLUSIONS: This study provides novel data on the effects of amiodarone and sotalol in dogs with tachyarrhythmias.
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Amiodarona , Antiarrítmicos , Enfermedades de los Perros , Sotalol , Animales , Perros , Sotalol/uso terapéutico , Amiodarona/uso terapéutico , Antiarrítmicos/uso terapéutico , Enfermedades de los Perros/tratamiento farmacológico , Estudios Retrospectivos , Masculino , Femenino , Resultado del Tratamiento , Taquicardia Ventricular/veterinaria , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Supraventricular/veterinaria , Taquicardia Supraventricular/tratamiento farmacológicoRESUMEN
BACKGROUND: The VICTORIA trial demonstrated a significant decrease in cardiovascular events through vericiguat therapy. This study aimed to assess the potential mechanisms responsible for the reduction of cardiovascular events with vericiguat therapy in a rabbit model of myocardial infarction (MI). METHODS: A chronic MI rabbit model was created through coronary artery ligation. Following 4 weeks, the hearts were harvested and Langendorff perfused. Subsequently, electrophysiological examinations and dual voltage-calcium optical mapping studies were conducted at baseline and after administration of vericiguat at a dose of 5 µmol/L. RESULTS: Acute vericiguat therapy demonstrated a significant reduction in premature ventricular beat burden and effectively suppressed ventricular arrhythmic inducibility. The electrophysiological influences of vericiguat therapy included an increased ventricular effective refractory period, prolonged action potential duration, and accelerated intracellular calcium (Cai) homeostasis, leading to the suppression of action potential and Cai alternans. The pacing-induced ventricular arrhythmias exhibited a reentrant pattern, attributed to fixed or functional conduction block in the peri-infarct zone. Vericiguat therapy effectively mitigated the formation of cardiac alternans as well as the development of reentrant impulses, providing additional anti-arrhythmic benefits. CONCLUSIONS: In the MI rabbit model, vericiguat therapy demonstrates anti-ventricular arrhythmia effects. The vericiguat therapy reduces ventricular ectopic beats, inhibiting the initiation of ventricular arrhythmias. Furthermore, the therapy successfully suppresses cardiac alternans, preventing conduction block and, consequently, the formation of reentry circuits.
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Compuestos Heterocíclicos con 2 Anillos , Infarto del Miocardio , Pirimidinas , Taquicardia Ventricular , Animales , Conejos , Fibrilación Ventricular , Calcio/uso terapéutico , Infarto del Miocardio/complicaciones , Infarto del Miocardio/tratamiento farmacológico , Arritmias Cardíacas/tratamiento farmacológico , Antiarrítmicos/uso terapéutico , Bloqueo Cardíaco , Taquicardia Ventricular/tratamiento farmacológicoAsunto(s)
Benzoatos , Bloqueadores de los Canales de Calcio , Taquicardia Ventricular , Benzoatos/administración & dosificación , Benzoatos/efectos adversos , Bloqueadores de los Canales de Calcio/administración & dosificación , Bloqueadores de los Canales de Calcio/efectos adversos , Rociadores Nasales , Recurrencia , Taquicardia Ventricular/tratamiento farmacológico , Humanos , Masculino , Femenino , AutoadministraciónRESUMEN
Despite impressive developments in the field of ventricular arrhythmias, there is still a relevant number of patients with ventricular arrhythmias who require antiarrhythmic drug therapy and may, e.g., in otherwise drug and/or ablation refractory situations, benefit from agents known for decades, such as mexiletine. Through its capability of blocking fast sodium channels in cardiomyocytes, it has played a minor to moderate antiarrhythmic role throughout the recent decades. Nevertheless, certain patients with structural heart disease suffering from drug-refractory, i.e., mainly amiodarone refractory ventricular arrhythmias, as well as those with selected forms of congenital long QT syndrome (LQTS) may nowadays still benefit from mexiletine. Here, we outline mexiletine's cellular and clinical electrophysiological properties. In addition, the application of mexiletine may be accompanied by various potential side effects, e.g., nausea and tremor, and is limited by several drug-drug interactions. Thus, we shed light on the current therapeutic role of mexiletine for therapy of ventricular arrhythmias and discuss clinically relevant aspects of its indications based on current evidence.
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Antiarrítmicos , Mexiletine , Mexiletine/uso terapéutico , Humanos , Antiarrítmicos/uso terapéutico , Taquicardia Ventricular/tratamiento farmacológico , Taquicardia Ventricular/fisiopatología , Resultado del TratamientoRESUMEN
Smartwatches provide health tracking in various ways and there has been a recent rise in reporting cardiac arrhythmias. While original studies focused on atrial fibrillation, fewer reports have been made on other arrhythmias especially in pregnancy. We report a pregnant patient who presented at 34 weeks' gestation with palpitations. An ECG recorded through her Apple Watch showed ventricular tachycardia. Hospital ECG confirmed monomorphic ventricular tachycardia likely caused by increased sympathetic tone from the gravid state. She was admitted to the cardiac intensive care unit for close monitoring with intravenous anti-arrhythmic agents; however, the rhythm persisted. She underwent a caesarean delivery and the arrhythmia resolved post partum. She later underwent a catheter ablation, after which she discontinued all anti-arrhythmic medications with no recurrence. This case highlights the importance of requesting relevant digital health information, if available, from patients in our modern era. Controlled clinical studies are needed to validate such practices.
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Fibrilación Atrial , Ablación por Catéter , Taquicardia Ventricular , Femenino , Embarazo , Humanos , Antiarrítmicos/uso terapéutico , Electrocardiografía , Taquicardia Ventricular/tratamiento farmacológico , Fibrilación Atrial/tratamiento farmacológico , Estimulación Cardíaca ArtificialRESUMEN
AIMS: In patients with catecholaminergic polymorphic ventricular tachycardia (CPVT), a rare inherited arrhythmia syndrome, arrhythmic events can be prevented by medication and lifestyle recommendations. In patients who experience breakthrough arrhythmic events, non-adherence plays an essential role. We aimed to investigate the incidence and potential reasons for non-adherence to medication and lifestyle recommendations in a large, international cohort of patients with CPVT. METHODS AND RESULTS: An online multilingual survey was shared with CPVT patients worldwide by their cardiologists, through peer-recruitment, and on social media from November 2022 until July 2023. Self-reported non-adherence was measured using the validated Medication Adherence Rating Scale (MARS) and a newly developed questionnaire about lifestyle. Additionally, validated questionnaires were used to assess potential reasons for medication non-adherence. Two-hundred-and-eighteen patients completed the survey, of whom 200 (92%) were prescribed medication [122 (61%) female; median age 33.5 years (interquartile range: 22-50)]. One-hundred-and-three (52%) were prescribed beta-blocker and flecainide, 85 (43%) beta-blocker, and 11 (6%) flecainide. Thirty-four (17%) patients experienced a syncope, aborted cardiac arrest or appropriate implantable cardioverter defibrillator shock after diagnosis. Nineteen (13.4%) patients were exercising more than recommended. Thirty (15%) patients were non-adherent to medication. Female sex [odds ratio (OR) 3.7, 95% confidence interval (CI) 1.3-12.0, P = 0.019], flecainide monotherapy compared to combination therapy (OR 6.8, 95% CI 1.6-31.0, P = 0.010), and a higher agreement with statements regarding concerns about CPVT medication (OR 1.2, 95% CI 1.1-1.3, P < 0.001) were independently associated with non-adherence. CONCLUSION: The significant rate of non-adherence associated with concerns regarding CPVT-related medication, emphasizes the potential for improving therapy adherence by targeted patient education.