A 35-year effective treatment of catecholaminergic polymorphic ventricular tachycardia with propafenone

Marx, Alexandra; Lange, Björn; Nalenz, C.; Hoffmann, Boris; Rostock, Thomas; Konrad, Torsten

doi:10.1016/j.hrcr.2018.04.003

Cited by 7 publications

(3 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Propafenone, another Class 1C anti-arrhythmic drug, has also been used to treat CPVT (Marx et al 2019). Similar to flecainide, propafenone inhibits RyR2 single channels (Hwang et al 2011) and inhibits arrhythmogenic calcium waves in CPVT cardiomyocytes (Savio-Galimberti & Knollmann, 2015).…”

Section: Flecainide and Propafenonementioning

confidence: 99%

Molecular and tissue mechanisms of catecholaminergic polymorphic ventricular tachycardia

Wleklinski

Kannankeril

Knollmann

2020

The Journal of Physiology

View full text Add to dashboard Cite

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a stress-induced cardiac channelopathy that has a high mortality in untreated patients. Our understanding has grown tremendously since CPVT was first described as a clinical syndrome in 1995. It is now established that the deadly arrhythmias are caused by unregulated 'pathological' calcium release from the sarcoplasmic reticulum (SR), the major calcium storage organelle in striated muscle. Important questions remain regarding the molecular mechanisms that are responsible Matthew Wleklinski is an MD/PhD student interested in studying how mutations in calcium handling proteins lead to cardiac arrhythmias and sudden death. His current work focuses on the protein calsequestrin and its role in catecholaminergic polymorphic ventricular tachycardia. Dr

show abstract

Section: Flecainide and Propafenonementioning

confidence: 99%

Molecular and tissue mechanisms of catecholaminergic polymorphic ventricular tachycardia

Wleklinski

Kannankeril

Knollmann

2020

The Journal of Physiology

View full text Add to dashboard Cite

show abstract

“…Propafenone, a class IC sodium channel blocking agent, has seen limited application in the context of CPVT and has not been a primary focus of investigative research. The existing evidence is scant and has been primarily derived from case reports [ 95 , 96 ]. Comprehensive studies are warranted to assess the efficacy of propafenone within this patient population, discerning its potential as an alternative in situations where flecainide is either unavailable or not well-tolerated.…”

Section: Therapeutic Strategiesmentioning

confidence: 99%

Catecholaminergic Polymorphic Ventricular Tachycardia: Clinical Characteristics, Diagnostic Evaluation and Therapeutic Strategies

Aggarwal,

Stolear,

Alam

et al. 2024

JCM

View full text Add to dashboard Cite

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a severe hereditary arrhythmia syndrome predominantly affecting children and young adults. It manifests through bidirectional or polymorphic ventricular arrhythmia, often culminating in syncope triggered by physical exertion or emotional stress which can lead to sudden cardiac death. Most cases stem from mutations in the gene responsible for encoding the cardiac ryanodine receptor (RyR2), or in the Calsequestrin 2 gene (CASQ2), disrupting the handling of calcium ions within the cardiac myocyte sarcoplasmic reticulum. Diagnosing CPVT typically involves unmasking the arrhythmia through exercise stress testing. This diagnosis emerges in the absence of structural heart disease by cardiac imaging and with a normal baseline electrocardiogram. Traditional first-line treatment primarily involves β-blocker therapy, significantly reducing CPVT-associated mortality. Adjunctive therapies such as moderate exercise training, flecainide, left cardiac sympathetic denervation and implantable cardioverter-defibrillators have been utilized with reasonable success. However, the spectrum of options for managing CPVT has expanded over time, demonstrating decreased rates of arrhythmic events. Furthermore, ongoing research into potential new therapies including gene therapies has the potential to further enhance treatment paradigms. This review aims to succinctly encapsulate the contemporary understanding of the clinical characteristics, diagnostic approach, established therapeutic interventions and the promising future directions in managing CPVT.

show abstract

“…Доказательная база эффективности флекаинида непрерывно растет [20,21]. Еще один препарат, снижающий возникновение проаритмогенных кальциевых токов в кардиомиоцитах и потенциально эффективный у больных с КПЖТ, -пропафенон [22,23]. Для того чтобы установить, эффективно ли добавление к терапии препаратов класса IC у больных с мутациями в гене CASQ2 и целе сообразно ли расширение показаний к назначению комбинированной антиаритмической терапии у данной группы больных, необходимо проведение дополнительных исследований.…”

Section: молекулярно-генетические данныеunclassified

<i>CASQ2</i>: clinical and genetic insights into catecholaminergic polymorphic ventricular tachycardia across three families

Kulbachinskaya

Bereznitskaya

2023

Almanac of Clinical Medicine

View full text Add to dashboard Cite

Catecholaminergic polymorphic ventricular tachycardia is a primary channelopathy with a high mortality rate if left untreated. In 3 to 5% of catecholaminergic polymorphic ventricular tachycardia patients, mutations in the CASQ2 gene, either in a homozygous or compound heterozygous form, have been identified. In this article, we present a clinical case series of patients from three unrelated families with mutations in the CASQ2 gene, including three novel mutations (p.Leu167Pro, p.Asp325GlyfsTer7, and p.Glu259Ter). All our patients with homozygous or compound heterozygous CASQ2 gene mutations experienced a severe disease course, with early manifestations and resistance to specific anti-arrhythmic treatment, including beta-blockers. They exhibited a wide range of heart rhythm abnormalities, both ventricular and supraventricular, and had a high risk of sudden cardiac death. In all cases, ventricular heart arrhythmias persisted despite regular treatment with specific anti-arrhythmic agents, unless selective left-sided sympathectomy had been performed. The management of this patient group emphasized an individualized approach, combining medical and surgical treatment methods tailored to each patient's unique needs and condition.

show abstract

A 35-year effective treatment of catecholaminergic polymorphic ventricular tachycardia with propafenone

Cited by 7 publications

References 12 publications

Molecular and tissue mechanisms of catecholaminergic polymorphic ventricular tachycardia

Molecular and tissue mechanisms of catecholaminergic polymorphic ventricular tachycardia

Catecholaminergic Polymorphic Ventricular Tachycardia: Clinical Characteristics, Diagnostic Evaluation and Therapeutic Strategies

<i>CASQ2</i>: clinical and genetic insights into catecholaminergic polymorphic ventricular tachycardia across three families

Contact Info

Product

Resources

About