Implantable cardioverter-defibrillator in patients with inherited arrhythmia syndromes: A systematic review

Medeiros, Paulo de Tarso Jorge; Santos, Manuel Lopes dos; Arantes, Carina; Pereira, Vítor Hugo; Rocha, Sérgia

doi:10.1016/j.hrtlng.2023.02.012

Cited by 3 publications

(2 citation statements)

References 48 publications

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“…In recent years, subcutaneous implantable cardioverter-defibrillators (S-ICDs) have emerged as a promising alternative for managing inherited arrhythmias. However, existing studies have primarily focused on patients with long QT syndrome (LQTS) and Brugada syndrome, with limited investigation into their application in CPVT [ 131 , 132 ]. Initial concerns persist regarding the use of S-ICDs in inherited arrhythmia populations, particularly related to the challenges in programming variables such as time to therapy and time to redetection [ 125 ].…”

Section: Therapeutic Strategiesmentioning

confidence: 99%

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

Aggarwal,

Stolear,

Alam

et al. 2024

JCM

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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.

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Section: Therapeutic Strategiesmentioning

confidence: 99%

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

Aggarwal,

Stolear,

Alam

et al. 2024

JCM

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show abstract

“…In recent years, multiple studies addressed the issue of appropriate stratification of the risk of delivering either appropriate or inappropriate therapies in patients with IAS, including those with BrS, and their results varied significantly, due to differences in programming and baseline arrhythmic risks. 5–8 …”

mentioning

confidence: 99%

Appropriate and inappropriate therapies in patients with Brugada syndrome. What to expect? Authors’ reply

Dyrbuś,

Gąsior,

Tajstra

2023

Europace

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Differences in underlying cardiac substrate among S-ICD recipients and its impact on long-term device-related outcomes: Real-world insights from the iSUSI registry

Gasperetti,

Schiavone,

Milstein

et al. 2024

Heart Rhythm

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Implantable cardioverter-defibrillator in patients with inherited arrhythmia syndromes: A systematic review

Cited by 3 publications

References 48 publications

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

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

Appropriate and inappropriate therapies in patients with Brugada syndrome. What to expect? Authors’ reply

Differences in underlying cardiac substrate among S-ICD recipients and its impact on long-term device-related outcomes: Real-world insights from the iSUSI registry

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