Implantable Defibrillators in Long QT Syndrome, Brugada Syndrome, Hypertrophic Cardiomyopathy, and Arrhythmogenic Right Ventricular Cardiomyopathy

Dohadwala, Mustafa; Link, Mark S.

doi:10.1016/j.ccl.2013.11.003

Cited by 4 publications

(4 citation statements)

References 114 publications

(117 reference statements)

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“…21 Medications have not been found to be greatly beneficial in BrS, although quinidine may be recommended in patients with a history of arrhythmic storm. 26 The only proven therapeutic intervention to prevent SCD in BrS is the placement of an ICD; however, every patient with BrS does not necessarily require a primary preventative ICD. Patients at higher risk for SCD include survivors of ventricular fibrillation/SCD; those who have a history of syncopal episodes with spontaneous type I Brugada ECG pattern at baseline; those who are male; and those who have a history of spontaneous atrial fibrillation.…”

Section: Brugada Syndromementioning

confidence: 99%

“…Specific channelopathies may also have unique goals in programming: for example, permanent pacing can be considered in LQTS to decrease bradycardia-dependent QT prolongation. 26 Inappropriate shocks are unfortunately common in pediatric patients with primary ion channelopathies. One study found that, in a group of 76 patients aged younger than 30 years of whom 33% had primary electrical disease, 19 patients (25%) received inappropriate therapy.…”

Section: Unique Considerations For Implantable Cardioverter-defibrillmentioning

confidence: 99%

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Assessing Candidacy for Primary Preventative Implantable Cardioverter-defibrillators in Pediatric Patients with Ion Channelopathies: Weighing the Risks and Benefits

Townsend

Aziz

2018

J Innov Cardiac Rhythm Manage.

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Inherited ion channelopathies have come to the forefront as a significant cause of sudden cardiac death (SCD) in pediatric patients with structurally normal hearts. Implantable cardioverter-defibrillator (ICD) placement can be a life-saving primary preventative therapy, but because of actors inherent in the pediatric population, careful thought must be given to the specific indications for placement in each patient. The most common inherited ion channelopathies are long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. All have the potential to cause SCD. However, thanks to current research, more is now known about the range of phenotypes present within each disorder and also the benefits that medical therapy can provide. Risk stratification can allow clinicians to best predict which patients may most benefit from a primary preventative ICD while at the same time avoid placement in the larger group who may remain asymptomatic with the aid of medical therapy or even simply observation.

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Section: Brugada Syndromementioning

confidence: 99%

Section: Unique Considerations For Implantable Cardioverter-defibrillmentioning

confidence: 99%

Assessing Candidacy for Primary Preventative Implantable Cardioverter-defibrillators in Pediatric Patients with Ion Channelopathies: Weighing the Risks and Benefits

Townsend

Aziz

2018

J Innov Cardiac Rhythm Manage.

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“…Diagnosis of LQTS is done by clinical symptoms in conjunction with EKG assessment and is best done using the Schwartz Diagnostic Criteria for LQTS, which is highlighted in In treating this arrhythmia syndrome, the mainstay of medical management is supported by β-blocker therapy [1,2,6]. Additional treatment modalities include medical via mexiletine, ranolazine, and/or flecainide, as well as procedural, via left cardiac sympathetic denervation and implantable cardiac defibrillator (ICD) [11][12][13]. The increased late sodium currents (INa-L) in LQTS and LQTS 3 can result in bradycardia-dependent QT prolongation, given that mexiletine is an inhibitor of INa-L [14,15].…”

Section: Introductionmentioning

confidence: 99%

“…ICDs have long been the mainstay of management of severe LQTS regardless of subtype. Prior to recent times, ICDs had been the main treatment modality for LQTS 3, but other treatment options are still proposed [ 11 ].…”

Section: Introductionmentioning

confidence: 99%

A Systematic Review on the Role of Βeta-Blockers in Reducing Cardiac Arrhythmias in Long QT Syndrome Subtypes 1-3

Went¹,

Sultan²,

Sapkota³

et al. 2021

Cureus

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Long QT syndrome (LQTS) is one of the most common inherited cardiac channelopathies with a prevalence of 1:2000. The condition can be congenital or acquired with 15 recognized genotypes; the most common subtypes are LQTS 1, 2, and 3 making up to 85%-90% of the cases. LQTS is characterized by delayed ventricular cardiomyocyte repolarization manifesting on the surface electrocardiogram (EKG) by a prolonged corrected QT (QTc) interval. The mainstay of treatment for this condition involves in part or combination medical therapy via β-blockers as first-line (or other anti-arrhythmic), left cardiac sympathectomy, or implantable cardiac defibrillator placement. Given the high rate of adverse cardiac events (ACE) or sudden cardiac death (SCD) in this population of patients with this disease, this review seeks to highlight the genotype-specific treatment consensus in β-blocker therapy of the most common subtypes.A database search of PubMed, PMC, and Medline was conducted to ascertain the most recent data in the last five years on the management of LQTS types 1-3 and the role of β-blockers in reducing ACE in these types. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were adhered to in the study selection, and selected studies focused on humans, written in the English Language, and within the last five years of LQTS subtypes 1, 2, and 3.Eleven relevant studies were selected after considering inclusion criteria, exclusion criteria, and quality appraisal within the last five years, focusing on β-blocker selection directed based on the subtypes of LQTS. Two meta-analyses, one cohort study, and eight reviews provided significant data that non-selective βblockers unequivocally are of benefit in these LQTS types. Summary of findings suggested nadolol followed by propranolol yields the best results in LQTS 1, while nadolol would yield the best effect in LQTS 2 and 3.

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A case of congenital long QT syndrome, type 8, undergoing laparoscopic hysterectomy with general anesthesia

Chang

Hung

et al. 2019

Taiwanese Journal of Obstetrics and Gynecology

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Implantable Defibrillators in Long QT Syndrome, Brugada Syndrome, Hypertrophic Cardiomyopathy, and Arrhythmogenic Right Ventricular Cardiomyopathy

Cited by 4 publications

References 114 publications

Assessing Candidacy for Primary Preventative Implantable Cardioverter-defibrillators in Pediatric Patients with Ion Channelopathies: Weighing the Risks and Benefits

Assessing Candidacy for Primary Preventative Implantable Cardioverter-defibrillators in Pediatric Patients with Ion Channelopathies: Weighing the Risks and Benefits

A Systematic Review on the Role of Βeta-Blockers in Reducing Cardiac Arrhythmias in Long QT Syndrome Subtypes 1-3

A case of congenital long QT syndrome, type 8, undergoing laparoscopic hysterectomy with general anesthesia

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