Successful acute and long-term management of electrical storm in Brugada syndrome using orciprenaline and quinine/quinidine

Schweizer, Patrick; Becker, Rüdiger; Katus, Hugo A.; Thomas, Dierk

doi:10.1007/s00392-010-0145-7

Cited by 60 publications

(34 citation statements)

References 27 publications

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“…Quinidine is recommended for BrS patients with ICD and multiple shocks when ICD implantation is contraindicated, and for the treatment of supraventricular arrhythmias in the event that other treatments have been contraindicated. 32 Unfortunately, quinidine remains unavailable in regions where BrS and SUNDS are endemic, such as Southeast Asia. 33 Finally, radiofrequency catheter ablation has also been postulated as a therapeutic approach in BrS.…”

Section: Clinical Managementmentioning

confidence: 99%

Brugada syndrome: clinical and genetic findings

Sarquella-Brugada

Campuzano

Arbelo

et al. 2016

Genetics in Medicine

128

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Review ARticleMore than 20 years ago, eight individuals were resuscitated from sudden cardiac death (SCD) caused by documented ventricular fibrillation (VF); all showed a characteristic ST segment elevation in the right precordial leads and a structurally normal heart. 1 In 1996, the term "Brugada syndrome" (BrS) was used to describe what was previously known as "right bundle branch block, persistent ST segment elevation, and sudden death syndrome. " 2 A year later, BrS was reported to be the same disease as sudden unexplained nocturnal death syndrome (SUNDS). In other countries, BrS has different names: Lai Tai (Thailand), Pokkuri (Japan), and Bangungut (Philippines). Currently, the prevalence of BrS is estimated at ~3-5 in 10,000 people, and it is higher in young men of Southeast Asian origin. It is the main cause of death among young healthy men in Southeast Asia. 3 Recent reports suggest that BrS could be responsible for 4% of all SCD and up to 12% of sudden death in patients with structurally normal hearts. The clinical phenotype manifests in adulthood, at a mean age of 41 years, and it is 8-to 10-times more frequent in males. Frequently, sudden death can be the first manifestation of the disease. 4 In 1998, the genetic basis of BrS was established when the sodium channel protein type V subunit α gene (SCN5A), which encodes the α-subunit of the voltage-gated Na v 1.5 cardiac sodium channel responsible for regulating rapid sodium current (I Na ), was associated with the disease. 5 Currently, BrS is recognized as a rare, inherited cardiac channelopathy caused by an alteration of ionic currents that leads to ventricular arrhythmias and SCD. It is clinically characterized by ST segment elevation in leads V1-V3 of the electrocardiogram, but incomplete penetrance and variable expressivity confound the diagnosis. 6 Despite the identification of 18 associated genes, 65-70% of clinically diagnosed cases remain without an identifiable genetic cause. 4 CLINICAL DIAGNOSIS Diagnostic criteriaThe clinical diagnosis of BrS requires the identification of the ST segment elevation in the right precordial leads at baseline or after the use of sodium blockers. Three different electrocardiographic (ECG) patterns can be often observed in families with BrS: type I, which consists of a coved-type ST segment elevation greater than 2 mm followed by a descending negative T wave in at least two right precordial leads (V1 to V3); type II ST elevation, saddleback-shaped patterns with a high initial increase followed by an ST elevation greater than 2 mm; and saddleback-shaped patterns with a high initial increase followed by an ST elevation less than 2 mm (Figure 1). The second Brugada Consensus Report proposed that only type I is diagnostic for BrS 7 and, in 2013, it was proposed that a definitive diagnosis of BrS considers both spontaneous type I pattern and a provoked type I pattern (with a baseline type II or III pattern) in at least one right precordial lead (V1 or V2). 8 The diagnosis of BrS is currently accepted in those patients ...

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Section: Clinical Managementmentioning

confidence: 99%

Brugada syndrome: clinical and genetic findings

Sarquella-Brugada

Campuzano

Arbelo

et al. 2016

Genetics in Medicine

128

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“…Agents that augment the L-type calcium channel current, such as β adrenergic agents like isoproterenol, denopamine or orciprenaline have been shown to be useful 166, 168, 226, 230, 235, 236 . Isoproterenol, sometimes in combination with quinidine, has been utilized successfully to suppress electrical storms and to normalize ST elevation particularly in children 51, 218, 219, 230, 233, 237–24346, 224, 244–247 .…”

Section: Approach To Therapymentioning

confidence: 99%

Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects

Antzelevitch¹,

Patocskai²

2016

Current Problems in Cardiology

103

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The Brugada syndrome (BrS) is an inherited cardiac arrhythmia syndrome first described as a new clinical entity in 1992. Electrocardiographically characterized by distinct coved type ST segment elevation in the right precordial leads, the syndrome is associated with a high risk for sudden cardiac death in young adults, and less frequently in infants and children. The ECG manifestations of the BrS are often concealed and may be unmasked or aggravated by sodium channel blockers, a febrile state, vagotonic agents, as well as by tricyclic and tetracyclic antidepressants. An implantable cardioverter defibrillator (ICD) is the most widely accepted approach to therapy. Pharmacological therapy is designed to produce an inward shift in the balance of currents active during the early phases of the right ventricular action potential and can be used to abort electrical storms or as an adjunct or alternative to device therapy when use of an ICD is not possible. Isoproterenol, cilostazol and milrinone boost calcium channel current and drugs like quinidine, bepridil and the Chinese herb extract Wenxin Keli inhibit the transient outward current, acting to diminish the action potential (AP) notch and thus to suppress the substrate and trigger for VT/VF. Radiofrequency ablation of the right ventricular outflow tract epicardium of BrS patients has recently been shown to reduce arrhythmia-vulnerability and the ECG-manifestation of the disease, presumably by destroying the cells with more prominent AP notch. This review provides an overview of the clinical, genetic, molecular and cellular aspects of the BrS as well as the approach to therapy.

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“…Agents that augment the L-type calcium channel current, such as β adrenergic agents like isoproterenol, denopamine or orciprenaline, are useful as well. 10, 121, 125, 131-133 Isoproterenol, sometimes in combination with quinidine, has been utilized successfully to control VF storms and normalizing ST elevation particularly in children 113, 114, 125, 129, 134-141. 119, 142-146 .…”

Section: Introductionmentioning

confidence: 99%

J-wave syndromes: Brugada and early repolarization syndromes

2015

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A prominent J wave is encountered in a number of life-threatening cardiac arrhythmia syndromes, including the Brugada (BrS) and early repolarization (ERS) syndromes. BrS and ERS differ with respect to the magnitude and lead location of abnormal J waves and are thought to represent a continuous spectrum of phenotypic expression termed J wave syndromes. Despite two decades of intensive research, risk stratification and the approach to therapy of these two inherited cardiac arrhythmia syndromes are still undergoing rapid evolution. Our objective in this review is to provide an integrated synopsis of the clinical characteristics, risk stratifiers, as well as the molecular, ionic, cellular and genetic mechanisms underlying these two fascinating syndromes that have captured the interest and attention of the cardiology community in recent years.

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Successful acute and long-term management of electrical storm in Brugada syndrome using orciprenaline and quinine/quinidine

Cited by 60 publications

References 27 publications

Brugada syndrome: clinical and genetic findings

Brugada syndrome: clinical and genetic findings

Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects

J-wave syndromes: Brugada and early repolarization syndromes

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