Intravenous drug challenge using flecainide and ajmaline in patients with Brugada syndrome

Wolpert, Christian; Echternach, Constanze; Veltmann, Christian; Antzelevitch, Charles; Thomas, George P.; Spehl, Susanne; Streitner, Florian; Kuschyk, Juergen; Schimpf, Rainer; Haase, Karl K.; Borggrefe, Martin

doi:10.1016/j.hrthm.2004.11.025

Cited by 170 publications

(110 citation statements)

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“…Intravenous administration of class IC antiarrhythmic agents, including ajmaline, flecainide, or procainamide, which act as sodium channel blockers, are able to elicit the coved-type ECG diagnostic pattern of BrS. 13 An unequal response to intravenous administration of flecainide and ajmaline has been reported in BrS patients, 14 suggesting that ajmaline is better at unmasking the ECG pattern. This was also observed in families with BrS-associated pathogenic variations in SCN5A, with an ability to detect 80% of genetic carriers, with the most powerful drug, ajmaline.…”

Section: Diagnostic Toolsmentioning

confidence: 99%

Brugada syndrome: clinical and genetic findings

Sarquella-Brugada

Campuzano

Arbelo

et al. 2016

Genetics in Medicine

114

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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: Diagnostic Toolsmentioning

confidence: 99%

Brugada syndrome: clinical and genetic findings

Sarquella-Brugada

Campuzano

Arbelo

et al. 2016

Genetics in Medicine

114

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“…), which exhibit less use-dependent block of fast INa due to faster dissociation of the drug for the sodium channels, show a weaker ST segment elevation than class IC drugs (30). Ajmaline has recently been reported to produce typical Brugada ECG more frequently than flecainide (33). Pilsicainide, a pure class IC drug developed in Japan, also more strongly induce ST segment elevation than flecainide ( Fig.…”

Section: Drug Challengementioning

confidence: 98%

The Brugada Syndrome-An Update-

Shimizu

2005

Intern. Med.

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“…18,74 This pattern can be concealed and often dynamic, unmasked by factors such as sleep 75 and pyrexia, 76 as well as provoked by sodium channel blocking drugs such as ajmaline, procainamide, and flecainide. 77 It can manifest with syncope because of polymorphic ventricular tachycardia, ultimately predisposing to ventricular fibrillation and SCD, which is commonly the first presentation. 78 The prevalence of a BrS type 1 ECG is higher in Asian countries, approximately 0.15% in adults.…”

Section: Causes Of Sads and The Role Of Genetic Testingmentioning

confidence: 99%