Infant Case with a Malignant Form of Brugada Syndrome

Sano, Hiroshi; Torigoe, Katsumi; Numata, Osamu; Yazaki, Satoshi

doi:10.1046/j.1540-8167.2000.01277.x

Cited by 102 publications

(56 citation statements)

References 17 publications

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“…During electrical storm associated with repetitive episodes of VF, continuous infusion of isoproterenol, a β-adrenergic agonist (0.005 − 0.02 μg/kg/min or 20 % increase of heart rate), is the first choice of therapy (Fig. 8) [97]. Injections of atropine may be another choice, although the effect is short-lived.…”

Section: Specific Therapy Based On Cellular Mechanismsmentioning

confidence: 99%

Specific Therapy Based on the Genotype and Cellular Mechanism in Inherited Cardiac Arrhythmias. Long QT Syndrome and Brugada Syndrome

Shimizu

Aiba²,

Antzelevitch

2005

CPD

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Seven forms of congenital long QT syndrome (LQTS) caused by mutations in ion channel genes have been identified. Genotype-phenotype correlation in clinical and experimental studies involving arterially-perfused canine left ventricular wedges suggest that ,β-blockers are protective in LQT1, less so in LQT2, but not protective in LQT3. A class IB sodium channel blocker, mexiletine, is most effective in abbreviating QT interval in LQT3, but effectively reduces transmural dispersion of repolarization (TDR) and prevents the development of Torsade de Pointes (TdP) in all 3 models, suggesting its potential as an adjunctive therapy in LQT1 and LQT2. High concentrations of intravenous nicorandil, a potassium channel opener, have been shown to be capable of decreasing QT and TDR, and preventing TdP in LQT1 and LQT2 but not in LQT3. The calcium channel blocker, verapamil, has also been suggested as adjunctive therapy for LQT1, LQT2 and possibly LQT3.Experimental data using right ventricular wedge preparations suggest that a prominent transient outward current (I to )-mediated action potential (AP) notch and a loss of AP dome in epicardium, but not in endocardium, give rise to a transmural voltage gradient, resulting in ST segment elevation and the induction of ventricular fibrillation (VF), characteristics of the Brugada syndrome. Since the maintenance of the AP dome is determined by the balance of currents active at the end of phase 1 of the AP, any intervention that reduces the outward current or boosts inward current at the end of phase I may normalize the ST segment elevation and suppress VF. Such interventions are candidates for pharmacological therapy of the Brugada syndrome. The infusion of isoproterenol, a β-adrenergic stimulant, strongly augments L-type calcium current (I Ca-L ), and is the first choice for suppressing electrical storms associated with Brugada syndrome. Quinidine, by virtue of its actions to block I to , has been proposed as adjunctive therapy, with an implantable cardioverter defibrillator as backup. Oral denopamine, atropine or cilostazol all increase I Ca-L , and for this reason may be effective in reducing episodes of VF.

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Section: Specific Therapy Based On Cellular Mechanismsmentioning

confidence: 99%

Specific Therapy Based on the Genotype and Cellular Mechanism in Inherited Cardiac Arrhythmias. Long QT Syndrome and Brugada Syndrome

Shimizu

Aiba²,

Antzelevitch

2005

CPD

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“…Typical ECG patterns, however, have been observed in small infants, where eventual lethal arrhythmias might actually resemble Sudden Infant Death Syndrome. 5,14 Hence, suspect symptoms with typical electrocardiographic features and/or a family history for sudden cardiac death, even at young age, should alert pediatricians to the possibility of Brugada syndrome.…”

Section: Electrocardiographic Characteristicsmentioning

confidence: 99%

“…Serious ventricular arrhythmias, including ventricular fibrillation (VF), may occur during the test. Immediate discontinuation of the drug is required, and isoproterenol infusion might be needed to treat the arrhythmias (1 to 3 g/min isoproterenol 14 ; P. Brugada, personal observations, 1999).…”

Section: Drug Challengementioning

confidence: 99%

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Proposed Diagnostic Criteria for the Brugada Syndrome

Wilde

Antzelevitch

Borggrefe

et al. 2002

European Heart Journal

307

122

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“…Isoproterenol, sometimes in combination with quinidine, has been shown to be effective in normalizing ST segment elevation in patients with the Brugada syndrome and in controling electrical storms, particularly in children (Alings et al 2001;Shimizu et al 2000b;Suzuki et al 2000;Tanaka et al 2001;Belhassen et al 2002;Mok et al 2004).…”

Section: Pharmacologic Approach To Therapymentioning

confidence: 99%

Therapy for the Brugada Syndrome

Antzelevitch

Fish

Basis and Treatment of Cardiac Arrhythmias

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The Brugada syndrome is a congenital syndrome of sudden cardiac death first described as a new clinical entity in 1992. Electrocardiographically characterized by a 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 and otherwise healthy adults, and less frequently in infants and children. The ECG manifestations of the Brugada syndrome are often dynamic or concealed and maybe revealed or modulated by sodium channel blockers. The syndrome may also be unmasked or precipitated by a febrile state, vagotonic agents, α-adrenergic agonists, β-adrenergic blockers, tricyclic ortetracyclic antidepressants, a combination of glucose and insulin, and hypokalemia, as well as by alcohol and cocaine toxicity. An implantable cardioverter-defibrillator (ICD) is the most widely accepted approach to therapy. Pharmacological therapy aimed at rebalancing the currents active during phase 1 of the right ventricular action potential is used to abort electrical storms, as an adjunct to device therapy, and as an alternative to device therapy when use of an ICD is not possible. Isoproterenol and cilostazol boost calcium channel current, and drugs like quinidine inhibit the transient outward current, acting to diminish the action potential notch and thus suppress the substrate and trigger for ventricular tachycardia/fibrillation (VT/VF). KeywordsBrugada syndrome; Phase 2 reentry; ST segment elevation; I Na ; I to ; Implantable cardioverterdefibrillator (ICD); VT; SCN5A mutations; Sudden death; Bradycardia Clinical Characteristics and Diagnostic CriteriaThe Brugada syndrome typically manifests in the third or fourth decade of life (average age of 41±15 years), although patients have been diagnosed with the syndrome at an age as young as 2 days and as old as 84 years. The prevalence of the disease is estimated to be at least 5 per 10,000 inhabitants in Southeast Asia, where the syndrome is endemic (Nademanee et al. 1997). In Japan, a Brugada syndrome ECG (type 1) is observed in 12 per 10,000 inhabitants; type 2 and type 3 ECGs, which are not diagnostic of Brugada syndrome, are much more prevalent, appearing in 58 per 10,000 inhabitants (Miyasaka et al. 2001). The true prevalence of the disease in the general population is difficult to estimate because the ECG pattern is often concealed (Brugada et al. 2003). Sudden unexplained nocturnal death syndrome (SUNDS also known as SUDS) and Brugada syndrome have been shown to be phenotypically, genetically, and functionally the same disorder (Vatta et al. 2002).Although syncope and sudden death are a consequence of ventricular tachycardia/fibrillation (VT/VF), approximately 20% of Brugada syndrome patients also develop supraventricular arrhythmias (Morita et al. 2002). Atrial fibrillation (AF) is reported in approximately 10%-20% of cases. Atrio-ventricular (AV) nodal reentrant tachycardia (AVNRT) and Wolf-ParkinsonWhite (WPW) syndrome have been described as well (Eckardt et al. 2001 et al. 2004)...

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Infant Case with a Malignant Form of Brugada Syndrome

Cited by 102 publications

References 17 publications

Specific Therapy Based on the Genotype and Cellular Mechanism in Inherited Cardiac Arrhythmias. Long QT Syndrome and Brugada Syndrome

Specific Therapy Based on the Genotype and Cellular Mechanism in Inherited Cardiac Arrhythmias. Long QT Syndrome and Brugada Syndrome

Proposed Diagnostic Criteria for the Brugada Syndrome

Therapy for the Brugada Syndrome

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