Brugada syndrome: Difference between revisions
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# Monomorphic VT is observed infrequently | # Monomorphic VT is observed infrequently | ||
# VT/VF often terminates spontaneously in patients with the Brugada syndrome which may explain why patients wake up at night after episodes of agonal respiration caused by the arrhythmia. | # VT/VF often terminates spontaneously in patients with the Brugada syndrome which may explain why patients wake up at night after episodes of agonal respiration caused by the arrhythmia. | ||
==Treatment== | ==Treatment== |
Revision as of 03:15, 31 August 2012
Brugada syndrome | |
ECG findings of Brugada Syndrome | |
ICD-10 | I42.8 |
ICD-9 | 746.89 |
OMIM | 601144 |
DiseasesDB | 31999 |
MeSH | D053840 |
Brugada syndrome Microchapters |
Diagnosis |
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Treatment |
Case Studies |
Brugada syndrome On the Web |
American Roentgen Ray Society Images of Brugada syndrome |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Synonyms and keywords: Sudden unexpected death syndrome; SUDS
Differential Diagnosis
Characteristics
- Characterized by a coved-type ST-segment elevation in the right precordial leads
- The Brugada ECG is often concealed, but can be unmasked or modulated by a number of drugs and pathophysiological states including sodium channel blockers, a febrile state, vagotonic agents, tricyclic antidepressants, as well as cocaine and Propranolol intoxication.
Lithium Treatment and Brugada Syndrome
Administration of Lithium can result in EKG manifestations of the Brugada syndrome. [1][2]. Syncope and sudden cardiac death have been observed in these patients.[3] The putative role of lithium has been suggested in so far as withdrawal of lithium results in either 1) normalization of the ECG or 2) conversion of the Brugada pattern to type 2 or 3. The appearance of Brugada type EKG patterns does not require toxic lithium levels.
Diagnosis
Sodium Challenge
- Drugs that can be used
- Ajmaline 1 mg/kg/5 min IV
- Flecainide 2 mg/kg/10 min IV or 400 mg PO
- Procainamide 10 mg/kg/10 min IV
- Pilsicainide 1 mg/kg/10 min IV
- The sodium challenge should be terminated when
- Diagnostic Type 1 ST-segment elevation or Brugada ECG, develops
- ST segment in Type 2 increases by ≥2 mm
- Premature ventricular beats or other arrhythmias develop
- QRS widens to ≥130% of baseline
Arrhythmias
- Polymorphic VT resembling a rapid Torsade de Pointes (TdP)
- Monomorphic VT is observed infrequently
- VT/VF often terminates spontaneously in patients with the Brugada syndrome which may explain why patients wake up at night after episodes of agonal respiration caused by the arrhythmia.
Treatment
The cause of death in Brugada syndrome is ventricular fibrillation.The episodes of syncope (fainting) and sudden death (aborted or not) are caused by fast polymorphic ventricular tachycardias or ventricular fibrillation. These arrhythmias appear with no warning. While there is no exact treatment modality that reliably and totally prevents ventricular fibrillation from occurring in this syndrome, treatment lies in termination of this lethal arrhythmia before it causes death. This is done via implantation of an implantable cardioverter-defibrillator (ICD), which continuously monitors the heart rhythm and will defibrillate an individual if ventricular fibrillation is noted. Some recently performed studies had evaluated the role of quinidine, a Class Ia antiarrythmic drug, for decreasing VF episodes occurring in this syndrome. Quinidine was found to decrease number of VF episodes and correcting spontaneous ECG changes, possibly via inhibiting Ito channels.[4] Those with risk factors for coronary artery disease may require an angiogram before ICD implantation.
- Aborted sudden death are at high risk for recurrence and should receive an ICD
- VT storm has been successfully treated with Isoproterenol. The mechanism is thought to be augmenting the cardiac L type channel.
- Asymptomatic patients require risk stratification and clinical judegement to help guide therapy
- Quinidine (class IA sodium channel blocker) blocks the Ito current and is proven to suppress spontaneous VF
- Cilostazol (phosphodiesterase III inhibitor that increases inward L type calcium channel current and reported to suppress spontaneous VF
- Bepridil suppress spontaneous VF probably through blocking Ito current
- Medical therapy alone with the above agents is currently not evaluated in randomized trials and should not be used as loan therapy.
See also
References
- ↑ Pirotte MJ, Mueller JG, Poprawski T. A case report of Brugada-type electrocardiographic changes in a patient taking lithium. Am J Emerg Med. 2008; 26: 113.
- ↑ Wright D, Salehian O. Brugada-Type Electrocardiographic Changes Induced by Long-Term Lithium Use. Circulation, FRCPC2010;122:e418-e419
- ↑ Laske C, Soekadar SR, Laszlo R, Plewnia C. Brugada syndrome in a patient treated with lithium. Am J Psychiatry. 2007; 164: 1440–1441.
- ↑ Belhassen B, Glick A, Viskin S (2004). "Efficacy of quinidine in high-risk patients with Brugada syndrome". Circulation. 110 (13): 1731–7. doi:10.1161/01.CIR.0000143159.30585.90. PMID 15381640.
External links
- GeneReviews: Brugada syndrome
- Algado et al: http://www.medspain.com/ant/n13_jun00/Brugada.htm
- Behr: http://www.c-r-y.org.uk/long_qt_syndrome.htm
- The Ramon Brugada Senior Foundation
- http://digilander.libero.it/martini_syndrome/
de:Brugada-Syndrom
it:Sindrome di Brugada
he:תסמונת ברוגדה
fi:Brugadan oireyhtymä