Brugada syndrome
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.
Diagnosis
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.[1] 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
- ↑ 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/
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