Brugada syndrome: Difference between revisions

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   Name          = Brugada syndrome |
   Name          = Brugada syndrome |
   Image          = BrugadaS.jpg|
   Image          = BrugadaS.jpg|
   Caption        = ECG findings of Brugada Syndrome|
   Caption        = ECG in Type I Brugada Pattern|
  DiseasesDB    = 31999 |
  ICD10          = {{ICD10|I|42|8|i|30}} |
  ICD9          = {{ICD9|746.89}} |
  ICDO          = |
  OMIM          = 601144 |
  MedlinePlus    = |
  MeshID        = D053840 |
}}
}}
'''For patient information, click [[Brugada syndrome (patient information)|here]]'''


{{Brugada syndrome}}
{{Brugada syndrome}}
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{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}
{{CMG}}; '''Associate Editor-In-Chief:''' {{CZ}}


{{SK}} Sudden unexpected death syndrome; SUDS
{{SK}} Sudden unexpected death syndrome; sudden unexplained death syndrome; SUDS; sudden unexpected nocturnal death syndrome; sudden unexplained nocturnal death syndrome; SUNDS; in the Phillipines ''bangungut'' (to rise and moan in sleep); in Thailand ''lai tai''; in Laos ''Dab tsog'' and in Japan ''Pokkuri disease''
==Differential Diagnosis==


==Characteristics==
==[[Brugada syndrome overview|Overview]]==


*Characterized by a coved-type ST-segment elevation in the right precordial leads
==[[Brugada syndrome classification|Classification]]==
*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==
==[[Brugada syndrome historical perspective|Historical Perspective]]==


==Arrhythmias==
==[[Brugada syndrome pathophysiology|Pathophysiology]]==


# Polymorphic VT resembling a rapid Torsade de Pointes (TdP)
==[[Brugada syndrome differential diagnosis|Differentiating Brugada syndrome from other Diseases]]==
# 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.
==[[Brugada syndrome epidemiology and demographics|Epidemiology and Demographics]]==
 
==[[Brugada syndrome risk factors|Risk Factors]]==
 
==[[Brugada syndrome screening|Screening]]==
 
==[[Brugada syndrome natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
 
==Brugada syndrome diagnosis|Diagnosis==
[[Brugada syndrome diagnostic criteria|Diagnostic Criteria]] | [[Brugada syndrome history and symptoms|History and Symptoms]] | [[Brugada syndrome physical examination|Physical Examination]] | [[Brugada syndrome laboratory findings|Laboratory Findings]] | [[Brugada syndrome electrocardiogram|Electrocardiogram]] | [[Brugada syndrome chest x ray|Chest X Ray]] | [[Brugada syndrome echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Brugada syndrome electrophysiologic studies|Electrophysiologic Studies]] | [[Brugada syndrome genetic testing|Genetic Testing]]


==Treatment==
==Treatment==
[[Brugada syndrome medical therapy|Medical Therapy]] | [[Brugada syndrome drugs to avoid|Drugs to Avoid]] | [[Brugada syndrome drugs to preferably avoid|Drugs to Preferably Avoid]] | [[Brugada syndrome cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Brugada syndrome future or investigational therapies|Future or Investigational Therapies]]


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 [[defibrillation|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 I</sup>to channels.<ref name="pmid15381640">{{cite journal |author=Belhassen B, Glick A, Viskin S |title=Efficacy of quinidine in high-risk patients with Brugada syndrome |journal=Circulation |volume=110 |issue=13 |pages=1731–7 |year=2004 |pmid=15381640 |doi=10.1161/01.CIR.0000143159.30585.90}}</ref> Those with risk factors for [[coronary artery disease]] may require an angiogram before ICD implantation.
==Case Studies==
[[Brugada syndrome case study one|Case #1]]


* Aborted sudden death are at high risk for recurrence and should receive an ICD
==Related Chapters==
* 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==
* [[Cardiac action potential]]
* [[Cardiac action potential]]
* [[Genetics]]
* [[Genetics]]
* [[Ion channel]]
* [[Ion channel]]
* [[Tambocor]]
* [[Tambocor]]
==References==
{{Reflist|2}}
==External links==
* [http://www.genetests.org/profiles/brugada 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
* [http://www.brugada.org The Ramon Brugada Senior Foundation ]
* http://digilander.libero.it/martini_syndrome/


{{Electrocardiography}}
{{Electrocardiography}}
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[[zh:布魯格達氏症候群]]
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[[Category:Electrophysiology]]
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[[Category:Channelopathy]]
[[Category:Channelopathy]]
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Latest revision as of 05:04, 20 August 2020

Brugada syndrome
ECG in Type I Brugada Pattern

For patient information, click here

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Overview

Historical Perspective

Classification

Pathophysiology

Differentiating Brugada syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

Examples of Type I Brugada Syndrome

Chest X Ray

Echocardiography or Ultrasound

Electrophysiologic Studies

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Case #1

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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; sudden unexplained death syndrome; SUDS; sudden unexpected nocturnal death syndrome; sudden unexplained nocturnal death syndrome; SUNDS; in the Phillipines bangungut (to rise and moan in sleep); in Thailand lai tai; in Laos Dab tsog and in Japan Pokkuri disease

Overview

Classification

Historical Perspective

Pathophysiology

Differentiating Brugada syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Brugada syndrome diagnosis|Diagnosis

Diagnostic Criteria | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | Echocardiography or Ultrasound | Electrophysiologic Studies | Genetic Testing

Treatment

Medical Therapy | Drugs to Avoid | Drugs to Preferably Avoid | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters


de:Brugada-Syndrom it:Sindrome di Brugada he:תסמונת ברוגדה fi:Brugadan oireyhtymä


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