Brugada syndrome treatment: Difference between revisions

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{{Brugada syndrome}}
{{Brugada syndrome}}


{{CMG}}
{{CMG}} {{AE}} {{S.G.}}
==Overview==
==Overview==
Implantation of a cardiac defibrillator is the only proven method of treatment in Brugada syndrome.Patients with aborted [[sudden cardiac death]] are at high risk for recurrence and should undergo [[AICD]] implantation, and do not require an electrophysiologic study to assess inducibility.  Patients with symptoms (either [[syncope]], [[seizures]] or nocturnal [[agonal respirations]]) should undergo implantation of a [[defibrillator]] if no other cause of their symptoms can be identified.  Asymptomatic patients should undergo electrophysiologic testing, and if [[VT]] / [[VF]] can be induced, they should undergo implantation of an [[ICD]].  Asymptomatic patients who cannot be induced should followed-up closely.  Patients who are asymptomatic with no family history of Brugada syndrome can be followed-up closely.
Implantation of a [[cardiac]] [[defibrillator]] is the only proven method of treatment in Brugada syndrome.[[Patient|Patients]] with aborted [[sudden cardiac death]] are at high risk for recurrence and should undergo [[AICD]] implantation, and do not require an [[Electrophysiologic Testing|electrophysiologic]] study to assess inducibility.  [[Patient|Patients]] with [[Symptom|symptoms]] (either [[syncope]], [[seizures]] or nocturnal [[agonal respirations]]) should undergo implantation of a [[defibrillator]] if no other cause of their [[Symptom|symptoms]] can be identified.  [[Asymptomatic]] [[Patient|patients]] should undergo [[Electrophysiologic Testing|electrophysiologic testing]], and if [[VT]] / [[VF]] can be induced, they should undergo implantation of an [[ICD]].  [[Asymptomatic]] patients who cannot be induced should followed-up closely.  [[Patient|Patients]] who are [[asymptomatic]] with no [[family history]] of Brugada syndrome can be followed-up closely.


==The Two Patient Groups==
==The Two Patient Groups==
The 2005 consensus statement divides patients into two groups:
Patients divides patients into two groups:<ref name="pmid30310491">{{cite journal |vauthors=Batchvarov VN |title=The Brugada Syndrome - Diagnosis, Clinical Implications and Risk Stratification |journal=Eur Cardiol |volume=9 |issue=2 |pages=82–87 |date=December 2014 |pmid=30310491 |pmc=6159405 |doi=10.15420/ecr.2014.9.2.82 |url=}}</ref>


*Higher risk patients with spontaneous Type I Brugada pattern
*Higher risk patients with spontaneous Type I Brugada pattern
*A less high risk cohort of patients who require infusion of a sodium channel blocker to induce a Type I Brugada pattern.
*A less high risk cohort of [[Patient|patients]] who require infusion of a [[sodium]] [[channel blocker]] to induce a Type I Brugada pattern.


The management of these two groups of patients will be discussed separately.
The management of these two groups of [[Patient|patients]] will be discussed separately.


==Management of Patients with a Spontaneous Type I Brugada Pattern==
==Management of Patients with a Spontaneous Type I Brugada Pattern==
Implantation of a cardiac defibrillator should be considered in the following patients:
Implantation of a [[cardiac]] [[defibrillator]] should be considered in the following [[Patient|patients]]:<ref name="pmid175681712">{{cite journal |vauthors=Abu Sham'a RA, Kufri FH, Yassin IH |title=Brugada syndrome: an unusual cause of syncope in a young patient |journal=Ann Saudi Med |volume=27 |issue=3 |pages=201–5 |date=2007 |pmid=17568171 |pmc=6077086 |doi=10.5144/0256-4947.2007.201 |url=}}</ref>
=== Symptomatic Patients===
=== Symptomatic Patients===
*Patients with aborted [[sudden cardiac death]]
*[[Patient|Patients]] with aborted [[sudden cardiac death]]
*Patients with [[syncope]], [[seizures]] or nocturnal [[agonal respirations]] who have no other identifiable cause for their symptoms
*[[Patient|Patients]] with [[syncope]], [[seizures]] or nocturnal [[agonal respirations]] who have no other identifiable cause for their [[Symptom|symptoms]]


The flowchart below summarizes the recommendations of the 2005 consensus panel:
<br />
[[File:Slide1.PNG|center|500px]]
----
----


=== Asymptomatic Patients===
=== Asymptomatic Patients===
*Patients with a family history of [[sudden cardiac death]] that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.
*[[Patient|Patients]] with a [[family history]] of [[sudden cardiac death]] that is suspected to be due to Brugada syndrome in whom [[Ventricular tachycardia|VT]] [[Ventricular fibrillation|VF]] can be induced on [[Electrophysiologic study|electrophysiologic testing]].<ref name="pmid30918662">{{cite journal |vauthors=Pappone C, Santinelli V |title=Brugada Syndrome: Progress in Diagnosis and Management |journal=Arrhythm Electrophysiol Rev |volume=8 |issue=1 |pages=13–18 |date=March 2019 |pmid=30918662 |pmc=6434501 |doi=10.15420/aer.2018.73.2 |url=}}</ref>
*Patients with no family history of [[sudden cardiac death]] in whom VT VF can be induced on electrophysiologic testing.
*[[Patient|Patients]] with no [[family history]] of [[sudden cardiac death]] in whom [[Ventricular tachycardia|VT]] [[Ventricular fibrillation|VF]] can be induced on [[Electrophysiologic Testing|electrophysiologic testing.]]


In essence, if VT VF can be induced on electrophysiologic testing in these patients, a cardiac defibrillator should be implanted.  It is unclear if the same recommendations apply to those patients who require that the electrodes be placed one to two intercostal spaces higher to demonstrate a Brugada type I electrocardiographic pattern.
In essence, if [[Ventricular tachycardia|VT]] [[Ventricular fibrillation|VF]] can be induced on [[electrophysiologic testing]] in these [[Patient|patients]], a [[cardiac]] [[defibrillator]] should be implanted.  It is unclear if the same recommendations apply to those [[Patient|patients]] who require that the [[Electrode|electrodes]] be placed one to two [[intercostal]] spaces higher to demonstrate a Brugada type I [[electrocardiographic]] [[pattern]].
 
The flowchart below summarizes the recommendations of the 2005 consensus panel:
[[File:Slide2.PNG|center|500px]]


<br />
==Management of Patients with a Sodium Channel Induced Type I Brugada Pattern==
==Management of Patients with a Sodium Channel Induced Type I Brugada Pattern==
Implantation of a cardiac defibrillator should be considered in the following patients:
[[Implantation]] of a [[cardiac]] [[Defibrillation|defibrillator]] should be considered in the following [[Patient|patients]]:<ref name="pmid282176152">{{cite journal |vauthors=Swe T, Dogar MH |title=Type 1 Brugada pattern electrocardiogram induced by hypokalemia |journal=J Family Med Prim Care |volume=5 |issue=3 |pages=709–711 |date=2016 |pmid=28217615 |pmc=5290792 |doi=10.4103/2249-4863.197295 |url=}}</ref>
=== Symptomatic Patients===
=== Symptomatic Patients===
*Patients with aborted [[sudden cardiac death]]
*[[Patients]] with aborted [[sudden cardiac death]]
*Patients with [[syncope]], [[seizures]] or nocturnal [[agonal respirations]] who have no other identifiable cause for their symptoms
*[[Patient|Patients]] with [[syncope]], [[seizures]] or nocturnal [[agonal respirations]] who have no other identifiable cause for their [[Symptom|symptoms]]
The flowchart below summarizes the recommendations of the 2005 consensus panel:
 
<br />
[[File:Slide3.PNG|center|500px]]
[[File:Slide3.PNG|center|500px]]
----
----
=== Asymptomatic Patients===
=== Asymptomatic Patients===
*Patients with a family history of [[sudden cardiac death]] that is suspected to be due to Brugada syndrome in whom VT VF can be induced on electrophysiologic testing.
*[[Patient|Patients]] with a [[family history]] of [[sudden cardiac death]] that is suspected to be due to Brugada syndrome in whom [[Ventricular tachycardia|VT]] [[VF]] can be induced on [[Electrophysiologic Testing|electrophysiologic testing.]]<ref name="pmid309186622">{{cite journal |vauthors=Pappone C, Santinelli V |title=Brugada Syndrome: Progress in Diagnosis and Management |journal=Arrhythm Electrophysiol Rev |volume=8 |issue=1 |pages=13–18 |date=March 2019 |pmid=30918662 |pmc=6434501 |doi=10.15420/aer.2018.73.2 |url=}}</ref>


The flowchart below summarizes the recommendations of the 2005 consensus panel.
<br />
[[File:Slide4.PNG|center|500px]]
[[File:Slide4.PNG|center|500px]]


==Pharmacotherapy==
==Pharmacotherapy==


* Pharmacotherapy alone may not be sufficient to treat Brugada syndrome, but it may be required in regions of the world where ICD implantation is cost prohibitive or in infants.<ref name="Al-KhatibStevenson2018">{{cite journal|last1=Al-Khatib|first1=Sana M.|last2=Stevenson|first2=William G.|last3=Ackerman|first3=Michael J.|last4=Bryant|first4=William J.|last5=Callans|first5=David J.|last6=Curtis|first6=Anne B.|last7=Deal|first7=Barbara J.|last8=Dickfeld|first8=Timm|last9=Field|first9=Michael E.|last10=Fonarow|first10=Gregg C.|last11=Gillis|first11=Anne M.|last12=Granger|first12=Christopher B.|last13=Hammill|first13=Stephen C.|last14=Hlatky|first14=Mark A.|last15=Joglar|first15=José A.|last16=Kay|first16=G. Neal|last17=Matlock|first17=Daniel D.|last18=Myerburg|first18=Robert J.|last19=Page|first19=Richard L.|title=2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death|journal=Circulation|volume=138|issue=13|year=2018|issn=0009-7322|doi=10.1161/CIR.0000000000000549}}</ref>
* Pharmacotherapy alone may not be sufficient to treat Brugada syndrome, but it may be required in regions of the world where ICD implantation is cost prohibitive or in [[Infant|infants]].<ref name="Al-KhatibStevenson2018">{{cite journal|last1=Al-Khatib|first1=Sana M.|last2=Stevenson|first2=William G.|last3=Ackerman|first3=Michael J.|last4=Bryant|first4=William J.|last5=Callans|first5=David J.|last6=Curtis|first6=Anne B.|last7=Deal|first7=Barbara J.|last8=Dickfeld|first8=Timm|last9=Field|first9=Michael E.|last10=Fonarow|first10=Gregg C.|last11=Gillis|first11=Anne M.|last12=Granger|first12=Christopher B.|last13=Hammill|first13=Stephen C.|last14=Hlatky|first14=Mark A.|last15=Joglar|first15=José A.|last16=Kay|first16=G. Neal|last17=Matlock|first17=Daniel D.|last18=Myerburg|first18=Robert J.|last19=Page|first19=Richard L.|title=2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death|journal=Circulation|volume=138|issue=13|year=2018|issn=0009-7322|doi=10.1161/CIR.0000000000000549}}</ref>


* [[Quinidine]] reduces the number of [[VF]] episodes and corrects spontaneous ECG changes, possibly via inhibiting I(to) channels.  No drug has demonstrated long term efficacy in the prevention of [[sudden cardiac death]].<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><ref name="TsuchiyaAshikaga2002">{{cite journal|last1=Tsuchiya|first1=Takeshi|last2=Ashikaga|first2=Keiichi|last3=Honda|first3=Toshihiro|last4=Arita|first4=Makoto|title=Prevention of Ventricular Fibrillation by Cilostazol, an Oral Phosphodiesterase Inhibitor, in a Patient with Brugada Syndrome|journal=Journal of Cardiovascular Electrophysiology|volume=13|issue=7|year=2002|pages=698–701|issn=1045-3873|doi=10.1046/j.1540-8167.2002.00698.x}}</ref><ref name="AbudBagattin2006">{{cite journal|last1=Abud|first1=Atilio|last2=Bagattin|first2=Daniel|last3=Goyeneche|first3=Raul|last4=Becker|first4=Carlos|title=Failure of Cilostazol in the Prevention of Ventricular Fibrillation in a Patient with Brugada Syndrome|journal=Journal of Cardiovascular Electrophysiology|volume=17|issue=2|year=2006|pages=210–212|issn=1045-3873|doi=10.1111/j.1540-8167.2005.00290.x}}</ref><ref name="MatsuiKiyosue1999">{{cite journal|last1=Matsui|first1=Kazunori|last2=Kiyosue|first2=Tatsuto|last3=Wang|first3=Jin-Cheng|last4=Dohi|first4=Kazuhiro|last5=Arita|first5=Makoto|journal=Cardiovascular Drugs and Therapy|volume=13|issue=2|year=1999|pages=105–113|issn=09203206|doi=10.1023/A:1007779908346}}</ref><ref name="MiyazakiMitamura1996">{{cite journal|last1=Miyazaki|first1=Toshihisa|last2=Mitamura|first2=Hideo|last3=Miyoshi|first3=Shunichiro|last4=Soejima|first4=Kyoko|last5=Aizawa|first5=Yoshifusa|last6=Ogawa|first6=Satoshi|title=Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome|journal=Journal of the American College of Cardiology|volume=27|issue=5|year=1996|pages=1061–1070|issn=07351097|doi=10.1016/0735-1097(95)00613-3}}</ref><ref name="SuzukiTorigoe2000">{{cite journal|last1=Suzuki|first1=Hiroshi|last2=Torigoe|first2=Katsumi|last3=Numata|first3=Osamu|last4=Yazaki|first4=Satoshi|title=Infant Case with a Malignant Form of Brugada Syndrome|journal=Journal of Cardiovascular Electrophysiology|volume=11|issue=11|year=2000|pages=1277–1280|issn=1045-3873|doi=10.1046/j.1540-8167.2000.01277.x}}</ref>
* [[Quinidine]] reduces the number of [[VF]] episodes and corrects spontaneous [[The electrocardiogram|ECG]] changes, possibly via inhibiting I(to) channels.  No drug has demonstrated long term efficacy in the prevention of [[sudden cardiac death]].<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><ref name="TsuchiyaAshikaga2002">{{cite journal|last1=Tsuchiya|first1=Takeshi|last2=Ashikaga|first2=Keiichi|last3=Honda|first3=Toshihiro|last4=Arita|first4=Makoto|title=Prevention of Ventricular Fibrillation by Cilostazol, an Oral Phosphodiesterase Inhibitor, in a Patient with Brugada Syndrome|journal=Journal of Cardiovascular Electrophysiology|volume=13|issue=7|year=2002|pages=698–701|issn=1045-3873|doi=10.1046/j.1540-8167.2002.00698.x}}</ref><ref name="AbudBagattin2006">{{cite journal|last1=Abud|first1=Atilio|last2=Bagattin|first2=Daniel|last3=Goyeneche|first3=Raul|last4=Becker|first4=Carlos|title=Failure of Cilostazol in the Prevention of Ventricular Fibrillation in a Patient with Brugada Syndrome|journal=Journal of Cardiovascular Electrophysiology|volume=17|issue=2|year=2006|pages=210–212|issn=1045-3873|doi=10.1111/j.1540-8167.2005.00290.x}}</ref><ref name="MatsuiKiyosue1999">{{cite journal|last1=Matsui|first1=Kazunori|last2=Kiyosue|first2=Tatsuto|last3=Wang|first3=Jin-Cheng|last4=Dohi|first4=Kazuhiro|last5=Arita|first5=Makoto|journal=Cardiovascular Drugs and Therapy|volume=13|issue=2|year=1999|pages=105–113|issn=09203206|doi=10.1023/A:1007779908346}}</ref><ref name="MiyazakiMitamura1996">{{cite journal|last1=Miyazaki|first1=Toshihisa|last2=Mitamura|first2=Hideo|last3=Miyoshi|first3=Shunichiro|last4=Soejima|first4=Kyoko|last5=Aizawa|first5=Yoshifusa|last6=Ogawa|first6=Satoshi|title=Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome|journal=Journal of the American College of Cardiology|volume=27|issue=5|year=1996|pages=1061–1070|issn=07351097|doi=10.1016/0735-1097(95)00613-3}}</ref><ref name="SuzukiTorigoe2000">{{cite journal|last1=Suzuki|first1=Hiroshi|last2=Torigoe|first2=Katsumi|last3=Numata|first3=Osamu|last4=Yazaki|first4=Satoshi|title=Infant Case with a Malignant Form of Brugada Syndrome|journal=Journal of Cardiovascular Electrophysiology|volume=11|issue=11|year=2000|pages=1277–1280|issn=1045-3873|doi=10.1046/j.1540-8167.2000.01277.x}}</ref>
*Pharmacological tests using a sodium-channel blocker drug should be used to evaluate suspected Brugada syndrome. Intravenous ajmaline and flecainide are the most widely used agents. The test is considered positive if a type 1 ECG pattern is identified during drug infusion.
*[[Pharmacological]] tests using a [[sodium]]-[[channel blocker]] [[drug]] should be used to evaluate suspected Brugada syndrome. Intravenous [[ajmaline]] and [[flecainide]] are the most widely used agents. The test is considered positive if a type 1 [[ECG]] [[pattern]] is identified during [[drug]] [[infusion]].<ref name="pmid22203660">{{cite journal |vauthors=Obeyesekere MN, Klein GJ, Modi S, Leong-Sit P, Gula LJ, Yee R, Skanes AC, Krahn AD |title=How to perform and interpret provocative testing for the diagnosis of Brugada syndrome, long-QT syndrome, and catecholaminergic polymorphic ventricular tachycardia |journal=Circ Arrhythm Electrophysiol |volume=4 |issue=6 |pages=958–64 |date=December 2011 |pmid=22203660 |doi=10.1161/CIRCEP.111.965947 |url=}}</ref>


<br />
<br />
===Drugs with Potential Antiarrhythmic Effect===
===Drugs with Potential Antiarrhythmic Effect===
(Alphabetical order generic name)
<font size="-1">
<font size="-1">
{| cellspacing="2" cellpadding="3" border="0"
{| cellspacing="2" cellpadding="3" border="0"
| '''Generic name'''
|'''Generic name'''
| '''Brand name®'''
|'''Brand name®'''
| '''Class / Clinical use'''
|'''Class / Clinical use'''
| '''References'''
|'''References'''
| '''Recommendation'''
|'''Recommendation'''
|-
|-
| [http://www.drugbank.ca/drugs/DB01166 Cilostazol]
|[http://www.drugbank.ca/drugs/DB01166 Cilostazol]
| e.g.<br />Pletal®
| e.g.<br />Pletal®
| Phosphodiesterase inhibitor
| Phosphodiesterase inhibitor
| [http://www.ncbi.nlm.nih.gov/pubmed/12139296?dopt=Citation Tsuchiya 2002]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16533260?dopt=Citation Abud 2006]<br />[http://www.ncbi.nlm.nih.gov/pubmed/10372225?dopt=Citation Matsui 1999]
|[http://www.ncbi.nlm.nih.gov/pubmed/12139296?dopt=Citation Tsuchiya 2002]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16533260?dopt=Citation Abud 2006]<br />[http://www.ncbi.nlm.nih.gov/pubmed/10372225?dopt=Citation Matsui 1999]
| Class IIb
| Class IIb
|-
|-
| [http://www.drugbank.ca/drugs/DB01064 Isoproterenol]<br />[http://www.drugbank.ca/drugs/DB01064 Isoprenaline]
|[http://www.drugbank.ca/drugs/DB01064 Isoproterenol]<br />[http://www.drugbank.ca/drugs/DB01064 Isoprenaline]
| e.g.<br />Isuprel®
| e.g.<br />Isuprel®
| Beta-adrenergic receptor stimulation
| Beta-adrenergic receptor stimulation
| [http://www.ncbi.nlm.nih.gov/pubmed/8609322?dopt=Citation Miyazaki 1996]<br />[http://www.ncbi.nlm.nih.gov/pubmed/11083249?dopt=Citation Suzuki 2000]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16760208?dopt=Citation Watanabe 2006]<br />[http://www.ncbi.nlm.nih.gov/pubmed/17556186?dopt=Citation Ohgo 2007]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16397147?dopt=Citation Ganesan 2006]
|[http://www.ncbi.nlm.nih.gov/pubmed/8609322?dopt=Citation Miyazaki 1996]<br />[http://www.ncbi.nlm.nih.gov/pubmed/11083249?dopt=Citation Suzuki 2000]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16760208?dopt=Citation Watanabe 2006]<br />[http://www.ncbi.nlm.nih.gov/pubmed/17556186?dopt=Citation Ohgo 2007]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16397147?dopt=Citation Ganesan 2006]
| Class I
| Class I
|-
|-
| [http://www.drugbank.ca/drugs/DB00816 Orciprenaline]
|[http://www.drugbank.ca/drugs/DB00816 Orciprenaline]
| e.g.<br />Alotec®<br />Metaprel®<br />Novasmasol®
| e.g.<br />Alotec®<br />Metaprel®<br />Novasmasol®
| Beta-adrenergic receptor stimulation
| Beta-adrenergic receptor stimulation
| [http://www.ncbi.nlm.nih.gov/pubmed/19346290?dopt=Citation Kyriazis 2009]
|[http://www.ncbi.nlm.nih.gov/pubmed/19346290?dopt=Citation Kyriazis 2009]
| Class IIa
| Class IIa
|-
|-
| [http://www.drugbank.ca/drugs/DB00908 Quinidine]
|[http://www.drugbank.ca/drugs/DB00908 Quinidine]
| e.g.<br />Quinalan®<br />Chinidin®
| e.g.<br />Quinalan®<br />Chinidin®
| Antiarrhythmic Agent
| Antiarrhythmic Agent
| [http://www.ncbi.nlm.nih.gov/pubmed/11083249?dopt=Citation Suzuki 2000]<br />[http://www.ncbi.nlm.nih.gov/pubmed/11584468?dopt=Citation Alings 2001]<br />[http://www.ncbi.nlm.nih.gov/pubmed/15381640?dopt=Citation Belhassen 2004]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16633076?dopt=Citation Mizusawa 2006]<br />[http://www.ncbi.nlm.nih.gov/pubmed/17404158?dopt=Citation Probst 2007]<br />[http://www.ncbi.nlm.nih.gov/pubmed/17556186?dopt=Citation Ohgo 2007]<br />[http://www.ncbi.nlm.nih.gov/pubmed/10517739?dopt=Citation Yan 1999]
|[http://www.ncbi.nlm.nih.gov/pubmed/11083249?dopt=Citation Suzuki 2000]<br />[http://www.ncbi.nlm.nih.gov/pubmed/11584468?dopt=Citation Alings 2001]<br />[http://www.ncbi.nlm.nih.gov/pubmed/15381640?dopt=Citation Belhassen 2004]<br />[http://www.ncbi.nlm.nih.gov/pubmed/16633076?dopt=Citation Mizusawa 2006]<br />[http://www.ncbi.nlm.nih.gov/pubmed/17404158?dopt=Citation Probst 2007]<br />[http://www.ncbi.nlm.nih.gov/pubmed/17556186?dopt=Citation Ohgo 2007]<br />[http://www.ncbi.nlm.nih.gov/pubmed/10517739?dopt=Citation Yan 1999]
| Class I
| Class I
|-
|-
Line 102: Line 97:
==Treatment of VT Storm==
==Treatment of VT Storm==


* VT storm has been successfully treated with [[isoproterenol]].  The mechanism is thought to be augmenting the cardiac L type channel.
*[[Ventricular tachycardia|VT]] storm has been successfully treated with [[isoproterenol]].  The mechanism is thought to be augmenting the [[cardiac]] L type channel.<ref name="pmid29757020">{{cite journal |vauthors=Argenziano M, Antzelevitch C |title=Recent advances in the treatment of Brugada syndrome |journal=Expert Rev Cardiovasc Ther |volume=16 |issue=6 |pages=387–404 |date=June 2018 |pmid=29757020 |pmc=6330094 |doi=10.1080/14779072.2018.1475230 |url=}}</ref>


==Treatment of Coronary Ischemia==
==Treatment of Coronary Ischemia==


* Patients with risk factors for [[coronary artery disease]] may require an angiogram before ICD implantation.
*[[Patient|Patients]] with [[Risk factor|risk factors]] for [[coronary artery disease]] may require an [[angiogram]] before [[Implantable cardioverter defibrillator|ICD]] [[implantation]].<ref name="pmid28090040">{{cite journal |vauthors=Kujime S, Sakurada H, Saito N, Enomoto Y, Ito N, Nakamura K, Fukamizu S, Tejima T, Yambe Y, Nishizaki M, Noro M, Hiraoka M, Sugi K |title=Outcomes of Brugada Syndrome Patients with Coronary Artery Vasospasm |journal=Intern. Med. |volume=56 |issue=2 |pages=129–135 |date=2017 |pmid=28090040 |pmc=5337455 |doi=10.2169/internalmedicine.56.7307 |url=}}</ref>


==Treatment of Factors that may Precipitate Brugada Type EKG Changes and Clinical Symptoms==
==Treatment of Factors that may Precipitate Brugada Type EKG Changes and Clinical Symptoms==
*Fever in a Brugada syndrome patient should be treated with an [[antipyretic]].
*[[Fever]] in a Brugada syndrome [[patient]] should be treated with an [[antipyretic]].<ref name="AminKlemens2010">{{cite journal|last1=Amin|first1=A. S.|last2=Klemens|first2=C. A.|last3=Meregalli|first3=P. G.|last4=Asghari-Roodsari|first4=A.|last5=de Bakker|first5=J. M. T.|last6=January|first6=C. T.|last7=Wilde|first7=A. A. M.|last8=Tan|first8=H. L.|title=Fever-triggered ventricular arrhythmias in Brugada syndrome and type 2 long-QT syndrome|journal=Netherlands Heart Journal|volume=18|issue=3|year=2010|pages=165–169|issn=1568-5888|doi=10.1007/BF03091755}}</ref>
*Brugada syndrome patients should avoid hot tubs, very hot baths or extremely hot climates.
*Brugada syndrome [[Patient|patients]] should avoid hot tubs, very hot baths or extremely hot climates.<ref name="pmid15627121">{{cite journal |vauthors=Antzelevitch C, Brugada P, Brugada J, Brugada R |title=Brugada syndrome: from cell to bedside |journal=Curr Probl Cardiol |volume=30 |issue=1 |pages=9–54 |date=January 2005 |pmid=15627121 |pmc=1475801 |doi=10.1016/j.cpcardiol.2004.04.005 |url=}}</ref>
*[[Hypokalemia]], [[hyperkalemia]], and [[hypercalcemia]] should be treated aggressively.
*[[Hypokalemia]], [[hyperkalemia]], and [[hypercalcemia]] should be treated aggressively.<ref name="pmid28217615">{{cite journal |vauthors=Swe T, Dogar MH |title=Type 1 Brugada pattern electrocardiogram induced by hypokalemia |journal=J Family Med Prim Care |volume=5 |issue=3 |pages=709–711 |date=2016 |pmid=28217615 |pmc=5290792 |doi=10.4103/2249-4863.197295 |url=}}</ref>
*Carbohydrate loading should be avoided.
* [[Carbohydrate|rugada syndrome  arbohydrate]] loading should be avoided.


====Contraindicated medications====
====Contraindicated medications====
Line 120: Line 115:
|MedCond = Brugada syndrome|Propafenone}}
|MedCond = Brugada syndrome|Propafenone}}


== ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) <ref name="pmid16935995">{{cite journal| author=Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M et al.| title=ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. | journal=Circulation | year= 2006 | volume= 114 | issue= 10 | pages= e385-484 | pmid=16935995 | doi=10.1161/CIRCULATIONAHA.106.178233 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16935995}}</ref> ==
== ACC/AHA/ESC 2017 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT)<ref>{{cite journal|doi=10.1161/CIR.000000000000054}}</ref>==


=== Recommendations for Brugada Syndrome ===
=== Recommendations for Brugada Syndrome ===
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{|class="wikitable"
{|class="wikitable"
|-
|-
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]] <ref name="pmid19398665">{{cite journal |vauthors=Hayashi M, Denjoy I, Extramiana F, Maltret A, Buisson NR, Lupoglazoff JM, Klug D, Hayashi M, Takatsuki S, Villain E, Kamblock J, Messali A, Guicheney P, Lunardi J, Leenhardt A |title=Incidence and risk factors of arrhythmic events in catecholaminergic polymorphic ventricular tachycardia |journal=Circulation |volume=119 |issue=18 |pages=2426–34 |date=May 2009 |pmid=19398665 |doi=10.1161/CIRCULATIONAHA.108.829267 |url=}}</ref><ref name="pmid25713214">{{cite journal |vauthors=Roston TM, Vinocur JM, Maginot KR, Mohammed S, Salerno JC, Etheridge SP, Cohen M, Hamilton RM, Pflaumer A, Kanter RJ, Potts JE, LaPage MJ, Collins KK, Gebauer RA, Temple JD, Batra AS, Erickson C, Miszczak-Knecht M, Kubuš P, Bar-Cohen Y, Kantoch M, Thomas VC, Hessling G, Anderson C, Young ML, Cabrera Ortega M, Lau YR, Johnsrude CL, Fournier A, Kannankeril PJ, Sanatani S |title=Catecholaminergic polymorphic ventricular tachycardia in children: analysis of therapeutic strategies and outcomes from an international multicenter registry |journal=Circ Arrhythm Electrophysiol |volume=8 |issue=3 |pages=633–42 |date=June 2015 |pmid=25713214 |pmc=4472494 |doi=10.1161/CIRCEP.114.002217 |url=}}</ref><ref name="pmid257132142">{{cite journal |vauthors=Roston TM, Vinocur JM, Maginot KR, Mohammed S, Salerno JC, Etheridge SP, Cohen M, Hamilton RM, Pflaumer A, Kanter RJ, Potts JE, LaPage MJ, Collins KK, Gebauer RA, Temple JD, Batra AS, Erickson C, Miszczak-Knecht M, Kubuš P, Bar-Cohen Y, Kantoch M, Thomas VC, Hessling G, Anderson C, Young ML, Cabrera Ortega M, Lau YR, Johnsrude CL, Fournier A, Kannankeril PJ, Sanatani S |title=Catecholaminergic polymorphic ventricular tachycardia in children: analysis of therapeutic strategies and outcomes from an international multicenter registry |journal=Circ Arrhythm Electrophysiol |volume=8 |issue=3 |pages=633–42 |date=June 2015 |pmid=25713214 |pmc=4472494 |doi=10.1161/CIRCEP.114.002217 |url=}}</ref><ref name="pmid22192666">{{cite journal |vauthors=Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, Giordano U, Pappone C, Mascioli G, Rossetti G, De Nardis R, Colombo M |title=Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry |journal=J. Am. Coll. Cardiol. |volume=59 |issue=1 |pages=37–45 |date=January 2012 |pmid=22192666 |doi=10.1016/j.jacc.2011.08.064 |url=}}</ref>
| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]]  
|-
|-
| bgcolor="LightGreen"|Suggested phrases for writing recommendations:
| bgcolor="LightGreen"|Suggested phrases for writing recommendations:
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2. In patients with Brugada syndrome with spontaneous type 1 Brugada electrocardiographic pattern and cardiac arrest, sustained VA or a recent history of syncope presumed due to VA, an ICD is recommended if meaningful survival of greater than 1 year is expected.<ref name="pmid221926662">{{cite journal |vauthors=Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, Giordano U, Pappone C, Mascioli G, Rossetti G, De Nardis R, Colombo M |title=Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry |journal=J. Am. Coll. Cardiol. |volume=59 |issue=1 |pages=37–45 |date=January 2012 |pmid=22192666 |doi=10.1016/j.jacc.2011.08.064 |url=}}</ref><ref name="pmid26797467">{{cite journal |vauthors=Sroubek J, Probst V, Mazzanti A, Delise P, Hevia JC, Ohkubo K, Zorzi A, Champagne J, Kostopoulou A, Yin X, Napolitano C, Milan DJ, Wilde A, Sacher F, Borggrefe M, Ellinor PT, Theodorakis G, Nault I, Corrado D, Watanabe I, Antzelevitch C, Allocca G, Priori SG, Lubitz SA |title=Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled Analysis |journal=Circulation |volume=133 |issue=7 |pages=622–30 |date=February 2016 |pmid=26797467 |pmc=4758872 |doi=10.1161/CIRCULATIONAHA.115.017885 |url=}}</ref>
2. In patients with Brugada syndrome with spontaneous type 1 Brugada electrocardiographic pattern and cardiac arrest, sustained VA or a recent history of syncope presumed due to VA, an ICD is recommended if meaningful survival of greater than 1 year is expected.<ref name="pmid221926662">{{cite journal |vauthors=Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, Giordano U, Pappone C, Mascioli G, Rossetti G, De Nardis R, Colombo M |title=Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry |journal=J. Am. Coll. Cardiol. |volume=59 |issue=1 |pages=37–45 |date=January 2012 |pmid=22192666 |doi=10.1016/j.jacc.2011.08.064 |url=}}</ref><ref name="pmid26797467">{{cite journal |vauthors=Sroubek J, Probst V, Mazzanti A, Delise P, Hevia JC, Ohkubo K, Zorzi A, Champagne J, Kostopoulou A, Yin X, Napolitano C, Milan DJ, Wilde A, Sacher F, Borggrefe M, Ellinor PT, Theodorakis G, Nault I, Corrado D, Watanabe I, Antzelevitch C, Allocca G, Priori SG, Lubitz SA |title=Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled Analysis |journal=Circulation |volume=133 |issue=7 |pages=622–30 |date=February 2016 |pmid=26797467 |pmc=4758872 |doi=10.1161/CIRCULATIONAHA.115.017885 |url=}}</ref>


3. In patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT, intensification of therapy with quinidine or catheter ablation is recommended
3. In patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT, intensification of therapy with quinidine or catheter ablation is recommended.<ref name="pmid26354972">{{cite journal |vauthors=Belhassen B, Rahkovich M, Michowitz Y, Glick A, Viskin S |title=Management of Brugada Syndrome: Thirty-Three-Year Experience Using Electrophysiologically Guided Therapy With Class 1A Antiarrhythmic Drugs |journal=Circ Arrhythm Electrophysiol |volume=8 |issue=6 |pages=1393–402 |date=December 2015 |pmid=26354972 |doi=10.1161/CIRCEP.115.003109 |url=}}</ref><ref name="pmid27453126">{{cite journal |vauthors=Zhang P, Tung R, Zhang Z, Sheng X, Liu Q, Jiang R, Sun Y, Chen S, Yu L, Ye Y, Fu G, Shivkumar K, Jiang C |title=Characterization of the epicardial substrate for catheter ablation of Brugada syndrome |journal=Heart Rhythm |volume=13 |issue=11 |pages=2151–2158 |date=November 2016 |pmid=27453126 |doi=10.1016/j.hrthm.2016.07.025 |url=}}</ref>


4. In patients with spontaneous type 1 Brugada electrocardiographic pattern and symptomatic VA who either are not candidates for or decline an ICD, quinidine or catheter ablation is recommended
4. In patients with spontaneous type 1 Brugada electrocardiographic pattern and symptomatic VA who either are not candidates for or decline an ICD, quinidine or catheter ablation is recommended.<ref name="pmid26354972" /><ref name="pmid21403098">{{cite journal |vauthors=Nademanee K, Veerakul G, Chandanamattha P, Chaothawee L, Ariyachaipanich A, Jirasirirojanakorn K, Likittanasombat K, Bhuripanyo K, Ngarmukos T |title=Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium |journal=Circulation |volume=123 |issue=12 |pages=1270–9 |date=March 2011 |pmid=21403098 |doi=10.1161/CIRCULATIONAHA.110.972612 |url=}}</ref><ref name="pmid27453126" />
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| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
|-
|-
| bgcolor="LemonChiffon" |In patients with suspected long QT syndrome, ambulatory electrocardiographic monitoring, recording the ECG lying and immediately on standing, and/or exercise treadmill testing can be useful for establishing a diagnosis and monitoring the response to therapy.
| bgcolor="LemonChiffon" |In patients with suspected long QT syndrome, ambulatory electrocardiographic monitoring, recording the ECG lying and immediately on standing, and/or exercise treadmill testing can be useful for establishing a diagnosis and monitoring the response to therapy.<ref name="pmid28431071">{{cite journal |vauthors=Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde AAM |title=J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge |journal=Europace |volume=19 |issue=4 |pages=665–694 |date=April 2017 |pmid=28431071 |pmc=5834028 |doi=10.1093/europace/euw235 |url=}}</ref><ref name="pmid24657429">{{cite journal |vauthors=Somani R, Krahn AD, Healey JS, Chauhan VS, Birnie DH, Champagne J, Sanatani S, Angaran P, Gow RM, Chakrabarti S, Gerull B, Yee R, Skanes AC, Gula LJ, Leong-Sit P, Klein GJ, Gollob MH, Talajic M, Gardner M, Simpson CS |title=Procainamide infusion in the evaluation of unexplained cardiac arrest: from the Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER) |journal=Heart Rhythm |volume=11 |issue=6 |pages=1047–54 |date=June 2014 |pmid=24657429 |doi=10.1016/j.hrthm.2014.03.022 |url=}}</ref>
|}
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| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
|-
|-
| bgcolor="LemonChiffon" |In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification
| bgcolor="LemonChiffon" |In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification.<ref name="pmid27491905" /><ref name="pmid26797467" /><ref name="pmid20978016">{{cite journal |vauthors=Delise P, Allocca G, Marras E, Giustetto C, Gaita F, Sciarra L, Calo L, Proclemer A, Marziali M, Rebellato L, Berton G, Coro L, Sitta N |title=Risk stratification in individuals with the Brugada type 1 ECG pattern without previous cardiac arrest: usefulness of a combined clinical and electrophysiologic approach |journal=Eur. Heart J. |volume=32 |issue=2 |pages=169–76 |date=January 2011 |pmid=20978016 |pmc=3021386 |doi=10.1093/eurheartj/ehq381 |url=}}</ref><ref name="KusumotoBailey2018">{{cite journal|last1=Kusumoto|first1=Fred M.|last2=Bailey|first2=Kent R.|last3=Chaouki|first3=Ahmad Sami|last4=Deshmukh|first4=Abhishek J.|last5=Gautam|first5=Sandeep|last6=Kim|first6=Robert J.|last7=Kramer|first7=Daniel B.|last8=Lambrakos|first8=Litsa K.|last9=Nasser|first9=Naseer H.|last10=Sorajja|first10=Dan|title=Systematic Review for the 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death|journal=Circulation|volume=138|issue=13|year=2018|issn=0009-7322|doi=10.1161/CIR.0000000000000550}}</ref><ref name="pmid25904495">{{cite journal |vauthors=Sieira J, Conte G, Ciconte G, de Asmundis C, Chierchia GB, Baltogiannis G, Di Giovanni G, Saitoh Y, Irfan G, Casado-Arroyo R, Juliá J, La Meir M, Wellens F, Wauters K, Van Malderen S, Pappaert G, Brugada P |title=Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience |journal=Circ Arrhythm Electrophysiol |volume=8 |issue=4 |pages=777–84 |date=August 2015 |pmid=25904495 |doi=10.1161/CIRCEP.114.002647 |url=}}</ref>
|-
|-
| bgcolor="LemonChiffon" |In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives
| bgcolor="LemonChiffon" |In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives.<ref name="pmid22840528">{{cite journal |vauthors=Crotti L, Marcou CA, Tester DJ, Castelletti S, Giudicessi JR, Torchio M, Medeiros-Domingo A, Simone S, Will ML, Dagradi F, Schwartz PJ, Ackerman MJ |title=Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing |journal=J. Am. Coll. Cardiol. |volume=60 |issue=15 |pages=1410–8 |date=October 2012 |pmid=22840528 |pmc=3624764 |doi=10.1016/j.jacc.2012.04.037 |url=}}</ref><ref name="pmid23414114">{{cite journal |vauthors=Risgaard B, Jabbari R, Refsgaard L, Holst AG, Haunsø S, Sadjadieh A, Winkel BG, Olesen MS, Tfelt-Hansen J |title=High prevalence of genetic variants previously associated with Brugada syndrome in new exome data |journal=Clin. Genet. |volume=84 |issue=5 |pages=489–95 |date=November 2013 |pmid=23414114 |doi=10.1111/cge.12126 |url=}}</ref>
|}
|}


Latest revision as of 17:12, 9 January 2020

Brugada syndrome Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

Implantation of a cardiac defibrillator is the only proven method of treatment in Brugada syndrome.Patients with aborted sudden cardiac death are at high risk for recurrence and should undergo AICD implantation, and do not require an electrophysiologic study to assess inducibility. Patients with symptoms (either syncope, seizures or nocturnal agonal respirations) should undergo implantation of a defibrillator if no other cause of their symptoms can be identified. Asymptomatic patients should undergo electrophysiologic testing, and if VT / VF can be induced, they should undergo implantation of an ICD. Asymptomatic patients who cannot be induced should followed-up closely. Patients who are asymptomatic with no family history of Brugada syndrome can be followed-up closely.

The Two Patient Groups

Patients divides patients into two groups:[1]

  • Higher risk patients with spontaneous Type I Brugada pattern
  • A less high risk cohort of patients who require infusion of a sodium channel blocker to induce a Type I Brugada pattern.

The management of these two groups of patients will be discussed separately.

Management of Patients with a Spontaneous Type I Brugada Pattern

Implantation of a cardiac defibrillator should be considered in the following patients:[2]

Symptomatic Patients


Asymptomatic Patients

In essence, if VT VF can be induced on electrophysiologic testing in these patients, a cardiac defibrillator should be implanted. It is unclear if the same recommendations apply to those patients who require that the electrodes be placed one to two intercostal spaces higher to demonstrate a Brugada type I electrocardiographic pattern.


Management of Patients with a Sodium Channel Induced Type I Brugada Pattern

Implantation of a cardiac defibrillator should be considered in the following patients:[4]

Symptomatic Patients


Asymptomatic Patients


Pharmacotherapy

  • Pharmacotherapy alone may not be sufficient to treat Brugada syndrome, but it may be required in regions of the world where ICD implantation is cost prohibitive or in infants.[6]


Drugs with Potential Antiarrhythmic Effect

Generic name Brand name® Class / Clinical use References Recommendation
Cilostazol e.g.
Pletal® 		Phosphodiesterase inhibitor Tsuchiya 2002
Abud 2006
Matsui 1999 		Class IIb
Isoproterenol
Isoprenaline 		e.g.
Isuprel® 		Beta-adrenergic receptor stimulation Miyazaki 1996
Suzuki 2000
Watanabe 2006
Ohgo 2007
Ganesan 2006 		Class I
Orciprenaline e.g.
Alotec®
Metaprel®
Novasmasol® 		Beta-adrenergic receptor stimulation Kyriazis 2009 Class IIa
Quinidine e.g.
Quinalan®
Chinidin® 		Antiarrhythmic Agent Suzuki 2000
Alings 2001
Belhassen 2004
Mizusawa 2006
Probst 2007
Ohgo 2007
Yan 1999 		Class I
  • Recommendation: Class I: convincing evidence/opinion; Class IIa: evidence/opinion less clear; Class IIb: conflicting evidence/opinion; Class III: very little evidence.

Treatment of VT Storm

  • VT storm has been successfully treated with isoproterenol. The mechanism is thought to be augmenting the cardiac L type channel.[14]

Treatment of Coronary Ischemia

Treatment of Factors that may Precipitate Brugada Type EKG Changes and Clinical Symptoms

Contraindicated medications

Brugada syndrome is considered an absolute contraindication to the use of the following medications:

ACC/AHA/ESC 2017 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT)[19]

Recommendations for Brugada Syndrome

Class I
Suggested phrases for writing recommendations:

1. In asymptomatic patients with only inducible type 1 Brugada electrocardiographic pattern, observation without therapy is recommended.[20][21]

2. In patients with Brugada syndrome with spontaneous type 1 Brugada electrocardiographic pattern and cardiac arrest, sustained VA or a recent history of syncope presumed due to VA, an ICD is recommended if meaningful survival of greater than 1 year is expected.[22][23]

3. In patients with Brugada syndrome experiencing recurrent ICD shocks for polymorphic VT, intensification of therapy with quinidine or catheter ablation is recommended.[24][25]

4. In patients with spontaneous type 1 Brugada electrocardiographic pattern and symptomatic VA who either are not candidates for or decline an ICD, quinidine or catheter ablation is recommended.[24][26][25]

Class IIa
In patients with suspected long QT syndrome, ambulatory electrocardiographic monitoring, recording the ECG lying and immediately on standing, and/or exercise treadmill testing can be useful for establishing a diagnosis and monitoring the response to therapy.[27][28]
Class IIb
In patients with asymptomatic Brugada syndrome and a spontaneous type 1 Brugada electrocardiographic pattern, an electrophysiological study with programmed ventricular stimulation using single and double extrastimuli may be considered for further risk stratification.[20][23][29][30][31]
In patients with suspected or established Brugada syndrome, genetic counseling and genetic testing may be useful to facilitate cascade screening of relatives.[32][33]


References

  1. ↑ Batchvarov VN (December 2014). "The Brugada Syndrome - Diagnosis, Clinical Implications and Risk Stratification". Eur Cardiol. 9 (2): 82–87. doi:10.15420/ecr.2014.9.2.82. PMC 6159405. PMID 30310491.
  2. ↑ Abu Sham'a RA, Kufri FH, Yassin IH (2007). "Brugada syndrome: an unusual cause of syncope in a young patient". Ann Saudi Med. 27 (3): 201–5. doi:10.5144/0256-4947.2007.201. PMC 6077086. PMID 17568171.
  3. ↑ Pappone C, Santinelli V (March 2019). "Brugada Syndrome: Progress in Diagnosis and Management". Arrhythm Electrophysiol Rev. 8 (1): 13–18. doi:10.15420/aer.2018.73.2. PMC 6434501. PMID 30918662.
  4. ↑ Swe T, Dogar MH (2016). "Type 1 Brugada pattern electrocardiogram induced by hypokalemia". J Family Med Prim Care. 5 (3): 709–711. doi:10.4103/2249-4863.197295. PMC 5290792. PMID 28217615.
  5. ↑ Pappone C, Santinelli V (March 2019). "Brugada Syndrome: Progress in Diagnosis and Management". Arrhythm Electrophysiol Rev. 8 (1): 13–18. doi:10.15420/aer.2018.73.2. PMC 6434501. PMID 30918662.
  6. ↑ Al-Khatib, Sana M.; Stevenson, William G.; Ackerman, Michael J.; Bryant, William J.; Callans, David J.; Curtis, Anne B.; Deal, Barbara J.; Dickfeld, Timm; Field, Michael E.; Fonarow, Gregg C.; Gillis, Anne M.; Granger, Christopher B.; Hammill, Stephen C.; Hlatky, Mark A.; Joglar, José A.; Kay, G. Neal; Matlock, Daniel D.; Myerburg, Robert J.; Page, Richard L. (2018). "2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death". Circulation. 138 (13). doi:10.1161/CIR.0000000000000549. ISSN 0009-7322.
  7. ↑ 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.
  8. ↑ Tsuchiya, Takeshi; Ashikaga, Keiichi; Honda, Toshihiro; Arita, Makoto (2002). "Prevention of Ventricular Fibrillation by Cilostazol, an Oral Phosphodiesterase Inhibitor, in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 13 (7): 698–701. doi:10.1046/j.1540-8167.2002.00698.x. ISSN 1045-3873.
  9. ↑ Abud, Atilio; Bagattin, Daniel; Goyeneche, Raul; Becker, Carlos (2006). "Failure of Cilostazol in the Prevention of Ventricular Fibrillation in a Patient with Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 17 (2): 210–212. doi:10.1111/j.1540-8167.2005.00290.x. ISSN 1045-3873.
  10. ↑ Matsui, Kazunori; Kiyosue, Tatsuto; Wang, Jin-Cheng; Dohi, Kazuhiro; Arita, Makoto (1999). Cardiovascular Drugs and Therapy. 13 (2): 105–113. doi:10.1023/A:1007779908346. ISSN 0920-3206. Missing or empty |title= (help)
  11. ↑ Miyazaki, Toshihisa; Mitamura, Hideo; Miyoshi, Shunichiro; Soejima, Kyoko; Aizawa, Yoshifusa; Ogawa, Satoshi (1996). "Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome". Journal of the American College of Cardiology. 27 (5): 1061–1070. doi:10.1016/0735-1097(95)00613-3. ISSN 0735-1097.
  12. ↑ Suzuki, Hiroshi; Torigoe, Katsumi; Numata, Osamu; Yazaki, Satoshi (2000). "Infant Case with a Malignant Form of Brugada Syndrome". Journal of Cardiovascular Electrophysiology. 11 (11): 1277–1280. doi:10.1046/j.1540-8167.2000.01277.x. ISSN 1045-3873.
  13. ↑ Obeyesekere MN, Klein GJ, Modi S, Leong-Sit P, Gula LJ, Yee R, Skanes AC, Krahn AD (December 2011). "How to perform and interpret provocative testing for the diagnosis of Brugada syndrome, long-QT syndrome, and catecholaminergic polymorphic ventricular tachycardia". Circ Arrhythm Electrophysiol. 4 (6): 958–64. doi:10.1161/CIRCEP.111.965947. PMID 22203660.
  14. ↑ Argenziano M, Antzelevitch C (June 2018). "Recent advances in the treatment of Brugada syndrome". Expert Rev Cardiovasc Ther. 16 (6): 387–404. doi:10.1080/14779072.2018.1475230. PMC 6330094. PMID 29757020.
  15. ↑ Kujime S, Sakurada H, Saito N, Enomoto Y, Ito N, Nakamura K, Fukamizu S, Tejima T, Yambe Y, Nishizaki M, Noro M, Hiraoka M, Sugi K (2017). "Outcomes of Brugada Syndrome Patients with Coronary Artery Vasospasm". Intern. Med. 56 (2): 129–135. doi:10.2169/internalmedicine.56.7307. PMC 5337455. PMID 28090040.
  16. ↑ Amin, A. S.; Klemens, C. A.; Meregalli, P. G.; Asghari-Roodsari, A.; de Bakker, J. M. T.; January, C. T.; Wilde, A. A. M.; Tan, H. L. (2010). "Fever-triggered ventricular arrhythmias in Brugada syndrome and type 2 long-QT syndrome". Netherlands Heart Journal. 18 (3): 165–169. doi:10.1007/BF03091755. ISSN 1568-5888.
  17. ↑ Antzelevitch C, Brugada P, Brugada J, Brugada R (January 2005). "Brugada syndrome: from cell to bedside". Curr Probl Cardiol. 30 (1): 9–54. doi:10.1016/j.cpcardiol.2004.04.005. PMC 1475801. PMID 15627121.
  18. ↑ Swe T, Dogar MH (2016). "Type 1 Brugada pattern electrocardiogram induced by hypokalemia". J Family Med Prim Care. 5 (3): 709–711. doi:10.4103/2249-4863.197295. PMC 5290792. PMID 28217615.
  19. ↑ . doi:10.1161/CIR.000000000000054. Missing or empty |title= (help)
  20. ↑ 20.0 20.1 Casado-Arroyo R, Berne P, Rao JY, Rodriguez-Mañero M, Levinstein M, Conte G, Sieira J, Namdar M, Ricciardi D, Chierchia GB, de Asmundis C, Pappaert G, La Meir M, Wellens F, Brugada J, Brugada P (August 2016). "Long-Term Trends in Newly Diagnosed Brugada Syndrome: Implications for Risk Stratification". J. Am. Coll. Cardiol. 68 (6): 614–623. doi:10.1016/j.jacc.2016.05.073. PMID 27491905.
  21. ↑ Probst V, Veltmann C, Eckardt L, Meregalli PG, Gaita F, Tan HL, Babuty D, Sacher F, Giustetto C, Schulze-Bahr E, Borggrefe M, Haissaguerre M, Mabo P, Le Marec H, Wolpert C, Wilde AA (February 2010). "Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry". Circulation. 121 (5): 635–43. doi:10.1161/CIRCULATIONAHA.109.887026. PMID 20100972.
  22. ↑ Priori SG, Gasparini M, Napolitano C, Della Bella P, Ottonelli AG, Sassone B, Giordano U, Pappone C, Mascioli G, Rossetti G, De Nardis R, Colombo M (January 2012). "Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry". J. Am. Coll. Cardiol. 59 (1): 37–45. doi:10.1016/j.jacc.2011.08.064. PMID 22192666.
  23. ↑ 23.0 23.1 Sroubek J, Probst V, Mazzanti A, Delise P, Hevia JC, Ohkubo K, Zorzi A, Champagne J, Kostopoulou A, Yin X, Napolitano C, Milan DJ, Wilde A, Sacher F, Borggrefe M, Ellinor PT, Theodorakis G, Nault I, Corrado D, Watanabe I, Antzelevitch C, Allocca G, Priori SG, Lubitz SA (February 2016). "Programmed Ventricular Stimulation for Risk Stratification in the Brugada Syndrome: A Pooled Analysis". Circulation. 133 (7): 622–30. doi:10.1161/CIRCULATIONAHA.115.017885. PMC 4758872. PMID 26797467.
  24. ↑ 24.0 24.1 Belhassen B, Rahkovich M, Michowitz Y, Glick A, Viskin S (December 2015). "Management of Brugada Syndrome: Thirty-Three-Year Experience Using Electrophysiologically Guided Therapy With Class 1A Antiarrhythmic Drugs". Circ Arrhythm Electrophysiol. 8 (6): 1393–402. doi:10.1161/CIRCEP.115.003109. PMID 26354972.
  25. ↑ 25.0 25.1 Zhang P, Tung R, Zhang Z, Sheng X, Liu Q, Jiang R, Sun Y, Chen S, Yu L, Ye Y, Fu G, Shivkumar K, Jiang C (November 2016). "Characterization of the epicardial substrate for catheter ablation of Brugada syndrome". Heart Rhythm. 13 (11): 2151–2158. doi:10.1016/j.hrthm.2016.07.025. PMID 27453126.
  26. ↑ Nademanee K, Veerakul G, Chandanamattha P, Chaothawee L, Ariyachaipanich A, Jirasirirojanakorn K, Likittanasombat K, Bhuripanyo K, Ngarmukos T (March 2011). "Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium". Circulation. 123 (12): 1270–9. doi:10.1161/CIRCULATIONAHA.110.972612. PMID 21403098.
  27. ↑ Antzelevitch C, Yan GX, Ackerman MJ, Borggrefe M, Corrado D, Guo J, Gussak I, Hasdemir C, Horie M, Huikuri H, Ma C, Morita H, Nam GB, Sacher F, Shimizu W, Viskin S, Wilde A (April 2017). "J-Wave syndromes expert consensus conference report: Emerging concepts and gaps in knowledge". Europace. 19 (4): 665–694. doi:10.1093/europace/euw235. PMC 5834028. PMID 28431071. Vancouver style error: initials (help)
  28. ↑ Somani R, Krahn AD, Healey JS, Chauhan VS, Birnie DH, Champagne J, Sanatani S, Angaran P, Gow RM, Chakrabarti S, Gerull B, Yee R, Skanes AC, Gula LJ, Leong-Sit P, Klein GJ, Gollob MH, Talajic M, Gardner M, Simpson CS (June 2014). "Procainamide infusion in the evaluation of unexplained cardiac arrest: from the Cardiac Arrest Survivors with Preserved Ejection Fraction Registry (CASPER)". Heart Rhythm. 11 (6): 1047–54. doi:10.1016/j.hrthm.2014.03.022. PMID 24657429.
  29. ↑ Delise P, Allocca G, Marras E, Giustetto C, Gaita F, Sciarra L, Calo L, Proclemer A, Marziali M, Rebellato L, Berton G, Coro L, Sitta N (January 2011). "Risk stratification in individuals with the Brugada type 1 ECG pattern without previous cardiac arrest: usefulness of a combined clinical and electrophysiologic approach". Eur. Heart J. 32 (2): 169–76. doi:10.1093/eurheartj/ehq381. PMC 3021386. PMID 20978016.
  30. ↑ Kusumoto, Fred M.; Bailey, Kent R.; Chaouki, Ahmad Sami; Deshmukh, Abhishek J.; Gautam, Sandeep; Kim, Robert J.; Kramer, Daniel B.; Lambrakos, Litsa K.; Nasser, Naseer H.; Sorajja, Dan (2018). "Systematic Review for the 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death". Circulation. 138 (13). doi:10.1161/CIR.0000000000000550. ISSN 0009-7322.
  31. ↑ Sieira J, Conte G, Ciconte G, de Asmundis C, Chierchia GB, Baltogiannis G, Di Giovanni G, Saitoh Y, Irfan G, Casado-Arroyo R, Juliá J, La Meir M, Wellens F, Wauters K, Van Malderen S, Pappaert G, Brugada P (August 2015). "Prognostic value of programmed electrical stimulation in Brugada syndrome: 20 years experience". Circ Arrhythm Electrophysiol. 8 (4): 777–84. doi:10.1161/CIRCEP.114.002647. PMID 25904495.
  32. ↑ Crotti L, Marcou CA, Tester DJ, Castelletti S, Giudicessi JR, Torchio M, Medeiros-Domingo A, Simone S, Will ML, Dagradi F, Schwartz PJ, Ackerman MJ (October 2012). "Spectrum and prevalence of mutations involving BrS1- through BrS12-susceptibility genes in a cohort of unrelated patients referred for Brugada syndrome genetic testing: implications for genetic testing". J. Am. Coll. Cardiol. 60 (15): 1410–8. doi:10.1016/j.jacc.2012.04.037. PMC 3624764. PMID 22840528.
  33. ↑ Risgaard B, Jabbari R, Refsgaard L, Holst AG, Haunsø S, Sadjadieh A, Winkel BG, Olesen MS, Tfelt-Hansen J (November 2013). "High prevalence of genetic variants previously associated with Brugada syndrome in new exome data". Clin. Genet. 84 (5): 489–95. doi:10.1111/cge.12126. PMID 23414114.

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