Right bundle branch block pathophysiology: Difference between revisions
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{{Right bundle branch block}} | {{Right bundle branch block}} | ||
{{CMG}} {{AE}} {{CZ}} | |||
Please help WikiDoc by adding more content here. It's easy! Click [[Help:How_to_Edit_a_Page|here]] to learn about editing. | Please help WikiDoc by adding more content here. It's easy! Click [[Help:How_to_Edit_a_Page|here]] to learn about editing. | ||
==Pathophysiology== | ==Pathophysiology== | ||
Three types of [[RBBB]] have been identified based upon electrophysiologic studies. | Three types of [[RBBB]] have been identified based upon electrophysiologic studies. | ||
In each of the three types of RBBB, the surface ECG pattern remains the same. | In each of the three types of RBBB, the surface ECG pattern remains the same. | ||
1. Proximal, or central, RBBB: | |||
This type of conduction defect occurs when the conduction block is located just distal to the bundle of His in the superior aspect of the right bundle branch. This type of block occurs when the proximal bundle is injured during surgery for an inlet or membranous [[ventricular septal defect]] ([[VSD]]). | This type of conduction defect occurs when the conduction block is located just distal to the bundle of His in the superior aspect of the right bundle branch. This type of block occurs when the proximal bundle is injured during surgery for an inlet or membranous [[ventricular septal defect]] ([[VSD]]). | ||
2. Interruption between the proximal and distal aspects of the right bundle branch: | |||
This type of right bundle branch block occurs when the impulse is interrupted between the proximal and distal aspects of the right bundle branch. This type of bright bundle branch block is most commonly observed after surgical division of the moderator band. | This type of right bundle branch block occurs when the impulse is interrupted between the proximal and distal aspects of the right bundle branch. This type of bright bundle branch block is most commonly observed after surgical division of the moderator band. | ||
3. Distal RBBB: | |||
This form is observed when distal ramifications of the right bundle are disrupted during right ventriculotomy or resection of muscle bundles in the right ventricular outflow tract. | This form is observed when distal ramifications of the right bundle are disrupted during right ventriculotomy or resection of muscle bundles in the right ventricular outflow tract. | ||
===Genetics === | ===Genetics=== | ||
There can be familial cases of [[right bundle branch block]] such as that observed in 4 Lebanese families and the abnormality was mapped to chromosome 19. | There can be familial cases of [[right bundle branch block]] such as that observed in 4 Lebanese families and the abnormality was mapped to chromosome 19. | ||
There is a subset of patients with [[Brugada syndrome]] who have mutations in SCN5A, the gene encoding for the voltage-gated cardiac sodium channel. | There is a subset of patients with [[Brugada syndrome]] who have mutations in SCN5A, the gene encoding for the voltage-gated cardiac sodium channel. | ||
===Associated | ===Associated Syndromes=== | ||
*[[Duchenne muscular dystrophy]] | *[[Duchenne muscular dystrophy]] | ||
*[[Myotonic dystrophy]] Other ECG findings include [[first-degree AV block]], [[left anterior fascicular block]], and intraventricular conduction delay. Patients may have arrhythmias and/or [[Stokes-Adams attacks]]. | *[[Myotonic dystrophy]] Other ECG findings include [[first-degree AV block]], [[left anterior fascicular block]], and intraventricular conduction delay. Patients may have arrhythmias and/or [[Stokes-Adams attacks]]. | ||
*[[Kearns-Sayre Syndrome]] | *[[Kearns-Sayre Syndrome]] | ||
*[[Brugada syndrome]]: This syndrome is due to a channelopathy mediated by the SCN5A gene. It is important to note that the RBBB pattern seen in patients with this syndrome is not actually RBBB but is instead due to a repolarization abnormality. In this syndrome, the ECG shows ST-segment elevation in leads V1-V3. [[Cocaine]] consumption and / or the use of the antiarrhythmic [[propafenone]] may unmask the ECG findings of [[Brugada syndrome]]. | *[[Brugada syndrome]]: This syndrome is due to a channelopathy mediated by the SCN5A gene. It is important to note that the RBBB pattern seen in patients with this syndrome is not actually RBBB but is instead due to a repolarization abnormality. In this syndrome, the ECG shows ST-segment elevation in leads V1-V3. [[Cocaine]] consumption and / or the use of the antiarrhythmic [[propafenone]] may unmask the ECG findings of [[Brugada syndrome]]. | ||
===Pseudo Right Bundle Branch Block=== | ===Pseudo Right Bundle Branch Block=== | ||
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==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Needs overview]] | |||
[[Category:Needs content]] | [[Category:Needs content]] | ||
[[Category:Electrophysiology]] | [[Category:Electrophysiology]] | ||
Revision as of 15:29, 6 February 2013
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]
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Pathophysiology
Three types of RBBB have been identified based upon electrophysiologic studies.
In each of the three types of RBBB, the surface ECG pattern remains the same.
1. Proximal, or central, RBBB: This type of conduction defect occurs when the conduction block is located just distal to the bundle of His in the superior aspect of the right bundle branch. This type of block occurs when the proximal bundle is injured during surgery for an inlet or membranous ventricular septal defect (VSD).
2. Interruption between the proximal and distal aspects of the right bundle branch: This type of right bundle branch block occurs when the impulse is interrupted between the proximal and distal aspects of the right bundle branch. This type of bright bundle branch block is most commonly observed after surgical division of the moderator band.
3. Distal RBBB: This form is observed when distal ramifications of the right bundle are disrupted during right ventriculotomy or resection of muscle bundles in the right ventricular outflow tract.
Genetics
There can be familial cases of right bundle branch block such as that observed in 4 Lebanese families and the abnormality was mapped to chromosome 19. There is a subset of patients with Brugada syndrome who have mutations in SCN5A, the gene encoding for the voltage-gated cardiac sodium channel.
Associated Syndromes
- Duchenne muscular dystrophy
- Myotonic dystrophy Other ECG findings include first-degree AV block, left anterior fascicular block, and intraventricular conduction delay. Patients may have arrhythmias and/or Stokes-Adams attacks.
- Kearns-Sayre Syndrome
- Brugada syndrome: This syndrome is due to a channelopathy mediated by the SCN5A gene. It is important to note that the RBBB pattern seen in patients with this syndrome is not actually RBBB but is instead due to a repolarization abnormality. In this syndrome, the ECG shows ST-segment elevation in leads V1-V3. Cocaine consumption and / or the use of the antiarrhythmic propafenone may unmask the ECG findings of Brugada syndrome.
Pseudo Right Bundle Branch Block
Brugada syndrome: This syndrome is due to a channelopathy mediated by the SCN5A gene. It is important to note that the RBBB pattern seen in patients with this syndrome is not actually RBBB but is instead due to a repolarization abnormality. It is for this reason that the RBBB like pattern in Brugada syndrome is referred to as a 'pseudo right bundle branch block.' In this syndrome, the ECG shows ST-segment elevation in leads V1-V3. Cocaine consumption and / or the use of the antiarrhythmic propafenone may unmask the ECG findings of Brugada syndrome.