SPOTTER PRESENTATION
RAVI KANTH
SPOTTER
70 year old female
Hx
 Presented to Cardiology OP with h/o fever since one month.
 h/o breathlessness on exertio...
 O/E-
 CVS- trachea central.
- no deformities of chest.
- all peripheral pulses felt normally.
- JVP - normal
- S1,S2 – ...
 2D ECHO : No Rwma , Grade I Diastolic
Dysfunction, Normal LV systolic Function
EF- 56%
 Troponin I - NEGATIVE .
 Holte...
BRUGADA SYNDROME(BRS)
 BRUGADA SYNDROME(BrS) is characterized by ST segment
elevation in right precordial leads (V1 to V3...
BRUGADA SYNDROME: ECG
CHANGES
 First described in 1992 by Pedro and Joseph
Brugada.
What is It?
 Pseudo-RBBB
 ST Elevat...
GENETICS
- familial disease displaying an autosomal dominant mode of transmission
with incomplete penetrance.
- The first ...
ELECTROCARDIOGRAPHIC CHARACTERISTICS
 Repolarization as well as Depolarization abnormalities in the
absence of identifiab...
NORMAL VS RBBB VS BRUGADA
SYNDROME
 Normal
 RBBB
 QRS ≥120ms
 Terminal R wave in V1 (RSR1)
 Slurred S wave in I and V...
• Pseudo-
RBBB
(but no
slurred S in
V6)
• ST
Elevation
V1-V3
• T wave
inversion
= Brugada
Syndrome
ECG
ST Patterns in Brugada Syndrome
Type 1 “Coved Type”
• J wave ≥ 2mm convex
• ST segment descends
• Inverted T wave
Type 2 “...
3 Different Patterns
Feature Type 1 Type 2 Type 3
J wave ≥2mm ≥2mm ≥2mm
T wave Negative Positive or
biphasic
Positive
ST-T...
 The QT-interval is often within normal limits (in the absence of anti-
arrhythmic drug therapy), but may be prolonged. I...
CLINICAL FEATURES
 mean age at which symptoms first appear in affected individuals (males and
females) is in the third to...
DIFFERENTIAL DIAGNOSIS
 Right or left bundle branch block, left
ventricular hypertrophy
 Acute myocardial ischemia or
in...
DRUG CHALLENGE
 Intravenous administration of certain drugs may modify the ECG pattern.
 Ajmaline (1 mg/kg body weight; ...
 An increase in the J-wave amplitude of more than 2 mm without the
development of a type 1 configuration is also consider...
ELECTROPHYSIOLOGICAL STUDIES
 Electrophysiological studies (EPS) may be helpful in risk
stratification and in some cases ...
MOLECULAR GENETICS
 Genetic linkage analysis may be of great help in arriving at a
definitive diagnosis.
 Limitations ;
...
HOW TO DIAGNOSE
 Brugada syndrome should be strongly considered in the following
cases:
 Appearance of a type 1 ST segme...
 Appearance of type 2 ST-segment elevation (saddleback type) in
more than one right precordial lead under baseline condit...
 Appearance of type 3 ST segment elevation in more than one lead
under baseline conditions with conversion to type 1 foll...
MANAGEMENT
 Symptomatic individuals;
 ICD: ICD implantation in BS patients who have survived cardiac
arrest (Class 1) or...
 Asymptomatic individuals
 While there is no dispute about the management of symptomatic
patients of BS, the management ...
DRUGS TO BE AVOIDED
 Antiarrhythmic drugs
 Ajmaline
 Flecainide
 Pilsicainide
 Procainamide
 Propafenone
 Psychotro...
DRUGS PREFERABLY AVOIDED
 Antiarrhythmic drugs
 Amiodarone
 Cibenzoline
 Disopyramide
 Lidocaine*
 Propranolol
 Ver...
THANK U
Brugada
Brugada
Brugada
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Brugada

  1. 1. SPOTTER PRESENTATION RAVI KANTH
  2. 2. SPOTTER 70 year old female Hx  Presented to Cardiology OP with h/o fever since one month.  h/o breathlessness on exertion class –II since one month.  Patient referred from a local hospital in view of ECG changes  h/o giddiness occasionally  no h/o syncopal attack . PMHx  h/o HTN on treatment with amlodipine 5 mg.  No h/o similar complaints in the past.  No h/o DM,CAD,CVA,CKD.
  3. 3.  O/E-  CVS- trachea central. - no deformities of chest. - all peripheral pulses felt normally. - JVP - normal - S1,S2 – normal. - no additional sounds. Other systems  Resp System- Wheeze +  CNS – tremors present
  4. 4.  2D ECHO : No Rwma , Grade I Diastolic Dysfunction, Normal LV systolic Function EF- 56%  Troponin I - NEGATIVE .  Holter analysis – no evidence of Arrythmias and Pauses  RFT- 28/0.96  SE- 140/4.2  Sr.ca+ - 9.0  Sr.mg+ - 2.0
  5. 5. BRUGADA SYNDROME(BRS)  BRUGADA SYNDROME(BrS) is characterized by ST segment elevation in right precordial leads (V1 to V3), unrelated to ischemia, electrolyte disturbances or obvious structural heart disease, was reported as early as 1953, but first described as a distinct clinical entity associated with a high risk of sudden cardiac death in 1992.  male predominance (8:1 ratio of males:females) and the appearance of arrhythmic events at an average age of 40 years (range: 1 to 77 years).
  6. 6. BRUGADA SYNDROME: ECG CHANGES  First described in 1992 by Pedro and Joseph Brugada. What is It?  Pseudo-RBBB  ST Elevation V1-V3
  7. 7. GENETICS - familial disease displaying an autosomal dominant mode of transmission with incomplete penetrance. - The first gene was identified in 1998 as being the cardiac sodium channel gene (SCN5A). - Recently the second gene for BrS has been reported in a single large family harboring a mutation in the GPD1-L gene.
  8. 8. ELECTROCARDIOGRAPHIC CHARACTERISTICS  Repolarization as well as Depolarization abnormalities in the absence of identifiable structural cardiac abnormalities or other conditions or agents known to lead to ST-segment elevation in the right precordial leads.  The diagnosis of BrS is complicated by the intermittent nature of the electrocardiogram (ECG) pattern.
  9. 9. NORMAL VS RBBB VS BRUGADA SYNDROME  Normal  RBBB  QRS ≥120ms  Terminal R wave in V1 (RSR1)  Slurred S wave in I and V6  Brugada Syndrome
  10. 10. • Pseudo- RBBB (but no slurred S in V6) • ST Elevation V1-V3 • T wave inversion = Brugada Syndrome ECG
  11. 11. ST Patterns in Brugada Syndrome Type 1 “Coved Type” • J wave ≥ 2mm convex • ST segment descends • Inverted T wave Type 2 “Saddle back” • J wave ≥ 2mm • ST segment ≥1mm • Upright or biphasic T Type 3 “Saddle back” • J wave ≥2mm • ST segment <1mm • Positive T wave
  12. 12. 3 Different Patterns Feature Type 1 Type 2 Type 3 J wave ≥2mm ≥2mm ≥2mm T wave Negative Positive or biphasic Positive ST-T Coved Saddleback Saddleback ST Segment Terminal Portion Gradual descent Elevated ≥1mm Elevated <1mm
  13. 13.  The QT-interval is often within normal limits (in the absence of anti- arrhythmic drug therapy), but may be prolonged. In the initial series described by Brugada and Brugada (1992), three out of six males had a QTc 440 ms.  Conduction disorders vary from non-specific to specific for any given part of the conduction system.
  14. 14. CLINICAL FEATURES  mean age at which symptoms first appear in affected individuals (males and females) is in the third to fourth decade.  syncope or sudden cardiac death is the only symptom in patients with Brugada syndrome.  Monitoring of such patients has revealed rapid polymorphic VTs as the underlying cause.  Clinical reports indicate that sudden death in Brugada patients most commonly occurs during sleep, in particular during the early morning hours.  higher than normal incidence of supraventricular tachyarrhythmias, including atrial and AV reentrant tachycardia in the Brugada population.  Family history is often positive for sudden cardiac death at a young age.
  15. 15. DIFFERENTIAL DIAGNOSIS  Right or left bundle branch block, left ventricular hypertrophy  Acute myocardial ischemia or infarction.  Acute myocarditis  Right ventricular ischemia or infarction  Dissecting aortic aneurysm  Acute pulmonary thromboemboli  Heterocyclic antidepressant overdose  Duchenne muscular dystrophy  Friedreich’s ataxia  Hypercalcemia  Hyperkalemia  Cocaine intoxication  Arrhythmogenic right ventricular dysplasia/cardiomyopathy  LQTS type 3.  Early repolarization syndrome
  16. 16. DRUG CHALLENGE  Intravenous administration of certain drugs may modify the ECG pattern.  Ajmaline (1 mg/kg body weight; 10 mg/ min), flecainide (2 mg / kg, max 150 mg; in 10 min) and procainamide (10 mg / Kg; 100 mg/min) exaggerate the ST-segment elevation or unmask it when it is initially absent.  Drug administration should be stopped when the test is positive and/or when ventricular arrhythmias, including ventricular premature complexes, are evident or when significant QRS widening is observed.  In the case of a negative baseline ECG, a J-wave amplitude of >2 mm in lead V1 and/or V2 and/or V3 with or without RBBB is considered positive.  In patients with type 1 ECGs, drug testing is not of additional diagnostic value.  In patients with types 2 and 3 ECGs, the test is recommended for the purpose of clarifying the diagnosis.  Conversion of types 2 or 3 ECG to type 1 is considered positive.
  17. 17.  An increase in the J-wave amplitude of more than 2 mm without the development of a type 1 configuration is also considered significant.  Conversion of type 3 ECG into type 2 is considered inconclusive  Monitoring is recommended until the ECG has normalized (plasma half-lives of the different drugs are: flecainide 20 h, procainamide 3– 4 h, ajmaline inactivated within a few minutes). Serious ventricular arrhythmias, including VF, may occur during the test.  Immediate discontinuation of the drug is required and isoproterenol infusion might be needed to treat the arrhythmias.
  18. 18. ELECTROPHYSIOLOGICAL STUDIES  Electrophysiological studies (EPS) may be helpful in risk stratification and in some cases in establishing the diagnosis.  In VF survivors, EPS may be of little or no diagnostic value.
  19. 19. MOLECULAR GENETICS  Genetic linkage analysis may be of great help in arriving at a definitive diagnosis.  Limitations ;  it may take weeks to months.  there are few diagnostic laboratories capable of screening for the disease.  only a small fraction of patients can be successfully genotyped at this time (estimates vary between 10 and 30%)
  20. 20. HOW TO DIAGNOSE  Brugada syndrome should be strongly considered in the following cases:  Appearance of a type 1 ST segment elevation (coved type1) in more than one right precordial lead (V1–V3), in the presence or absence of a sodium channel blocker, and one of the following: documented ventricular fibrillation, self terminating polymorphic ventricular tachycardia, a family history of SCD (<45 years), coved type ECGs in family members, electrophysiological inducibility, syncope or nocturnal agonal respiration.  There should be no other factor(s) accounting for the ECG abnormality.
  21. 21.  Appearance of type 2 ST-segment elevation (saddleback type) in more than one right precordial lead under baseline conditions with conversion to type 1 following challenge with a sodium channel blocker is considered .  Drug-induced ST-segment elevation to a value greater than 2 mm should raise the possibility of Brugada syndrome, when one or more clinical criteria are present .  Based on limited knowledge at present, a patient with a negative drug test (i.e. no change in the ST-segment in response to a sodium channel blocker) is unlikely to have Brugada syndrome; drug induced ST-elevation to <2 mm is considered inconclusive.
  22. 22.  Appearance of type 3 ST segment elevation in more than one lead under baseline conditions with conversion to type 1 following challenge with a sodium channel blocker is considered equivalent to case 1 above and should be screened accordingly.  Drug-induced conversion of type 3 to type 2 ST segment elevation is considered inconclusive.
  23. 23. MANAGEMENT  Symptomatic individuals;  ICD: ICD implantation in BS patients who have survived cardiac arrest (Class 1) or have a history of syncope and documented ventricular arrhythmia (Class 2A).  Drugs:  Quinidine.  Isoprenaline infusion increases Ca+ current and is helpful in emergency treatment of arrhythmic storms in BS.
  24. 24.  Asymptomatic individuals  While there is no dispute about the management of symptomatic patients of BS, the management of asymptomatic patients is a highly controversial topic.  The consensus report recommends EPS in asymptomatic patients with spontaneous Type 1 ECG and ICD implantation be recommended if the EPS is positive (Class 2A) and a close follow up, if the EP is negative.  If the patient is asymptomatic and BS ECG changes are brought out only after a drug challenge, it recommends EPS and ICD implantation if EPS is positive, (Class 2B indication)
  25. 25. DRUGS TO BE AVOIDED  Antiarrhythmic drugs  Ajmaline  Flecainide  Pilsicainide  Procainamide  Propafenone  Psychotropic drugs  Amitriptyline  Clomipramine  Desipramine  Lithium  Oxcarbazepine  Anesthetics / analgesics  Bupivacaine  Procaine  Propofol  Other substances  Acetylcholine  Alcohol  Cannabis  Cocaine
  26. 26. DRUGS PREFERABLY AVOIDED  Antiarrhythmic drugs  Amiodarone  Cibenzoline  Disopyramide  Lidocaine*  Propranolol  Verapamil  Vernakalant  Other substances  Metoclopramide  Terfenadine/ Fexofenadine  Psychotropic drugs  Carbamazepine*  Doxepin  Fluoxetine  Fluvoxamine  Imipramine  Lamotrigine  Phenytoin  Anesthetics / analgesics  Ketamine  Tramadol
  27. 27. THANK U

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