1. Ventricular Tachycardia
Mohammad Hajjiri, MD FACC FHRS
Director, Interventional Electrophysiology
OhioHealth Medical Center, Mansfield, OH
Assistant Professor of Medicine
Ohio University

2. Wide Spectrum of Symptoms
Definitions
VT: ³3 consecutive ventricular ³100 bpm
Nonsustained VT: VT lasting ³3 beats, <30 secs
and not requiring intervention for termination
Sustained VT: VT lasting ³30 secs or requiring intervention for termination.
Wide Spectrum of Symptoms
•Asymptomatic
•Palpitations
•Shortness of breath
•Syncope
•Sudden cardiac death

3. Mechanisms of VT Triggered activity Increased automaticity Reentry
EADs
DADs
increased automaticity
AIVR
idiopathic VT
TdP
idiopathic VT
Reentry
MMVT (in SHD)
V Fib

4. Specific VT Syndromes Structural Heart Disease (SHD)
Ischemic or Nonischemic CM
Arrhythmogenic RV Dysplasia/CM (ARVD/ARVC)
HCM
Monomorphic VT a/w SHD –often a/w scar (prior MI, sarcoid, ARVD, CM), ­ risk of SD (esp. c LV dysfunction)
No significant SHD
Normal Hearts – “Idiopathic” VTs
Outflow Tract VTs
LV fascicular VTs
PMVT - TdP/LQTS, CPVT
Brugada’s Syndrome

5. What is the most likely cause of his arrhythmia?
J.C. is a 66 year old man with prior MI, who presented to the ER complaining of palpitations and syncope. On exam his BP was 90/70. His 12 lead ECG is shown below.
What is the most likely cause of his arrhythmia?
Atrial tachycardia with LBBB aberration
Inferior wall ventricular tachycardia
Scar-related ventricular tachycardia
Antidromic AV reciprocating tachycardia

8. exceptions amiodarone)
Secondary vs Primary Prevention
of Sudden Cardiac Death
Secondary Prevention of SCD
Patient has had a sustained VT/VF/SCD event
Prevention of recurrent VT/VF/SCD
Main treatment is ICD (AVID1, CASH2, CIDS3)
Adjunctive treatment
Antiarrhythmic drugs (potential ­mortality with empiric use; possible
exceptions amiodarone)
Catheter ablation
Endocardial resection/aneurysmectomy
Primary Prevention of SCD
Patient has not yet had a sustained VT/VF event
Risk stratification (low LVEF, NSVT)
1AVID Investigators NEJM 1997;337: ; 2Kuck et al Circulation 2000;102:748-54; 3Connolly et al Circulaton 2000;101:

9. Case 2
50 year old male presents with rapid palpitations and nearsyncope playing basketball. FH Father died in an MVA in his 40s. His ECG on presentation is treated with cardioversion.

10. Case 2 - continued ECG after cardioversion is shown. Echo shows normal
LV function, mild RV enlargement.
Diagnostic procedures are performed. What are likely results?
EP study shows VT from the RVOT, which is ablated
EP study shows an accessory pathway, which is ablated
SAECG is normal
MRI shows fibrofatty infiltration of the RV

11. ARVD Characteristics Abnormal SAECG Epsilon wave
Anterior precordial T wave abnormalities
Epsilon wave
From: McRae et al, CCJM 68:459-67,
2001
VT in ARVD
•Fibrofatty infiltration
•RV dyskinetic bulges, dilatation
Typically LB, Non-RVOT
morphology
T.H.

12. Revised Task Force Criteria for Clinical Diagnosis of ARVC/D
Definite: 2 Major or 1 Major+2 Minor or 4 Minor*
Possible: 1 major or 2 minor*
Borderline: 1 major and 1 minor or 3 minor*
*Minor criteria from different categories
Global or regional dysfunction / structural changes
•Echo, MRI, RV angiography
Tissue Characterization
•Endomyocardial biopsy
Repolarization Abnormalities
•ECG precordial T wave inversion
Depolarization/Conduction Abnormalities
•ECG Epsilon wave, terminal QRS
•SAECG late potential
Arrhythmias
•LBBB morphology ventricular arrhythmias
Family History
•Relatives with ARVD/C
•Identified mutation
•Premature SD
Marcus et al Circulation 2010;121:

13. ARVD Therapy ICD – for pts meeting strict dx criteria
Cardiac arrest, spontaneous or inducible rapid VT, depressed LV
function
Familial ARVD at high risk of SD
Drug therapy and catheter ablation – adjunctive for
increased VT frequency
Antiarrhythmic drugs
Sotalol, Amiodarone, beta blockers, IA, III
Catheter Ablation
adjunctive, multiple VT circuits and progressive disease common (acute success 60-90%, recurrence in 1 study)
Avoid competitive athletics, long-distance biking, running, swimming, weight training
Screen all 1st degree family members

14. Hypertrophic Cardiomyopathy
Most common genetic CVD (1:500)
•Autosomal dominant transmission
•Mutations in genes encoding components of the cardiac sarcomere (e.g. MYBPC3, MYH7, TNNT2, TNNI3)
•Genetically, phenotypically heterogeneous
Highly variable clinical course and expression
•Compatible with normal longevity
SCD risk
•Most frequent in asx young patients (<35 yrs)
•May occur in young athletes
•May be the initial presentation of HCM
Symptoms can result from diastolic dysfcn, LVOT obstruction
Risk stratification for SCD - based on clinical rather than genetic factors

15. -Septal myectomy (¯ risk)
Indications for ICDs in HCM
Gersh et al ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy. Circulation, 2011.
For 1o prevention:
5 established risk factors Major:
FH SCD due to HCM
LV > 30 mm
Recent unexplained syncope
•Minor:
NSVT
Abnl BP response
*Other established and emerging SCD risk modifiers:
-LVOT obstruction ³30 mmHg
-Septal myectomy (¯ risk)
-LGE on MRI
-LV apical aneurysm
[low PPV]

16. Case 3
34 y/o man has intermittent palpitations. Baseline ECG and echo are normal. He has failed b blockers and Ca++ channel blockers. During stress testing the following ECG is observed. He prefers to avoid antiarrhythmic drugs.
Which of the following is the most optimal approach?
Implant an ICD
Amiodarone 200 mg/day
Flecainide 100 mg bid
EP testing and catheter ablation

17. Outflow Tract VT (e.g. RVOT, LVOT, aortic cusp)
“Idiopathic” VT in NORMAL HEARTS
Outflow Tract VT (e.g. RVOT, LVOT, aortic cusp)
Idiopathic LV (fascicular) VT

18. Outflow Tract VTs RVOT, LVOT, Aortic Cusp VT MORPHOLOGY
LB inferior axis
Tall R waves 2, 3, and F
Relatively narrow QRS

19. Repetitive Monomorphic Idiopathic VT
VT pattern
Frequent PVCs
Repetitive runs of NSVT
Sustained VT

20. Outflow Tract Tachycardias
Clinical Characteristics
No SHD, but may have tachycardia-induced CM
Asymptomatic or symptomatic
A/w sympathetic tone
Stress, exercise, high catecholamine states, caffeine, premenstrual period, menopause, gestation
Adenosine or vagal maneuvers may suppress
Treatment
b-blockers, CCB, 1C AADs
Catheter ablation – success rate ~90%+

21. Idiopathic LV or Fascicular VT (ILVT)
VT in normal hearts #2
Idiopathic LV or Fascicular VT (ILVT)
Paroxysmal VT
Reentry in Ca++ dependent Purkinje-fascicular fibers
Potentiated, though not typically precipitated by exercise
RBBB morphology
– Most common RB/LSA (LAD, - in II, aVF)
No definite SHD; normal SAECG
Terminated or suppressed by verapamil or diltiazem
– NOT usually adenosine sensitive
Ablation: sites where the VT is preceded by a Purkinje/fascicular potential
– Most common site – LV inferior septum (mid-apical)

22. LV Fascicular VT I
aVF
V1 V6
MAP d MAPp

23. Long QT Syndrome Inherited ion channel disorder (Na and K channels)
Autosomal dominant inheritance most common
Places patient at risk for TdP
PMVT associated with long QT
Mechanism: early afterdepolarizations
May cause syncope and sudden death
Clinical features can vary with genetic mutation
Variable expression of severity
Syncope and SD have d frequency in adolescence

24. Long QT Syndrome: How do I diagnose it?
QTc is prolonged when ≥ 0.45 in men and ≥ 0.46 in women and children (<2.5%); <1% have QTc >0.48
Modified Diagnostic Criteria for LQTS
•ECG Findings Points
•Clinical History Points
•Syncope*
•QTc > 480 ms
•QTc ms
•QTc: ms (m) 3 2 1
–with stress
–without stress
•Congenital deafness 2 1
0.5
•QTc 4th min recovery from
exercise stress test ³480ms 1
•Torsade de pointes* 2
•T wave alternans
•Notched T in 3 leads
•Heart rate < 2% for age
0.5
Family History Points
•Positive family hx definite LQTS 1
•Sudden death <30 yo among
immediate family members 0.5
*mutually exclusive
Probability of LQTS: £1 pt: LOW pts: INTERMEDIATE ³3.5 pts: HIGH
Schwartz et al. Circ 88:782,1993. Keating. Circ 85:1973, 1992.
Schwartz et al. Circ 124:2181-4, 2011.

25. Diagnosis of LQTS LQTS is diagnosed in the presence of:
LQTS risk score ³3.5 in the absence of a 2o cause for QT prolongation, and/or
Unequivocally pathogenic mutation in a LQTS gene, or
QTc ³ 500 ms in repeated 12-lead ECGs in the absence of a 2o cause for QT prolongation
LQTS can be diagnosed in the presence of:
QTc ms on repeated 12-lead ECGs in a patient with unexplained syncope in the absence of a 2o cause for QT prolongation and absence of a pathogenic mutation
Priori SG, et al. HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and Management of Patients with Inherited Primary Arrhythmia Syndromes. Heart Rhythm 2013

26. LQTS Clinical characteristics Gene Ion current change Frequency LQT1
Exercise, swimming; broad T wave
KCNQ1
¯IKs a subunit
30-35%
LQT2
Auditory, startle, emotion;
notched/bifid or low T wave
KCNH2 (HERG);
KCNE2
¯IKr a subunit
25-40%*
LQT3
Sleep; bradycardia; long flat ST
SCN5A
­Ina a subunit
5-10%*
T Wave Morphology in LQTS
Roden et al Circulation 1996;94:
Other forms, genotypes for LQTS - rare

27. Long QT Syndrome: Evaluation
History (QT prolonging drugs etc)
ECG
Holter or event monitor (TdP)
Exercise testing (diagnostic value not well
defined)
No role for EP testing
Genetic testing

28. Long QT Syndrome: Genetic Testing
Recommended for
Patient with a strong suspicion for LQTS based on hx, FH, ECG
Asx pt with idiopathic QTc>480 ms (prepuberty) or >500 ms (adults)
1st degree and other appropriate relatives following identification of the LQTS-causative mutation in an index
case
Considered for
Asx pt c otherwise idiopathic QTc values >460 ms
(prepuberty) or >480 ms (adults) on serial 12-lead ECGs
Index case of drug-induced TdP
HRS/EHRA Expert Consensus Statement 2011

29. Prognosis: Risk Factors for Cardiac Events
Long QT Syndrome
Prognosis: Risk Factors for Cardiac Events
Length of QTc
LQT1 and LQT2 with long QTc; males LQT3
Adolescence
Males before puberty
Females during adulthood
Pregnancy (and postpartum period – esp LQT2)
Family history
Syncope
Resuscitated cardiac arrest
•Genotype (e.g. transmembrane, pore mutations,
LQT3 males, others)

30. Other Recommendations
ICDs in LQTS
Beta blockers recommended
Asx with QTc³470ms and/or
Sx c syncope or VT/VF
Beta blockers can be useful
Asx with QTc£470ms
Na channel blockers can be useful
As add-on therapy, for LQT3 patients with QTc>500 ms, who shorten QTc by >40ms after acute oral drug load with one of these compounds
[Possible exceptions: 2 or more gene mutations and QTc>500 ms, including JLN c congenital deafness, Timothy syndrome (LQT8), very high risk patients, esp with contraindication to a beta-blocker, QTc>600 ms, certain mutation locations]
Priori SG, et al. HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and
Management of Patients with Inherited Primary Arrhythmia Syndromes. Heart Rhythm 2013

31. Catecholaminergic Polymorphic VT (CPVT)
PMVT not associated with long QT
– Evaluate/treat ischemia or electrolyte abnormality
Exercise-induced PMVT, bidirectional VT
Mutations in genes controlling intracellular Ca++
•CPVT1: Ryanodine release channel (RYR2)
•CPVT2: Calsequestrin (CASQ2)
•Other loci postulated
CPVT: Bidirectional VT degenerating to VF
Priori et al Circulation 2002; 106:69-74
PMVT degenerating to VF

32. CPVT - Treatment
Limit/avoid: competitive sports, strenuous exercise, exposure to stressful environments
Beta blockers: recommended for all symptomatic pts with dx of CPVT [effective ~60%]; can be useful in carriers of a pathogenic CPVT mutation
Less/Not effective: Calcium channel blockers, Na
channel blockers, Amiodarone
Flecainide can be a useful addition to beta blockers
Re-exercise stress test
ICDs for cardiac arrest, recurrent syncope or PM/bidirectional VT despite medical mgt

33. Brugada Syndrome RBBB with STE right precordium Risk for VFib and SCD
Genetics: Loss of function mutations in Na+ channel
gene (SCN5A) and others
Male predominance, SE Asia
AV conduction abnormalities and atrial arrhythmias
common

34. Brugada Syndrome - Diagnosis
ECG changes may be variable:
3 types (I – III) based on the ST, T wave morphology in right precordial leads
(V1 – V3)
May be unmasked by Na+ channel blockers (proc., flec., etc)
Brugada syndrome DIAGNOSED with:
Type 1 ECG: ³2 mm coved STE in ³1 V1-V2 leads positioned in the 2nd, 3rd, or 4th intercostal space (spontaneous or induced) OR
Type 1 ECG
Type 2 (saddleback STE) or type 3 (£ 1mm STE) ECG when a provocative drug (IV class 1 Na ch blocker AAD) induces a Type 1 ECG
Type 2 (saddleback STE)
Type 1 ECG
Type 3 (£ 1mm STE)

35. Brugada Syndrome Management
Lifestyle changes:
•Avoid drugs that induce/aggravate STE - e.g. Na channel blockers
– Brugadadrugs.org
•Avoid excessive alcohol
•Immediately treat fever with antipyretics
No effective drug therapy identified - Amiodarone and b
blockers inadequate
Quinidine (Inhibits Ito; may prevent sxs, ECG features)
•Can be useful with arrhythmic storms, ICD contraindication/refusal
•May be considered in asx pts with BrS and spontaneous Type 1
ECG
Isoproterenol can be useful in arrhythmic storms
Priori SG, et al. HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and
Management of Patients with Inherited Primary Arrhythmia Syndromes. Heart Rhythm 2013

36. Brugada Syndrome: ICDs
Controversies:
Prognostic value of EPS
ICD in asymptomatic patients
ICD is NOT indicated in asx BrS with drug- induced type 1 ECG and on basis of FH alone
Priori SG, et al. HRS/EHRA/APHRS Expert Consensus Statement on the Diagnosis and
Management of Patients with Inherited Primary Arrhythmia Syndromes. Heart Rhythm 2013

37. Ventricular Fibrillation / Sudden Cardiac Death
Definition: “ Unexpected death within one hour of the onset of symptoms.”
•Results from multiple reentrant wavelets in the ventricle
•Usually occurs in setting of structural heart disease
•80% - coronary artery disease
•15% - cardiomyopathy
•5% - structurally normal heart
•300,000 episodes per year, > 50% all CV deaths
•Rapidly lethal if not defibrillated
•<5% of patients are successfully resuscitated

38. Implantable Cardioverter-Defibrillators
Reduce risk of sudden death
Shown to be effective in:
Secondary prevention of SCD
Primary prevention of SCD if cardiomyopathy
Also used frequently in long QT syndrome,
HCM, ARVD, Brugada syndrome

39. Catheter Ablation of Ventricular Tachycardia
Antiarrhythmic Drugs
Only BBs lower SCD risk
Risks of proarrhythmia are very real
Amiodarone has a neutral impact on mortality
EP guided AAD therapy is not effective
AADs may ¯ the frequency of VT episodes
If SCD risk is present, best to avoid AADs or use as an adjunct to ICD
Catheter Ablation of Ventricular Tachycardia
VT Type
Idiopathic ventricular tachycardia (RVOT,
fascicle)
Efficacy
> 90 %
Bundle branch reentry
> 95%
CAD / prior MI %
Primary cardiomyopathy
< 50%
Ventricular fibrillation ?

40. Management of Asymptomatic Ventricular Arrhythmias
Minimal / No SHD
+SHD (low LVEF, post MI)
Not associated with ­d mortality
Treatment usually not indicated
Associated with ­d mortality
Only beta blockers ­ survival
Also ICDs if meet MADIT or
SCDHeFT profile
Inherited CM/Arrhythmia syndrome (e.g. HCM, LQTS, ARVD, Brugada)
ICDs often recommended if fit a “high risk” profile
Do not forget beta blockers