Electrophysiology Flashcards
What is the prevalence of congenital LQTS?
1 in 2000
Describe Jervell Lange Nielsen Syndrome.
Autosomal recessive congenital LQTS with associated sensorineural deafness. Associated with malignant course, cardiac events precipitated by emotional or physical stress.
What is the rate of SCD in those with Lange jervell Nielsen Syndrome?
25%
In those with jervell Lange Nielsen Syndrome, what were the event rates for those aged 1, 3 and 18?
15, 50, 90%
How does pregnancy influence risk of events in those with LQTS (particularly LQTS2)
Higher risk of events 6-9 months post partum.
Describe pause dependence.
Duration of repolarisation is dependent on the duration of the preceding RR interval
What are the external triggers that classically exacerbate LQTS?
LQT1 - exercise, swimming, diving
LQT2 - noise, emotion
What preceding symptoms prior to syncope/seizure is highly suggestive of LQTS?
Emotional stress
Physical exertion
What types of LQTS have triggered events during sleep?
LQT2 and 3
Describe Andersen Tawil Syndrome.
Autosomal dominant condition characterised by episodes of potassium related paralysis, ventricular arrhythmia (LQT) and dysmorphic features.
What is the Schwartz score and what is it’s clinical use?
Estimate of the clinical likelihood of LQTS based on ECG, clinical and family history.
Used to guide investigation with genetic testing.
What is the role of ecg stress testing in those with suspected LQTS?
Exercise associated arrhythmias
Change in T wave morphology
Maladaptive QT response during recovery
What are the maladaptive T wave changes during recovery after ecg stress testing in a patient with LQTS?
QTc >470 at 2-4 mins - 70% PPV for LQT1
QTC >470 a 5 min - 70% PPV for LQT2
In what type of LQT is stress ecg not helpful and why?
LQT3 - QTc is short in exercise and recovery
What is the management of patients with symptomatic congenital LQTS?
- General measures (avoid drugs, electrolyte optimisation, AED plan)
- Lifelong beta blockade - propranolol (nadolol and metoprolol)
- ICD as secondary prevention
- ICD or sympathetic denervation if recurrent events despite maximum beta blockade
What is the rate of cardiac events in athletes with LQTS who chose to remain in competitive athletics?
Genotype pos/phenotype negative - 0% after 5 years (70 patients)
Genotype and phenotype pos - 1 in 60 after five years
How does type of LQTS inform on management?
LQT1 - better to avoid competitive athletics
LQT2 - avoid loud alarms, acute arousal events
LQT3 - less benefit with beta blockade. Consider adding mexilitine
When are ICDs recommended in Long QT?
- Sudden cardiac arrest for secondary prevention
- Recurrent cardiac syncope despite beta blockers and LCSD
- Recurrent cardiac syncope despite beta blockers in those not a candidate for LCSD
- Asymptomatic patient with LQT2/3 with resting QTc of 550
- Post pubertal women LQT2 QTc>500
When is family history of LQTS associated SCD an indication for ICD?
Never
What is the rate of major complications from ICD placement over 5 years?
31% (infection, lead fracture, dislodgement, inappropriate discharges, psychiatric)
What percentage of congenital LQTS will require ICD?
10%
Describe Romano Ward Syndrome.
Autosomal dominant LQTS
Describe Brugada Syndrome.
Autosomal dominant disorder with variable expression characterised by characteristic ECG findings with risk of ventricular arrhythmias.
What percentage of patients with Brugada pattern ECGs will have an event in 2.5 years?
10%
What mental health disease is associated with high rates of Brugada pattern on ECG?
Schizophrenia even after controlling for sodium channel blockers
What genes are causative of Brugada?
SCN5A
SCN10A
What channels are associated with Brugada?
Sodium channels 50%
Calcium channels 10%
Potassium channels
What a drugs are associated with transient Brugada pattern?
Cocaine - sodium channel blockade
TCA
Neuroleptic Drugs
How does autonomic tone influence Brugada Syndrome?
increased arrhythmia at night suggests parasympathetic worsening of Brugada
What is the incidence of AF in Brugada?
20%
Increased disease severity and higher risk of VF
What is the significance of agonal nocturnal breathing in a patient with suspected cardiac arrhythmia?
Equivalent of syncope/ventricular arrhythmia. ?aborted event
How does fever influence Brugada?
Reduction in sodium current resulting in predisposition to ventricular arrhythmia
What is the diagnostic criteria for type 1 Brugada Syndrome?
- Symptomatic
- Type 1 Brugada pattern (V1 or V2) at rest or with flecainide challenge - Asymptomatic
- type 1 Brugada pattern with
- first degree block, LAD
- atrial fibrillation
- late potentials, fragmented qrs, st-t wave alternans with VEB
- absence of structural heart disease
- absence of coronary artery disease
What is the definition of “symptomatic” in patients with Brugada?
VF/VT Inducible VT on EP Unexplained syncope Nocturnal agonal respiration Family history of SCD <45 years Family history Type 1 Brugada pattern
What is the definition of Type 2 Brugada?
- Type 2 Brugada pattern
- Conversion to Type 1 with flecainide challenge
- Additional feature listed below
- VF/VT
- inducible VT on EP
- family history SCD <45
- family history of Type 1 ECG
- agonal respiration
- unexplained syncope
What is the evaluation of suspected Brugada Syndrome?
Echo
+/-CMR
Stress testing/imaging
In the setting of wide complex tachycardia, what are the features suggestive of VT over SVT with aberrant conduction?
Capture beat
Fusion beat
Positive or negative concordance in QRS in chest leads
No mans land axis (pos aVR and neg aVF and lead 1)
RSR pattern with taller left rabbit ear
Absence of typical LBBB or RBBB pattern
How do you risk assess people with Brugada pattern without symptoms?
Family history
Cardiac imaging - echo/MRI
Drug Challenge if Type 2
Signal averaged ECG
Consider EP study in Type 1 asymptomatic patients
Genetic testing if mutation identified in family
What are the indications for Flecanide challenge in those with Type 2 brugada pattern?
Family history of SCD<45 years
Family history of Type 1 pattern
No family history, recommend against challenge
What is the sensitivity of flecainide drug challenge in patients with Type 2 Brugada for Type 1 pattern?
15-100%
What are the indications to stop a drug challenge for ?Brugada?
Type 1 brugada pattern
>2mm increase in ST elevation with Type 2
Ventricular premature beats
QRS widening >30%
What is the rate of sustained ventricular arrhythmia following drug challenge?
1-2%
What is the duration of ECG monitoring post Flecainide challenge for Brugada?
30min if IV
4 hours if oral
ECG at 90 min is useful
VT on exertion makes you suspicious of what diseases?
HOCM
CPVT
What proportion of patients with Brugada pattern ECG with inducible VT on EP studies will develop and arrhythmic event?
3.3%
What is the management of Brugada Syndrome?
- Treat pyrexia
- ICD implantation
- Antiarrrhythmics indicated if refusing ICD or multiple shocks (quinidine or amiodarone)
- Avoid sodium channel blockers (apart from quinidine)
- Catheter ablation - if recurrent events
- Family Testing
What is the family testing in a patient with Brugada Syndrome?
ECG and clinical history
If negative for repeat assessment every 1-2 years as may still develop disease
What are the rates of complications with ICD placement over 6 years?
37% inappropriate shock
30% lead issues
What is the incidence of AF >30s in patients in sinus with mitral stenosis on holter?
What is the rate of TIA/stroke?
29%
5.7%
What are the ecg features of RVOT VT?
LBBB morphology Rightward axis (positive inferior leads)
Physiology of typical slow fast AVNRT
Atrial premature beat allows slow pathway conduction while fast is refractory.
Slow pathway then is able to conduct through to the fast pathway resulting in AVNRT
Which has the shorter refractory period, slow or fast pathway of AVN?
Slow pathway - short refractory period
Fast pathway - long refractory period