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ECG Workshop in Cardiac Ion Channel Diseases Dr. Ngai-Shing Mok Dept of Medicine & Geriatrics Princess Margaret Hospital Hong Kong
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M/31 Good past health Found dead on bed in the morning Hx of syncope without warning while walking 2 weeks before his death No family hx of sudden death Autopsy – no structural heart disease Toxicology screening -ve Case One
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Baseline ECG before Treadmill Exercise Test
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ECG during Treadmill Exercise Test
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Questions (1)What are the ECG abnormalities ? (2)How are you going to confirm the diagnosis ?
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Baseline ECG before Treadmill Exercise Test
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ECG during Treadmill Exercise Test
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Mean age : 32 yrs M:F - 9:2 N = 289 BMI - 24.6
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Baseline ECG – normal During exercise test – 2mm coved-type ST elevation in V1 only Baseline ECGECG during exercise test Brugada syndrome ???
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ECG of his parent showing complete RBBB but no Brugada ECG pattern Family ECG screening
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1st molecular autopsy in PMH
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P1956_P1959del Molecular autopsy found a heterozygous 12- nucleotide deletion in CACNA1C
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Cardiac LTCC plays a pivotal role to regulate heart rhythm and contractility Mutations in LTCC reported to be associated with inherited arrhythmogenic diseases Brugada syndrome (BrS) Long & short QT syndrome (LQTS, SQTS) Idiopathic VF (IVF) Early repolarization syndrome (ERS) Cardiac L-type calcium channel (LTCC)
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Learning Points
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M/40 Good past health 3 younger brothers died of sudden nocturnal death at age 30+ years with –ve autopsy Referred for family screening All along no hx of syncope Case Two
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Baseline ECG at rest
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(1)What are the ECG abnormalities ? (2)What are the DDx ? (3)What further test would you like to perform to confirm your Dx ? Questions
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Baseline ECG at rest
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Type 2 Brugada ECG ?
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Corrado Index (STJ/ST80 >1) c/w Type 2 Brugada ECG
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6mm The base of the triangle 5mm below high take off > 3.5mm Favours type 2 Brugada ECG
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β >58 degrees β >58 degrees favours type 2 Brugada ECG & predicts a positive drug provocation test to unmask Type 1 Brugada ECG
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ECG after IV flecainide provocation test V1 & V2 on 4 th ICS Flecainide provocation test
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V1 & V2 recorded on 3 rd ICS after IV flecainide provocation
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Pre-flecainide Post-flecainide Flecainide provocation test converted type 2 to type 1 Brugada ECG
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Controversy in the prognostic value of EP Study in BrS
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PRELUDE Study PRogrammed ElectricaL stimUlation PreDictivE value in BrS A large prospective study to determine the role of EPS in risk stratification in BrS Priori et al. JACC 2012 Inducible VT/VF does not predict high risk in BrS
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Priori et al. Circulation 2002 Risk factors and prognosis of BrS
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(1)ICD implanted despite –ve EP study in view of very strong FHx of SD (considered as risk predictor in Japanese guidelines) (2)Genetic study found mutation in his CACNAIC gene ? pathogenicity
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Learning Points
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F/14 Good past health Suddenly collapsed with LOC after chasing and boarding a bus Hx of syncope 9 months ago after she quarrelled with her friend No family hx of sudden death Case Three
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ECG recorded by AED
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NS Mok ECG recorded in sinus rhythm after successful defibrillation
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NS Mok Wide-complex tachycardia recorded in ICU
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NS Mok Signal-averaged ECG : no late potential Echo : no structural heart disease CT brain : evidence of hypoxic brain damage Coronary MRA : no anomalous origin of coronary arteries Viral study for myocarditis : negative Investigations
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Questions (1)What are the ECG features during sinus rhythm ? (2)What is the tachycardia recorded in ICU ? (3)What are the DDx of such tachycardia ? (4)How would you confirm the Dx ?
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NS Mok ECG recorded in sinus rhythm after successful defibrillation
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NS Mok Wide-complex tachycardia recorded in ICU
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NS Mok VT with beat-to-beat alteration of QRS axis Bi-directional VT
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NS Mok 1.Advanced heart disease 2.Digitalis intoxication 3.Aconite poisoning due to overdose of “ 川烏、 草烏 ” 4.Familial hypokalaemic periodic paralysis 5.ARVD Type 2 6.Catecholaminergic polymorphic VT (CPVT) DDx of bi-directional VT
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Polymorphic VT Bi-directional VT Monomorphic PVCs Mok NS et al CMJ 2006 CPVT confirmed by hRyR2 mutation Adrenaline provocation test
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SCD / NS Mok CPVT should be suspected in young patients without structural heart disease presenting with syncope /sudden cardiac arrest / polymorphic VT / bi- directional VT induced by exercise or emotion Absence of structural heart disease Manifests in childhood and adolescence with a high lethality rate (30 – 50% mortality by age 30) Stress test (exercise or adrenaline infusion) and/or genetic test should be done if CPVT a DDx β-blockers is the cornerstone of therapy and will improve prognosis of patients Catecholaminergic Polymorphic VT (CPVT)
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Bi-directional VT with a RBBB pattern & alternating QRS axis Catecholaminergic Polymorphic VT (CPVT)
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SCD / NS Mok VFVTVT VT Exercise test induced bi-drectional VT in CPVT FF, Female; 17yrs; Exercise test
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NS Mok P.G, female, 9yrs Catecholaminergic bi-directional VT degenerating into VF
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Learning Points
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Case Four F/55 Hx of DM, IHD s/p PCI to RCA done Sudden collapse with LOC while shopping in Shenzhen Spontaneous recovery Visited PMH AED in the same afternoon
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NS Mok ECG recorded in AED during a witnessed convulsion
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(1)What are the ECG abnormalities ? (2)What is the single most important question you should ask the patient ? (3)What is the underlying cause ? (4)How would you treat this patient ? Questions
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NS Mok ECG recorded in AED during a witnessed convulsion
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NS Mok SSL Torsades de pointes QTc 600ms S-L-S sequence initiated Torsades de Pointes resulting in syncope
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NS Mok Progress (1) TdP suppressed by IV magnesium sulphate & transvenous temporary pacing at 100 beats/min
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NS Mok Normalization of QTc 4 days after withdrawal of ketoconazole Progress (2)
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NS Mok Drugs associated with LQTS www.torsades.org K+ Na+ Antiarrhythmic Drugs Quinidine, Procainamide Disopyramide Sotalol, Amiodarone Sotalol, AmiodaroneAntibiotics Erythromycin, Trimethoprim & Sulfamethaxazole, Pentamidine, Clarithromycin, Azithromycin Antihistamines Terfenadine, Astemizole, diphenhydramine Antifungal Fluconazole, Ketoconazole Antimalarial Chloroquine, Halofantrine Antipsychotic Drugs Haloperidol, Tricyclic antidepressants
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NS Mok 1.Avoid QT-prolonging drugs in patients at risk of TdP 2.Avoid >1 QT-prolonging drug at the same time 3.Avoid combination of QT-prolonging drug & cytochrome P450 inhibitor 4.Cardiac & QTc monitoring in the first few days when giving QT-prolonging anti-arrhythmic drugs to at-risk patients 5.Avoid hypokalaemia in patients receiving QT-interval prolonging drugs Good Practice to Avoid Drug-induced TdP
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Learning Points
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Case Five M/12 History of syncope while running Younger sister died of drowning at age of 10 LQTS suspected and Treadmill exercise test was done
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Resting ECG prior to exercise stress testing
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ECG during exercise stress testing
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(1) What are the ECG findings at rest and during exercise ? (2) What is his Schwartz score ? (3) Does he suffer from Long QT syndrome ? Questions
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Resting ECG prior to exercise stress testing
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ECG during exercise stress testing
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Resting ECG prior to exercise stress testing QT = 470ms RR = 1.16s QTc = 440ms
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Resting ECG prior to exercise stress testing QT = 470ms RR = 1.16s QTc = 440ms
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NS Mok Diagnostic criteria of LQTS ≤ 1 point – low probability >1-3 points – intermediate probability ≥ 3.5 points – high probability of LQTS (revised 2006) Schwartz PJ 1993 Schwartz Schwartz Score Total Schwartz score = 2.5
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ECG during exercise stress testing QT = 340ms RR = 0.52s QTc = 470ms QTc prolonged by ≥ 30ms Suggesting LQT1 syndrome
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Learning Points
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Thank You ! Princess Margaret Hospital Hong Kong
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