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Normal Heart VT. Syndromes. Long QT Short QT Brugada Catecholaminergic Polymorphic VT-CPVT Idiopathic VF Short coupled TdP Lev-Lenegre Syndrome. Channelopathies ECG and the Action Potential. Case 1. 13 yo girl presents with syncope while swimming QTc ≥500 msec

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Normal Heart VT

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Normal heart vt

Normal Heart VT


Syndromes

Syndromes

  • Long QT

  • Short QT

  • Brugada

  • Catecholaminergic Polymorphic VT-CPVT

  • Idiopathic VF

  • Short coupled TdP

  • Lev-Lenegre Syndrome


Channelopathies ecg and the action potential

ChannelopathiesECG and the Action Potential


Case 1

Case 1

  • 13 yo girl presents with syncope while swimming

    • QTc ≥500 msec

    • ß-blocker initiated with no further events

    • Presents five years later inquiring about stopping medications

      • Do you stop ß-blocker?

      • Is an ICD indicated?


Long qt syndrome history

1957

1963-1964

1958-1970

1971

1979

1991-2007

1st LQTS family reported

Romano-Ward Syndrome

25 LQTS cases reported

1st treatment – left stellate ganglionectomy

LQTS registry started

10 LQTS genes identified

Long QT SyndromeHistory


Long qt syndrome overview

Long QT SyndromeOverview

  • Incidence: 1/7,000

  • Presentation: mean age 8-14 years

  • Symptoms:

    • Syncope, palpitations, seizures, sudden death

    • Syncope in pediatric population should be considered malignant until proven otherwise

  • Arrhythmia: Torsades des pointes


Lqt 1 broad t waves

LQT 1Broad T waves


Lqt 2 bifid t waves

LQT 2Bifid T waves


Lqt 3 prolonged st segment

LQT 3Prolonged ST segment


Long qt syndrome diagnosis

Long QT SyndromeDiagnosis

  • ECG definition

    • QTc > 460 females

    • QTc > 450 males

  • Challenges

    • 25 to 50% of LQT 1, 2, & 3 individuals will have QTc ≤ 460 msec

    • Genetic testing has 3 to 5% false (+) rate

  • Epinepherine challenge

    • Useful in evaluating LQT1 (∆QT 30 msec)

      • Iks response to epinepherine in LQT1 impaired

      • NPV 93%, PPV 76%, Sens 92%, Spec 86%

      • Less useful when on beta-blockers


Lqts gene specific triggers

LQTSGene Specific Triggers

Lethal and Non-lethal

CV Events

Schwartz PJ et al. Circulation 103:89-95, 2001


Long qt syndrome high risk features

Aborted cardiac arrest

Family history (< 50 y) of cardiac arrest or unexplained syncope

History of “seizures” or congenital deafness

Prolonged QTc ≥500 msec on ECG

Positive genetic test

Long QT SyndromeHigh Risk Features


Lqts risk of cardiac event syncope cardiac arrest or sudden death

LQTSRisk of Cardiac Event (syncope, cardiac arrest, or sudden death)

  • Risk > 50%

    • QTc ≥ 500 msec: LQT1, LQT2, Male LQT3

  • Risk 30-50%

    • QTc ≥ 500 msec: Female LQT3

    • QTc < 500 msec: Female LQT2/3, Male LQT3

  • Risk < 30%

    • QTc < 500 msec: LQT1, Male LQT2


Lqt subtypes

LQT Subtypes


Lqts management options

LQTSManagement Options

  • Lifestyle modification (IB)

  • Beta-blockers (IB)

    • Very effective LQT1, Moderate LQT2

    • Minimal effect LQT3

  • ICD plus BB

    • Cardiac arrest (IA)

    • Syncope / VT (IB)

    • Prophylactic in LQT2 or LQT3 (IIB)

  • Left stellate ganglionectomy (IIB)


Lqt resources

LQTResources

  • Cardiac Arrhythmias Research & Education (CARE)

    • www.longqt.org

  • Cardiac Arrest Survivors Network (CASN)

    • www.casn-network.org

  • International Registry for Drug Induced Arrhythmias

    • www.qtdrugs.com


Case 1 review

Case 1 Review

  • 13 yo girl with syncope during swimming and QTc ≥500 msec

    • Asymptomatic for 5 y on BB

    • Swimming…suggests LQT1

    • High risk subgroup based LQT1 and QTc ≥500 msec

    • Recommendation

      • Continue BB given very effective in LQT1

      • Consider ICD if has arrest, syncope, or VT


Case 2

Case 2

  • 17 yo girl presents with atrial fibrillation

    • QT 268 msec at HR 69

    • Mother, age 51, and brother, age 21 with QT intervals of <300 msec also

    • History, exam, and cardiovascular workup otherwise negative

    • First reported family

      • Cardiology 2000;94:99-102


Short qt syndrome 1999 dr p bjerregaard

Short QT Syndrome1999 – Dr. P. Bjerregaard


Short qt ecg characteristics

Short QTECG Characteristics

  • QT < 300msec

  • No significant QT change with HR ∆s

  • Short ST segment with tall, narrow peaked T-waves in V1-V6

  • Reentrant arrhythmias

  • Other clues

    • Lone AF, VF

    • Family Hx of SCD


Short qt

Short QT

  • EP testing

    • Short atrial and ventricular refractory periods

  • Management

    • Pharmacological (small studies)

      • Only hydroquinidine effective in increasing QT

      • Fleicanide, sotalol, ibutilide ineffective

    • ICD experience (limited)

      • T wave oversensing/inappropriate shocks

      • Device selection (St.Jude – delay/decay)


Case 2 review

Case 2 Review

  • 17 yo girl with AF and short QT. Mother and brother with short QT.

    • Treated with quinidine

      • For atrial fibrillation suppression

      • QT prolongation via K+ channel blockade

      • Long-term follow-up unavailable


Case 3

Case 3

  • 39y man c/o cp, palpitations, and presyncope

    • PMH: none

    • SH: married, carpenter, occasional beer

    • FH: (-) sudden death, arrhythmias, premature CVD

    • Normal cardiac markers, echo


Brugada syndrome overview

Brugada SyndromeOverview

  • Identified 1992

  • Age spectrum - 2d to 84y

  • Mean age sudden death 40 ± 15y

  • Men > 5x risk of arrhythmic events

  • Prevalence

    • 5/10,000 - overall

    • #2 cause of death SE Asian men <40y

  • Dynamic but characteristic ECG changes

  • 1 in 5 have Na channel mutation (SCN5A)


Brugada syndrome definition

Brugada SyndromeDefinition

  • Type 1 pattern ECG in V1-V3 plus 1 of following:

    • Pharm conversion to Type 1 from Type 2/3 ECG

      • Na channel blocker (procainamide, fleicanide, ajmaline)

    • Documented VF/polymorphic VT

    • Family history of SCD < 45y

    • Inducible VT at EP study

    • Syncope

    • Nocturnal agonal respirations

  • ECG pattern only = Brugada pattern ECG but not Brugada Syndrome

  • Exclude other heart conditions


Brugada pattern ecg st elevation v1 v3

Brugada pattern ECGST elevation V1-V3

  • Type 1 (DIAGNOSTIC)

    • Coved ST elevation ≥ 2mm with negative T wave

    • sensitivity by moving V2/V3 from 4th to 2nd/3rd intercostal space

  • Type 2

    • Saddleback ST elevation ≥ 2mm w/ ST trough ≥ 1mm

    • Positive/biphasic T wave

  • Type 3

    • Coved/saddle ST elevation ≥ 2mm w/ ST trough < 1mm

  • Also reported in inferior leads and left precordial leads

    • Some individuals also had SCN5A mutation


Brugada syndrome

Utility of EP study

Controversial

6-9% of healthy individuals of induced VF at EPS

Brugada, +EPS associated w/ 8x risk

Other conduction abnormalities

QT prolongation (R > L precordial)

Prolonged action potential duration in RV epicardium

P, PR, & QRS

PR prolongation associated with His-purkinje delay

Brugada Syndrome


Brugada consensus conference spontaneous type 1 ecg

Brugada Consensus ConferenceSpontaneous Type 1 ECG


Brugada consensus conference sodium channel blocker induced type 1 ecg

Brugada Consensus ConferenceSodium Channel Blocker Induced Type 1 ECG


Case 3 review

Case 3 Review

  • 39y man c/o cp, palpitations, and presyncope

    • Spontaneous type 1 ECG

    • “Asymptomatic”

    • Negative family hx

    • EP study (IIA indication)

      • Sustained VT with DES at 500 from RVA

      • No supraventricular arrhythmias induced

      • Normal AV node and His-Purkinje function

    • ICD was implanted (IIA indication)

      • Asymptomatic without events at 32 mo f/u

      • ** Most events occur at night - autonomic role?

    • Other tx options: ablation, quinidine


Case 4

Case 4

  • 16 yo girl suddenly arrests running into store

  • History of exertional palpitations and syncope

  • Successful resuscitation by bystander nurse


Catecholaminergic polymorphic ventricular tachycardia cpvt

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT)

  • Clinical Features

    • Direct correlation with adrenergic stimulation (physical/emotional)

      • Threshold heart rate 120-130 bpm

      • Abnormal automaticity or triggered activity

    • Bidirectional VT

    • Symptom onset in childhood

    • Genetic mutations – Ryanodine / Calsequestrin


Cpvt genetic mutations

CPVTGenetic Mutations

  • Calsequestrin (CASQ2)

    • Autosomal Recessive

    • Calcium storage protein w/in lumen of sarcoplasmic reticulum

  • Cardiac Ryanodine Receptor (RyR2)

    • Autosomal Dominant

    • Regulates Ca++ from sarcoplasmic reticulum

    • Delayed after-depolarizations

    • Associated with ARVC

    • RYR1 - malignant hyperthermia syndrome


Cpvt management

CPVTManagement

  • Anti-adrenergic treatment

    • Beta blockers are the mainstay of treatment

  • ICDs

    • B-blockers not always effective


Case 4 review

Case 4 Review

  • 16 yo with history of palpitations and syncope who collapses in store

    • Arrested 3 times en route to hospital

    • ICD implanted and atenolol started

      • 3 ICD revision procedures

      • 2 lead dislodgements resulting in inappropriate ICD therapies


Case 5

Case 5

  • 24 yo man with recurrent syncope

    • Signs and symptoms

      • Recent decrease in exercise tolerance

      • Lower extremity edema

      • Mild elevation in liver transaminases

    • Family hx + for sudden death – paternal uncle

    • Telemetry strip below


Arrhythmogenic right ventricular cardiomyopathy arvc

Arrhythmogenic Right Ventricular Cardiomyopathy - ARVC


Arvc indik jh et al indian pacing electrophys j 2003 3 148

ARVCIndik JH et al. Indian Pacing Electrophys J. 2003:3:148

  • Top picture:

    • Fibro-fatty replacement of the myocardium

    • Thin and enlarged RV wall.

  • Bottom picture:

    • Trichrome stain

    • Areas of mature fibrosis (F) and adipose tissue (A) within the epicardial (Epi) and mid-myocardial zones


Normal heart vt

ARVC

  • Diffuse fibrosis of the RV wall with preservation of normal LV tissue

    • Fibrous tissue appears white

    • Normal cardiac tissue appears black

    • www.geneticheartdisease.org

  • Ventriculogram demonstrating fibrofatty infiltration

    • Indik JH et al. Indian Pacing Electrophys J. 2003:3:148


Normal heart vt

ARVC

  • Signal-Averaged ECG -SAECG (below left):

    • Characteristic high-frequency low-amplitude late-potential

    • SAECG averages multiple QRS complexes that are then digitalized and filtered and further processed with spectral analysis to eliminate noise.

    • Late-potentials represent areas of delayed activation due to slowed conduction from either regions of scar or fibrosis  electrical substrate that initiates and perpetuates ventricular tachycardia.


Arvc high risk features

ARVC High Risk Features

  • Younger patients

  • Recurrent syncope

  • History of cardiac arrest or sustained VT

  • Clinical signs of RV failure or LV involvement

  • Patients with or having a family member with the high risk ARVD gene (ARVD2)

  • Increase in QRS dispersion ≥ 40 msec

    • QRS dispersion = max measured QRS minus min measured QRS

  • Naxos disease


Case 5 review

Case 5 Review

  • Diagnosis

    • Rhythm strip and ECG notable for epsilon waves and T wave inversion in right precordial leads

  • Risk

    • High risk features present – young age, recurrent syncope, signs of RV failure, family history of sudden cardiac arrest

  • Management

    • ICD implantation


Normal heart vt

  • Idiopathic Ventricular Fibrillation

    • Sodium channel mutation

  • Short-coupled Torsades des Pointes

    • Normal QT interval with coupling interval of 1st ectopic beat < 300 msec

    • Prognosis poor with unproven tx (BB or CCB); ablation?

  • Lev-Lenegre Syndrome

    • Progressive cardiac conduction defect associated with bradyarrhythmias although tachyarrhythmias may also occur

    • Sodium channel defect


Idiopathic ventricular fibrillation

Idiopathic Ventricular Fibrillation


Lev lenegre syndrome

Lev-Lenegre Syndrome

  • Progressive Cardiac Conduction Defect

    • Acquired complete heart block

    • Idiopathic fibrosis and calcification of cardiac conduction system

  • Very rare

  • Sodium channel mutations (subtype-SCN 5A)

  • Often result in bradyarrhythmias although tachyarrhythmias may also result

  • Lev M. Anatomic basis for atrioventricular block. Am J Med 1964;37:742-8.

  • Lenegre J. Etiology and pathology of bilateral bundle branch block in relation to complete heart block. Prog Cardiovasc Dis 1964;6:409-444


Hypertrophic cardiomyopathy

Hypertrophic Cardiomyopathy

  • #1 cause of SCA in athletes

    • > 1/3 of deaths

    • Often associated with physical activity

    • 60% high school age

    • >90% males

  • Genetic disorder left ventricular hypertrophy

  • First symptom often sudden death


Hcm ecg

HCM - ECG


Hcm echo www hcmny org

HCM – Echowww.hcmny.org


Hcm vs athletic heart

HCM

Septum > 15mm

Assymetrical (septum:posterior wall thickness > 1.5:1)

Occasional family history

No change with deconditioning

Athletic Heart

Septum < 15mm

Symmetrical thickening

No family history

Resolves with deconditioning – 3 mo

HCM vs. Athletic Heart


Thank you

Thank You

Questions?


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