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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


Syndromes

  • Long QT

  • Short QT

  • Brugada

  • Catecholaminergic Polymorphic VT-CPVT

  • Idiopathic VF

  • Short coupled TdP

  • Lev-Lenegre Syndrome


ChannelopathiesECG and the Action Potential


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?


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 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 1Broad T waves


LQT 2Bifid T waves


LQT 3Prolonged ST segment


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


LQTSGene Specific Triggers

Lethal and Non-lethal

CV Events

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


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


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


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)


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

  • 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

  • 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 Syndrome1999 – Dr. P. Bjerregaard


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

  • 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

  • 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

  • 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 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 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 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


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 ConferenceSpontaneous Type 1 ECG


Brugada Consensus ConferenceSodium Channel Blocker Induced Type 1 ECG


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

  • 16 yo girl suddenly arrests running into store

  • History of exertional palpitations and syncope

  • Successful resuscitation by bystander nurse


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


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


CPVTManagement

  • Anti-adrenergic treatment

    • Beta blockers are the mainstay of treatment

  • ICDs

    • B-blockers not always effective


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

  • 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


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


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


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

  • 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

  • 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


  • 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


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

  • #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 – Echowww.hcmny.org


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


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