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Disclosures St. Jude medical Honorarium Recipient Boston - - PDF document

Jan 23, 2023 271 likes •465 views

10/10/2015 Geonomic Arrhythmia Syndromes: ECG Recognition and Therapy Melvin Scheinman, MD Disclosures St. Jude medical Honorarium Recipient Boston scientific Honorarium Recipient Medtronick Honorarium Recipient Amgen Panel Member

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Geonomic Arrhythmia Syndromes: ECG Recognition and Therapy

Melvin Scheinman, MD

Disclosures

  • St. Jude medical

Boston scientific Medtronick Amgen Biotronik Tremura Bio-Sence Webster Honorarium Recipient Honorarium Recipient Honorarium Recipient Panel Member Honorarium Recipient Honorarium Recipient Honorarium Recipient

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Genetics of Sudden Cardiac Death

  • 1. Ion Channelopathy
  • A. Long and Short QT syndromes,

K+, Na+ Ca++ . B Brugada Na+,Ca++

C Early Repolarization Syndromes

D CPV T Ca++ 2. Sarcomeric Protein Abnormalities (IHSS)

ARVD intercellular connection abnormalities

3. IHSS

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  • CLINICAL EXPRESSION OF SODIUM CHANNEL

ABNORMALITIES

  • GAIN OF FUNCTION
  • LONG QT SYNDROME (LQT3)
  • LOSS OF FUNCTION
  • BRUGADA SYNDROME
  • IDIOPATHIC CARDIAC CONDUCTION DISEASE
  • CONGENITAL SINUS NODE DYSFUNCTION
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Brugada: Pathophysiology

SCN5A Sodium Channel

Predicted structure of SCN5A. Green: mutations causing Brugada syndrome Red: mutations causing long QT syndrome Type Current Gene B Subunit Clinical features

LQT1 Iks KCNQ1 MinK a) Sx with stress, especially swimming

b) Responsive to beta-blockers,AICD seldom required

LQT2 Ikr KCNH2 MiRP1 a) Stress, auditory

b) Partially responsive to beta-blockers

LQT3 Ina SCN5A a) Sx at rest

b) Unresponsive to beta-blockers c) Higher mortality

Major Genetic Abnormalities for LQTS

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1. Use leads II, V5/V6 avoid V2/V3 2. Average 3-5 beats 3. Stable rhythm 4. Bazett correction is not accurate for HR < 50 (under corrects) or > 90-100 (over corrects)

ECG Analyses for LQTS

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1. QT prolongation and pattern of T wave 2. Response to epinephrine / exercise 3. Family screening 4. Genetic testing – very valuable if positive

Diagnosis of Congenital LQS

Wilde JACC 54:143 ‘09

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H.R.

  • 1. Cardiac event prior to age 18

2.43

  • 2. LQT1 > LQT2

2.27

  • 3. Female gender

2.77

  • 4. QTc > 500

10.07

  • 5. Beta Blocker Rx

0.41

Source: JACC 49:329 ‘07

Long-QT Syndrome in adults (Registry) LQT1 = 428 LQT2 = 302 LQT3 = 82

Treatment of LQTS

1.Beta blockers for all forms (LQT1,2,3) 2.Pacemaker/Defibrillator 3.Left Cervical sympathectomy 4.Mexilitine,Flecainide and Ranolazine for LQT3 5.Beta blockers for pregnancy especially LQT2

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BRUGADA SYNDROME

  • First described in 1992 in 8 patients with

aborted sudden cardiac death

  • Characterized by:

– ECG findings of RBBB and persistent ST segment elevation in V1-V3 – Structurally normal hearts – Propensity for life threatening ventricular arrhythmias

Brugada: Diagnosis

ECG Findings

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ASYMPTOMATIC PROVOCABLE BY DRUGS AVOID SODIUM CHANNEL BLOCKERS/TREAT FEVER SYNCOPE/ABORTED SCD AICD DRUG THERAPY QUINIDINE AMINOPHYLLINE IV ISUPREL FOR VF STORM

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1. Elevation of QRS-ST junction (J wave) in at least 1mm in 2 leads 2. Slurring of terminal QRS into the ST segment or notching 3. These findings were limited to inferior and/or lateral leads

Definition of early repolarization:

Haissaguerre NEJM May’08

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ARVD(DEFINITION)

  • A DISEASE CHARACTERIZED BY

FIBROFATTY REPLACEMENT OF THE FREE WALL OF THE RV

  • STRUCTURAL ABNORMALITIES

PREDISPOSE TO VENTRICULAR ARRHYTHMIAS AND RISK FOR SUDDEN DEATH

  • APPEARS TO BE GENETICALLY

TRANSMITTED IN @ 30%-50%

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Figure 1

Figure 1 Fibro-fatty tissue infiltration of the right ventricle free wall in a patient with ARVC/D.

http://reference.findtarget.com/search/Arrhythmogenic%20right%20ventricular%20dysplasia/

Lombardi et al Ped Card 01/2011

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< 1% males 19 – 45 < 3% females 19 – 45

Marcus, F. Am J Cardiol;95:1070

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  • 1. Beta blockers (Sotalol)
  • 2. Amiodarone
  • 3. Catheter ablation
  • 4. AICD

M S

ARVD Therapy

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  • 1. Beta blockers (ineffective in 30%)
  • 2. Beta blockers + Ca++ channel

blockers

  • 3. Flecainide
  • 4. AICD, Cervical Sympathectomy
  • 5. Cardiac transplantation

CPVT Treatment