Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High...

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Raul Weiss MD, FAHA, FACC, FHRS, CCDS Director, Electrophysiology Fellowship Program Associate Professor of Medicine The Ohio State University May 2011 HRS Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures • Educational and research support from Boston Scientific, Medtronic, St Jude Medical, Biotronik, Biosense Webster, Gene Dx, Familion, Cameron Health and Stereotaxis • Advisory Boards honoraria from Stereotaxis, Biosense Webster, Cameron Health and St Jude medical • Speaker honoraria from St Jude Medical, Biotronik, Medtronic and Boston Scientific • I will be discussing non FDA approved devices

Transcript of Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High...

Page 1: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Raul Weiss MD, FAHA, FACC, FHRS, CCDSDirector, Electrophysiology Fellowship Program

Associate Professor of MedicineThe Ohio State University

May 2011 HRS

Primary Therapy for High Risk LQT Patients Should Be an ICD

Disclosures • Educational and research support from Boston

Scientific, Medtronic, St Jude Medical, Biotronik, Biosense Webster, Gene Dx, Familion, Cameron Health and Stereotaxis

• Advisory Boards honoraria from Stereotaxis, Biosense Webster, Cameron Health and St Jude medical

• Speaker honoraria from St Jude Medical, Biotronik, Medtronic and Boston Scientific

• I will be discussing non FDA approved devices

Page 2: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Dr. London Did A Great Job Describing The ICD-Road-Ahead in LQTS Patients

But Let’s Get Closer…

Page 3: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Who Are The Patients At TheHighest Risk for SCD That an

ICD Should be Considered

Current ACC/AHA/ESC Guidelines for Implantable Cardioverter Defibrillator Prescription in long QT

Syndromes

• Class I: Implantation of an ICD along with the use of beta-blockers is recommended for LQTS patients with previous cardiac arrest (level of evidence: A)

• Class IIa: Implantation of an ICD with continued use of beta-blocker can be effective to reduce SCD in LQTS patients experiencing syncope and/or VT while receiving beta-blockers (level of evidence: B)

• Class IIb: Implantation of an ICD with the use of beta-blockers may be considered for prophylaxis of SCD for patients in categories possibly associated with higher risk of cardiac arrest such as LQT2 and LQT3 (level of evidence: B)

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Rate of ACA or SCD by Genotype and QTc Category

Ilan Goldberg J Am Coll Cardiol 2011;57:51–9 (MODIFIED)

Age of 10 10%

24%

15%

4% 1%

Rate of ACA or SCD in PatientsWith Normal-Range QTc by Mutation Location

and Type

Ilan Goldberg J Am Coll Cardiol 2011;57:51–9

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Cumulative Event-Free Survival For a First Appropriate ICD Shock According to Genotype

Schwartz, P. J. et al. Circulation 2010;122:1272-1282

Five-Year Cumulative Probability of ACA/SCD by Number of Syncopal

Events and QTc

Liu, J Am Coll Cardiol 2011;57:941–50)

Page 6: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Cumulative Event-Free Survival For a First Appropriate ICD Shock

Schwartz, P. J. et al. Circulation 2010;122:1272-1282 (modified)

By Corrected QT interval

By Risk Factors

ICD Reports in LTQS Patients

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Schwartz, P. J. et al. Circulation 2010;122:1272-1282

Distribution of Patients at Implantation by Age and Gender

Cumulative Probability of Total Death in LQTS Pts with ACA or Recurrent

Syncope on ß-Blockers

Zareba, JCE,April 2003 Vol14,337-342

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ICD In LQTS Patients

• Thirty-five LQTS Pts 75% ACA• Mean Age of 29 Y/o 83%F• 43% were Younger than 21 y/o• No deaths in 31 ± 21 months• 21 Pts with appropriate shocks

Groh et. al. AJC 1996;78:703-6

Summary of Individual and Cumulative ICD Risk Factor

Scorecard Elements Related to Appropriate ICD Therapies

Justin M. Horner Heart Rhythm 2010;7:1616–1622

*= p <0.05

Page 9: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Pros and Cons of ICD Over Medical Therapy

Pros• Highly effective• Compliance

– If you forget to take you BB– Diarrheal illnesses

• LQT-Prolonging Drug– Hypokalemia

• Family comfort/reassurance

Cons• Cost• Inappropriate therapies• Procedural complications• Long term complications

Pros and Cons of ICD Over Medical Therapy

Pros• Highly effective• Compliance

– If you forget to take you BB– Diarrheal illnesses

• LQT-Prolonging Drug– Hypokalemia

• Family comfort/reassurance

Cons• Cost• Inappropriate therapies• Procedural complications• Long term complications

Solutions• Longer detection times• Higher rate cut-off• Rate smoothing

• S-ICD• Increase battery longevity • Alert algorithms

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ICD Utilization in The US in Patients Younger than 18 Y/O

Burns K. Heart Rhythm 2011;8:23–28

Hospital Data on ICD Implants on Patients <18 Years-old

Burns K. Heart Rhythm 2011;8:23–28

Page 11: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

In Addition To Guidelines: ICD Should Be Considered in Pts With• LQTS patients with double mutations• Corrected QT of ≥500 msec and certainly if over ≥550 msec

• Congenital deafness• Patients that had a syncopal event regardless of the

QT duration• Genetically positive LQT 1 to 3 with

+Transmembrane-missense mutation• Family History is Not an indication for ICD

With Great Power Comes Great Responsibilities

Spiderman’s Uncle Ben

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Thank you

Back up slides

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What is the Problem?

Asymptomatic

SCD

Syncope

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Liu, J Am Coll Cardiol 2011;57:941–50)

Risk Stratification

• Notably, women with the LQT2 genotype who experienced a first cardiac event exhibited an extremely high rate of subsequent events (58% during only 2 years of follow-up), further stressing the importance of careful follow-up and timely therapeutic intervention in this high-risk population

Liu, J Am Coll Cardiol 2011;57:941–50)

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Liu, J Am Coll Cardiol 2011;57:941–50)

Liu, J Am Coll Cardiol 2011;57:941–50)

Page 16: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Liu, J Am Coll Cardiol 2011;57:941–50)

• How do you account to see of BB are working? Stress Test? HR? follow the QT? Assess for symptoms?

• How do you know your patient is taking the medication (compliance)

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Distribution of QTc Interval Duration

in Genotype-Positive Patients With LQTS

Ilan Goldberg J Am Coll Cardiol 2011;57:51–9

Ilan Goldberg J Am Coll Cardiol 2011;57:51–9

Page 18: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Ilan Goldberg J Am Coll Cardiol 2011;57:51–9

Ilan Goldberg J Am Coll Cardiol 2011;57:51–9

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Comparison of Clinical Characteristics of LQTS Patients With ACA Who Did and Did Not

Receive ICDs

Zareba, JCE,April 2003 Vol14,337-342

Zareba, JCE,April 2003 Vol14,337-342

Page 20: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

Comparison of Clinical Characteristics of LQTS Pts With Recurrent Episode of

Syncope Despite BB Who Did and Did Not Receive an ICD

Zareba, JCE,April 2003 Vol14,337-342

Cumulative Probability of Total Death in LQTS Pts with ACA or Recurrent Syncope

on BB

Zareba, JCE,April 2003 Vol14,337-342

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Incidence of ICD-Implant Related Complications

Page 22: Primary Therapy for High Risk LQT Patients Should Be … Weiss - Primary...Primary Therapy for High Risk LQT Patients Should Be an ICD Disclosures ... Pros and Cons of ICD Over Medical

What if ICDs …

• Only shocks appropriately• Negligible risk of complication at initial

implant and/or during device change out

• Do not invade the intravascular space• Low cost ICD

• At 7.3 years of follow-up, more than 80% of those patients implanted with an ICD as primary prevention remained free of an appropriate discharge, compared with just 40% to 50% of those who received implantations for secondary prevention indications

Justin M. Horner Heart Rhythm 2010;7:1616–1622

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• The most common reasons of inappropriate therapy overall were T-wave oversensing (35%) and sinus tachycardia (19%). The average supraventricular rate triggering inappropriate therapies was 207 beats/min.

Justin M. Horner Heart Rhythm 2010;7:1616–1622

Asymptomatic Patients: Absolute Event Rates for SCD According to genotype and

QTc• LQT1 0.3%/y (M: 0.33%, F 0.28%)• LQT2 0.6%/y (M: 0.46%, F: 0.82%)• LQT3 0.56%/y (M: 0.96%, F: 0.30%)• QTc was particularly relevant, with a QTc of

500–549 ms (vs. ,499 ms) associated with an HR of 3.34, and a QTc interval of .550 ms (vs. ,499) contributed an HR of 6.35. Moreover,

• QTc interval ,499 ms was found not to contribute independently to an increased risk of a lethal event (compared with a QTc interval ,439 ms)

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Individual/cumulative risk factors and likelihood of an appropriate VF-terminating ICD therapy

Justin M. Horner Heart Rhythm 2010;7:1616–1622

Comparison of Clinical Characteristics of LQTS Pts With Recurrent Episode of

Syncope Despite BB Who Did and Did Not Receive an ICD

Zareba, JCE,April 2003 Vol14,337-342


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