Primary Therapy for High Risk LQT Patients Should Be an ICD

Transcription

1 Primary Therapy for High Risk LQT Patients Should Be an ICD Raul Weiss MD, FAHA, FACC, FHRS, CCDS Director, Electrophysiology Fellowship Program Associate Professor of Medicine The Ohio State University May 2011 HRS 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

2 Dr. London Did A Great Job Describing The ICD-Road-Ahead in LQTS Patients But Let s Get Closer

3 Who Are The Patients At The Highest 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 betablockers is recommended for LQTS patients with previous cardiac arrest (level of evidence: A) Class IIa: Implantation of an ICD with continued use of betablocker can be effective to reduce SCD in LQTS patients experiencing syncope and/or VT while receiving betablockers (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)

4 Rate of ACA or SCD by Genotype and QTc Category 24% Age of 15% 10 10% 4% 1% Ilan Goldberg J Am Coll Cardiol 2011;57:51 9 (MODIFIED) Rate of ACA or SCD in Patients With Normal-Range QTc by Mutation Location and Type Ilan Goldberg J Am Coll Cardiol 2011;57:51 9

5 Cumulative Event-Free Survival For a First Appropriate ICD Shock According to Genotype Schwartz, P. J. et al. Circulation 2010;122: Five-Year Cumulative Probability of ACA/SCD by Number of Syncopal Events and QTc Liu, J Am Coll Cardiol 2011;57:941 50)

6 Cumulative Event-Free Survival For a First Appropriate ICD Shock By Corrected QT interval By Risk Factors Schwartz, P. J. et al. Circulation 2010;122: (modified) ICD Reports in LTQS Patients

7 Distribution of Patients at Implantation by Age and Gender Schwartz, P. J. et al. Circulation 2010;122: Cumulative Probability of Total Death in LQTS Pts with ACA or Recurrent Syncope on ß-Blockers Zareba, JCE,April 2003 Vol14,

8 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 *= p <0.05 Justin M. Horner Heart Rhythm 2010;7:

9 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 Solutions Longer detection times Higher rate cut-off Rate smoothing Cons Cost Inappropriate therapies Procedural complications Long term complications S-ICD Increase battery longevity Alert algorithms

10 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

11 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

12 Thank you Back up slides

13 What is the Problem? SCD Asymptomatic Syncope

14 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)

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

16 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)

17 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

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

19 Comparison of Clinical Characteristics of LQTS Patients With ACA Who Did and Did Not Receive ICDs Zareba, JCE,April 2003 Vol14, Zareba, JCE,April 2003 Vol14,

20 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, Cumulative Probability of Total Death in LQTS Pts with ACA or Recurrent Syncope on BB Zareba, JCE,April 2003 Vol14,

21 Incidence of ICD-Implant Related Complications

22 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:

23 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: 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 ms (vs.,499 ms) associated with an HR of 3.34, and a QTc interval of.550 ms (vs.,499) contributed an HR of 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)

24 Individual/cumulative risk factors and likelihood of an appropriate VF-terminating ICD therapy Justin M. Horner Heart Rhythm 2010;7: 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,