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Efficacy Of ICDs For The Prevention Of Sudden Death In Patients With Hypertrophic Cardiomyopathy

Efficacy Of ICDs For The Prevention Of Sudden Death In Patients With Hypertrophic Cardiomyopathy. *Maron BJ et al. N Engl J Med. 2000:342;365-373. Etiology: Autosomal dominant trait caused by a variety of genetic mutations of sarcomere proteins

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Efficacy Of ICDs For The Prevention Of Sudden Death In Patients With Hypertrophic Cardiomyopathy

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  1. Efficacy Of ICDs For The Prevention Of Sudden Death In Patients With Hypertrophic Cardiomyopathy *Maron BJ et al. N Engl J Med. 2000:342;365-373.

  2. Etiology: Autosomal dominant trait caused by a variety of genetic mutations of sarcomere proteins Prevalence: Relatively common for a genetic disease, although uncommon in cardiologic practice (1:500 in general population) Presentation: Heterogeneous primary cardiac disease with particularly diverse clinical, morphologic and genetic features Natural History:Variable, often benign, but associated withrisk for sudden death in some patients Hypertrophic Cardiomyopathy

  3. Causes of SCD in Young People Mildly increased cardiac mass(10%) Congenital coronaryanomalies(19%) Ruptured aorta 5% Tunnelled LAD 5% Aortic stenosis 4% Myocarditis 3% HCM (36%) Dilated cardiomyopathy 3% ARVC 3% MVP 2% CAD 2% Other 6% Maron BJ et al. Circulation. 1996;94:850-56.

  4. HCM: Modes of Death Age at Death (years)

  5. Profiles in Prognosis for HCM Sudden Death Risk Symptom Progression End- Stage AF Spirito P et al. N Engl J Med. 1997;336:775-85.

  6. Usually no or only mild prior symptoms Usually occurs while sedentary or with mild physical exertion; not infrequently with physical exertion May occur at any age; but most commonly in the young Profile of Sudden Death in HCM

  7. Mortality in HCM % Mortality 5-15 16-25 26-35 36-45 46-55 56-65 66-75 >75 Age at Death or Most Recent Evaluation (years)

  8. Wall Thickness and Sudden Death In HCM 18.2 11.0 Incidence of Sudden Death (per 1000 person – yr) 7.4 2.6 0 <15 16 - 19 20 - 24 25 - 29 > 30 Spirito P. et al. N Engl J Med. 2000:342;1781.

  9. Triggers of Sudden Death Myocardial Ischemia  Outflow Obstruction Intense physical exertion Atrial fibrillation Substrate (Disorganized myocardial architecture) Ventricular Tachyarrhythmias Maron BJ. Hypertrophic cardiomyopathy. Curr Prob Cardiol. 1993;18:639-704.

  10. ICD Strongest SCD Risk Factors: Cardiac arrest/sustained VT Family history of sudden death Malignant genotype Recurrent syncope Multiple-repetitive NSVT Exercise hypotension(?) Massive LVH Highest Amiodarone (?) Intermediate Lowest Maron BJ et al. Curr Prob Cardiol. 1993;18:637-704.

  11. Drugs Limitation ß-adrenergic blockers no dataverapamil procainamide proarrhythmiaquinidine amiodaroneunresolved efficacy; chronic use unrealistic Previously Proposed Pharmacological Therapy For Sudden Death Prevention in HCM

  12. Sudden cardiac death in HCM is triggered by ventricular tachyarrhythmias that usually occur unpredictably and without warning. The implantable defibrillator will reliably sense and automatically terminate these arrhythmias when they occur. This hypothesis can be confirmed by a carefully designed retrospective study. ICD-HCM Trial:Hypothesis Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  13. Unequivocal diagnosis of HCM with two-dimensional echocardiography Successful implantation of a defibrillator for the purpose of sudden death prevention Minimum three month follow-up period after implant ICD-HCM Trial: Patient Selection Criteria Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  14. Primary: Prophylactic:Prevention with > 1 risk factor Secondary: Following cardiacPrevention arrest or sustained VT ICD-HCM Trial: Definition of Implant Treatment Objectives Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  15. ICD-HCM Trial: Primary Prevention Risk Factors* No. Patients % (n=128) Syncope 41 32% Family history of sudden death due to HCM 39 30% Nonsustained VT on Holter 32 25% Massive LVH (> 30mm) 10 8% *patients frequently had multiple risk factors Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  16. No. patients: 128 Male gender: 70% Age at implant: 8-82 (mean 40)52% < 40 years 25% < 30 years Outcome: Alive 126 Died 2* ICD for HCM Clinical Trial: Demographics * end-stage disease; one with a prior appropriate discharge

  17. Implant years: 1984-98 (80% > 1994) Mean follow-up: 3.1 years Mode of implant: Transvenous 80% Thoracotomy 20% Device capability: Bradycardia / ATP 80% Electrograms 75% ICD-HCM Trial: Demographics Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  18. NYHA Class I 65% NYHA Class II 21% NYHA Class III / IV 14% Mean Max. LV wall thickness (mm) 23 + 7 Mean LV end-diastolic cavity (mm) 44 + 8 Mean left atrial dimension (mm) 44 + 6 LV outflow obstruction (basal grad. > 30mmHg) 18% Antiarrhythmic drugs (amiodarone; sotalol; disopyramide) Before ICD 41% After ICD 32% ICD-HCM Trial: Clinical and Echocardiographic Data Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  19. Appropriate ICD termination of VT / VF, as surrogate for sudden death (n=29) Based on analysis of stored ECG cycle length data / electrograms (n=21) In absence of stored data, based on clinical circumstances (n=8) ICD-HCM Trial: Clinical End-point Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  20. ICD-HCM Trial Age at Implant No. of Patients <10 11-15 16-20 21-25 26-30 31-35 36-40 41-45 46-50 51-55 56-60 61-65 66-70 71-75 >76 Age At Implant (years) Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  21. ICD-HCM Trial: Appropriate Interventions 128 No. patients Appropriate discharges 29 Follow-up = 3.1 years ICD discharge rate 7.3% / yr 11.0% 4.5% 2º prevention 1º prevention Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  22. ICD-HCM Trial 1.0 1 prevention 2 prevention 0.8 0.6 Event-Free Survival 0.4 0.2 P=0.004 0.0 0 2 4 6 8 10 12 14 16 No. at risk 1 : 85 39 17 3 1 0 02 : 43 17 16 6 3 1 1 Years Post-Implant Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  23. Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  24. ICD-HCM Trial: Age At 1st Intervention Percent <10 11-15 16-20 21-25 26-30 31-35 36-40 41-45 46-50 51-55 56-60 61-65 66-70 >70 Age Groups (years) Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  25. No. Patients 0-4 5-9 10-14 15-19 20-24 25-29 30-34 35-39 40-44 45-49 50-54 55-59 60-64 65-69 >69 Months ICD-HCM TrialTime to 1st Intervention Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  26. ICD-HCM Trial: Number ofInterventions No. Patients  No. Appropriate Interventions Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  27. ICD-HCM Trial: Arrhythmias Triggering ICD Interventions VT 48%only VTVF 14% VT and VF 9% VF 29%only Bradyarrhythmias = 0 Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  28. A B C D

  29. ICD-HCM Trial: Interventions and Implant Justification Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  30. ICD-HCM Trial: Concomitant Drug Treatment With Appropriate Discharge Without Appropriate Discharge No. patients 29 99 Pct. on anti-arrhythmicdrugs* 52% 21% p < 0.04 * amiodarone, sotalol, disopyramide Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  31. ComplicationsNo. Patients Inappropriate Discharges Sinus tachycardia 13 AF with rapid ventricular rate 10 Lead dislodgement, disruption, 9 or oversensing ICD-HCM Trial:Complications Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  32. ComplicationsNo. Patients Lead fracture / disruption 12 Infection / explant 2 Subclavian thrombus 1 Hemorrhage 1 Hematoma 1 Clinical depression 1 ICD-HCM Trial:Complications Maron BJ, et al. N Engl J Med. 2000;342:365-373.

  33. The implantable defibrillator in HCM: Is highly effective in terminating life threatening ventricular tachyarrhythmias, often in young patients with few or no symptoms Has demonstrated a life-saving role both for secondary prevention (following aborted cardiac arrest or sustained VT) and the prophylactic, primary prevention of sudden death in patients judged to be at high-risk based on their clinical profile Has demonstrated primary VT / VF to be the principal mechanism of sudden death ICD-HCM Trial: Conclusions Maron BJ, et al. N Engl J Med. 2000;342:365-373.

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