1. ICD terapija kod dece i adolescenata Goran Milašinović PMC, KCS

2. Disclosure Receive mild to moderate compensation for lectures and clinical studies from Medtronic, St Jude Medical, Biotronik and BioControl Co. Receive no grant from any company.

3. ICD terapija: prevencija iznenadne srčane smrti

4. ArrhythmiaPVCs; VT-NS VT-S; VF Heart DiseaseAbsentPresentPresent LV Dysfunction AbsentAbsentPresentPresent Potential Risks for SCD MinimalIntermediateHigh PVCs VT-NS Risk-Stratification for Sudden Cardiac Death PVC=premature ventricular complexes; VT-NS=nonsignificant ventricular tachycardia; VT-S=significant ventricular tachycardia; VF=ventricular fibrillation. Prystowsky EN. Am J Cardiol. 1988;61:102A-107A.

5. CAST: Survival CAST Investigators. N Engl J Med. 1989;321:406-412. P=0.0003 Survival (%) 100 95 90 85 0400 450 50050100150200250300350 Days After Randomization Placebo (N=725) Encainide or flecainide (N=730)

6. Julian DG, et al. Lancet. 1997;349:667-674. EMIAT: All-Cause Mortality LVEF and by Group Months Since Randomization Probability of Survival Amiodarone Placebo Ejection fraction < 30% Ejection fraction 31%-40%

7. CAMIAT: All-Cause Mortality and Nonarrhythmic Death Cairns JA, et al. Lancet. 1997;349:675-682. Months Since Randomization Cumulative Risk (%) Months Since Randomization Cumulative Risk (%) P=0.072 P=0.130 Amiodarone Placebo

8. Primary Prevention Post-MI Trials 1.Buxton AE, et al. N Engl J Med. 1999;341:1882-1890. 2.Moss AJ, et al. N Engl J Med. 1996;335:1933-1940. 3.Moss AJ, et al. N Engl J Med. 2002;346:877-882. 0 10 20 30 40 50 60 70 80 MUSTT 1 27 Months MADIT 2 27 Months MADIT-II 3 20 Months Mortality Reduction w/ICD Rx (%) 55 54 31

9. MUSTT Randomized Patients: Total Mortality Event-Free Rate P<0.001 EP ICD Control Months After Enrollment EP no ICD Buxton AE, et al. N Engl J Med. 1999;341:1882-1890. 0.0 0.1 0.2 0.3 0.4 0.5 0.6 0.7 0.8 0.9 1.0 03691215182124273033363942454851545760

10. MADIT: Survival by Treatment Groups Moss AJ, et al. N Engl J Med. 1996;335:1933-1940. Months After Enrollment Probability of Survival ICD Conventional Therapy P=0.009 0.0 0.2 0.4 0.6 0.8 1.0 03691215182124273033363942454851545760

11. MADIT-II: Survival by Treatment Group Moss AJ, et al. N Engl J Med. 2002;346:877-882. 0.78 0.69 P=0.007 01234 Defibrillator Group Conventional Group Probability of Survival Years 0.5 0.6 0.7 0.8 0.9 1.0

12. Secondary Prevention Trials: AVID, CASH, CIDS 1.AVID Investigators. N Engl J Med. 1997;337:1576-1583. 2.Kuck KH, et al. Circulation. 2000;102:748-754. 3.Connolly SJ, et al. Circulation. 2000;101:1297-1302. 0 10 20 30 40 50 60 70 80 AVID 1 3 Years CASH 2 3 Years CIDS 3 3 Years Mortality Reduction w/ICD Rx (%) 31 28 20

13. AVID: Overall Survival 01 23 Years After Randomization Defibrillator Group Antiarrhythmic Drug Group Proportion Surviving P<0.02 AVID Investigators. N Engl J Med. 1997;337:1576-1583. 0.0 0.2 0.4 0.6 0.8 1.0

14. CIDS Update: 11-Year Follow-Up ICD Amiodarone 100 80 60 40 20 0 20 40 60 80 100 120 140 P=0.021 Months Actuarial Survival (%) Bokhari FA, et al. Circulation. 2002;106(19 suppl II):II-497.

15. Primarna prevencija iznenadne srčane smrti kod dece Hipertrofična ili Dilatativna CMP Faktori rizika: 1. Istorija sinkopa u porodici 2. Značajna hipertrofija LK 3. NSVT na holteru 4. Pad TA prilikom testa opterećenjem 2 od 4 ICD From SURGERY for CONGENITAL HEART DEFECTS, 3rd Ed., Editors J STARK, M. de LEVAL and VT TSANG

16. Primarna prevencija iznenadne srčane smrti kod dece Long QT sy. (LQTS), koji ne reaguje na beta- blokatore ili ne mogu da se uzimaju. From SURGERY for CONGENITAL HEART DEFECTS, 3rd Ed., Editors J STARK, M. de LEVAL and VT TSANG

17. Primarna prevencija iznenadne srčane smrti kod dece Long QT sy. (LQTS) = Polimorfna VT From SURGERY for CONGENITAL HEART DEFECTS, 3rd Ed., Editors J STARK, M. de LEVAL and VT TSANG

18. Primarna prevencija iznenadne srčane smrti kod dece CPVT (kateholamin-senzitivna (ili zavisna) polimorfna VT). From SURGERY for CONGENITAL HEART DEFECTS, 3rd Ed., Editors J STARK, M. de LEVAL and VT TSANG

19. Primarna prevencija iznenadne srčane smrti kod dece Brugada sy. From SURGERY for CONGENITAL HEART DEFECTS, 3rd Ed., Editors J STARK, M. de LEVAL and VT TSANG Genetska bolest koju karakteriše abnormalan EKG i povišen rizik za iznenadnu srčanu smrt. Celularna električka aktivnost izmenjena zbog izlaska natrijuma iz ćelija.

20. ISS kod adolescenata i mladih sportista Cardiomyopathies Dilated Hypertrophic Right ventricular dysplasia Electrical abnormalities Long-QT syndromes Brugada syndrome W-P-W syndrome n Viral myocarditis n Abnormal coronary arteries n Drug-induced arrhythmias n Inflammatory/ infiltrative diseases n Idioventricular fibrillation n Diseases of heart valves From Ventricular Arrhythmias and Sudden Cardiac Death. Edited by Paul J. Wang, Chapter 17, Epidemiology and etiologies of sudden cardiac death, Keane K. Lee et al, 2008

21. 0–1 d94–155 1–3 d92–158 3–7 d90–166 7–30 d107–182 1–3 mo120–179 3–6 mo106–186 6–12 mo108–168 1–3 yr90–152 3–5 yr73–137 5–8 yr64–133 8–12 yr63–130 12–16 yr61–120 From Davignon A, Rautaharju P, Boisselle E, et al: Normal ECG standards for infants and children. Pediatr Cardiol 1:123-152, 1979. Normalna srčana frekvenca kod dece

22. Srčana fr. i intervali kod dece Godine Fr PR interval QRS interval 1 - 3 nedelje 100 - 180.07 -.14.03 -.07 1 - 6 meseca 100 - 185.07 -.16.03 -.07 6 - 12 meseci 100 - 170.08 -.16.03 -.08 1 - 3 godine 90 - 150.09 -.16.03 -.08 3 - 5 godine 70 - 140.09 -.16.03 -.08 5 - 8 godine 65 - 130.09 -.16.03 -.08 8 - 12 godine 60 - 110.09 -.16.03 -.09 12 - 16 godina 60 - 100.09 -.18.03 -.09 Adapted from: Benson, D.W. (1989). The normal electrocardiogram. In G.C. Emmanouilides, H.D. Allen, T.A. Riemenschneider, & H.P. Gutgesell (Eds.), Moss and Adams heart disease in infants, children, and adolescents (5th ed.) (pp. 152-164). Baltimore: Williams & Wilkins.

23. An ICD should be implanted in pediatric survivors of a cardiac arrest when a thorough search for a correctable cause is negative and the patients are receiving optimal medical therapy and have reasonable expectation of survival with a good functional status for more than 1 year. Hemodynamic and EP evaluation should be performed in the young patient with symptomatic, sustained VT. ICD therapy in conjunction with pharmacological therapy is indicated for high-risk pediatric patients with a genetic basis (ion channel defects or cardiomyopathy) for either SCD or sustained ventricular arrhythmias. The decision to implant an ICD in a child must consider the risk of SCD associated with the disease, the potential equivalent benefit of medical therapy, as well as risk of device malfunction, infection, or lead failure and that there is reasonable expectation of survival with a good functional status for more than 1 year. VA & SCD in Pediatric Patients

24. ICD therapy is reasonable for pediatric patients with spontaneous sustained ventricular arrhythmias associated with impaired (LVEF of 35% or less) ventricular function who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than 1 year. Ablation can be useful in pediatric patients with symptomatic outflow tract or septal VT that is drug resistant, when the patient is drug intolerant or wishes not to take drugs. VA & SCD in Pediatric Patients

25. Pharmacological treatment of isolated PVCs in pediatric patients is not recommended. Digoxin or verapamil should not be used for treatment of sustained tachycardia in infants when VT has not been excluded as a potential diagnosis. Ablation is not indicated in young patients with asymptomatic NSVT and normal ventricular function. VA & SCD in Pediatric Patients

26. Epikardno ili transvenski Indikacije za permanentu epikardnu stimulaciju u dece. < 8 kg Desno-levi šant Problem sa venama Više tipova hirurških rešavanja srčanih mana podrazumevaju prethodnu implantaciju pejsmejkera Opstrukcija VCS Težak pristup DK Mehanička trikuspidna valvula Neuspeli transvenski pristup From SURGERY for CONGENITAL HEART DEFECTS, 3rd Ed., Editors J STARK, M. de LEVAL and VT TSANG

27. Epikardno vs. Endokardno Epicardial Advantages Avoiding concerns of Venous Thrombus Disadvantages Having to enter the chest cavity Poor pacing and sensing thresholds Transvenous Advantages Avoid Thoracotomy Lower Pacing Thresholds Lower incidence of Exit Block Disadvantages Higher Dislodgement Rates Potential for Venous Occlusion Risk for Embolic Vascular Event Risk of Subclavian Crush Endocarditis Potential for Tricuspid Valve damage From presentation on Pediatric Pacing by Christine Youngs.

28. Comparison of epicardial with endocardial electrode use in children. Note the gradually increasing use of endocardial electrodes. (Data from the Midwest Pediatric Pacemaker Registry.)

29. White ring on 4968 identifies cathode leg Suture-down holes 4968 4965 Surgical Approach a. Subxiphoid b. Left Lateral Thoracotomy* c. Median Sternotomy d. Other approaches include: Subcostal Right Thoracotomy

30. 3830 n 4.1 french lumen-less catheter-delivered lead  C315 delivery catheters for 3830 leads Inner diameter 5.5 Fr Outer diameter 7.0 Fr “Pacemaker leads with smaller body design may help in preservation of venous patency in children.” Implantation of SelectSecure Leads in Children, PACE 7/07 VOLKAN TUZCU, M.D.

31. Extra lead slack is usually left in the pediatric patient’s atrium to allow for growth Adhesions can still form preventing lead slack from helping Following slides show slack being taken up as patient grows Graphic From SURGERY for CONGENITAL HEART DEFECTS, 3rd Ed., Editors J STARK, M. de LEVAL and VT TSANG

32. From Cardiac Pacing, Defibrillation, and Resychronization: A Clinical Approach, 2008, 2nd Ed., pg. 187, Daniel L. Hayes and Paul A. Friedman

34. From Extracardiac ICD implantation in an infant, T. Kriebel et al., Zeitschrift für Kardiologie, Band 94, Heft 6 (2005)

35. Chest radiograph showing placement of a single-coil transvenous defibrillator system with the lead tunneled down the lateral chest wall to the device, which also serves as the second defibrillation electrode and is placed in a left upper quadrant abdominal pocket. Use of Transvenous Lead tunneled to abdominally placed ICD

36. Programiranje ICD u dece ICD VR, zbog veličine Jedna zona detekcije, jer su najčešće indikacije kod dece VF, a ne VT (re-entry) “Pain-free” studija nije studirana kod dece. Max. SVT diskriminator Jedan elektrodni vodič da se izbegne venska opstrukcija

37. Case Report: 2001 14 god, ženski pol Abdominalni ICD sa 10 god. Indikacija: VT 170, operisana ToF sa 2 god. Intravenska lektroda + subkutani “pač” u aksili

38. Age 10 ICD Subcut. patch

39. On the age 14

40. Age 14

41. Subcutaneous lead Pg

42. Jap. Circulation, 2005

43. Zaključak Najčešća indikacija primarna prevencija Sekundarna prevencija kod operisanih USM ICD na tržišitu primenjivi za decu Često korišćenje epikardnih i supkutanih elektroda Programiranje drukčije nego kod odraslih