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ARVD/ARVC

ARVD/ARVC. Monique Bosman AIOS-SEH 4-2-2013. Inleiding. Casus Ziektebeeld Pathogenese Diagnose Behandeling Literatuur. Casus. Jongeman, 17 jaar RvK/ collaps VG/ ADHD, migraine Med / Strattera 2dd 40 en 12 mg, Immigran zn A LO Fam : neef WPW. Casus: ECG. Casus: scoop.

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ARVD/ARVC

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  1. ARVD/ARVC Monique Bosman AIOS-SEH 4-2-2013

  2. Inleiding • Casus • Ziektebeeld • Pathogenese • Diagnose • Behandeling • Literatuur

  3. Casus • Jongeman, 17 jaar • RvK/ collaps • VG/ ADHD, migraine • Med/ Strattera 2dd 40 en 12 mg, Immigranzn • A • LO • Fam: neef WPW

  4. Casus: ECG

  5. Casus: scoop

  6. Aritmogene Rechter Ventrikel Cardiomyopathie • Vervetting en fibrosering myocardweefsel • RV: apex, uitstroombaan en vrije wand • Meest aangedaan: net onder tricuspidaliskleppen • Gevolg: RV dilatatie en wandbewegingsstoornissen

  7. ARVC: ziektebeeld • Leidend tot: • Ventriculaire aritmiëen met origine van rechter ventrikel • Acute hartdood (SCD)  onbekend, schatting: 15-25% • Palpitatieklachten, duizeligheid, syncope • Later stadium: tekenen van hartfalen

  8. ARVC: ziektebeeld • ARVD oude term • World Health Organisation/ International Society andFederation of Cardiology • Jonge mensen en athleten • Mannen > Vrouwen • Geen symptomen tijdens vroege kindertijd of onder de leeftijd van 60 jaar

  9. ARVC: pathogenese • Diffuus of segmentaal verlies van myocardweefsel van het rechter ventrikel wat wordt vervangen voor fibrose en vetweefsel • Vaak transmuraal waardoor aneurysmatische dilataties van de diaphragmatisch, apicaal en infundibulaireregionen  Triangle of dysplasia(50% bij obductie)

  10. ARVC: pathogenese

  11. ARVC: pathogenese • 1:2000 tot 1:5000 • Wisselende kennis over de ziekte • Moeilijk te detecteren, aandacht bij obductie: septum bijna nooit aangedaan • Autosomaal dominant overerfbaar • 9 chromosomale afwijkingen tot dusver • Ziekte van Naxos: autosomaal recessief

  12. ARVC: diagnose • Meest voorkomend: • Ventriculaire aritmie met LBTB morfologie • ECG depolarisatie/repolarisatie veranderingen in precordiale leads • Dysfunctie en structurele veranderingen van het rechter ventrikel Echter: asymptomatisch komt ook voor

  13. ARVC: diagnose

  14. ARVC: diagnose • Major en minor critera ontwikkeld door European Society of Cardiology/ International Society andFederation of Cardiology • 2 major criteria of • 1 major criteria + 2 minor criteria of • 4 minor criteria

  15. ARVC: diagnose

  16. ARVC: diagnose

  17. ARVC: diagnose

  18. ARVC: diagnose

  19. ARVC: diagnose

  20. ARVC: diagnose

  21. ARVC: diagnose

  22. ARVC: behandeling Bij verdenking verrichten: ECG, Holter 24 uur, inpanningstest, signalaverage ECG • Medicatie: Sotalol, Amiodaron • Catheter ablatie • ICD-implantatie

  23. ARVC: behandeling

  24. ARVC: behandeling • ACC/AHA/ESC 2006 Guidelinesfor Management of PatientsWithVentricularArrhythmiasand Prevention of SuddenCardiacDeath • Aanbevelingen: • Class 1: • ICD implantation is recommendedfor the prevention of SCD in patientswith ARVC withdocumentedsustained VT of VF who are recievingchronicoptimalmedicaltherapyandwho have reasonableexpectation of survival with a goodfunctional status for more than 1 year (LoE B)

  25. ARVC: behandeling • ClasIIa: • 1. ICD implantationcanbeeffectivefor the prevention of SCD in patientswith ARVC withextensivedisease, inclusingthosewith LV involvement, 1 or more affected family member with SCD, or undiagnosed syncope when VT or VF has not been excluded as the cause of syncope, who are recievingchronicoptimalmedicaltherapy, andwho have reasonableexpectation of survival with a goodfunctional status for more than 1 year. (LoE C)

  26. ARVC: behandeling • 2. Amiodarone or sotalolcanbeeffectivefor treatment of sustained VT of VF in patientswith ARVC when ICD implantation is notfeasabe (LoE C) • 3. Ablationcanbeuseful as adjunctivetherapy in management of patientswith ARVC withrecurrent VT, despiteoptimalantiarrhytmic drug therapy (LoE C) • Class IIb: • EP testingmightbeusefulfor risk assesment of SCD in patientswith ARVC (LoE)

  27. ARVC: Casus • Vragen?

  28. Literatuur • Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular cardiomyopathie: diagnosis, prognosisand treatment. Education in Heart. 2000; 83:588-595 • Paul M, Wichter T, Fabritz L, Waltenberger J, Schube-Bahr E, Kirchhof P. Arrhythmogenic right ventruicular cardiomyopathie: An update on pathophysiology, genetics, diagnosis, and risk stratification. HerzschrElektrophys. 2012 • McKenna WJ, Thiene G, Nava A ea. Diagnosis of arrhythmogenic right ventriculardysplasia/ cardiomyopathie. Br Heart J 1994; 71: 215-218 • Zipes DP, Camm AJ ea. ACC/AHA/ESC 2006 Guidelinesfor Management of PatientsWithVnetricularArrhythmiasand the Prevention of SuddenCardiacDeath. Journal of the American College of Cardiology. 2006; vol 48, nr 5 • www.ECGpedia.nl: ARVC • www.cardiogenetica.nl • Nasir K, Bomma C, Tandri H ea. Electrocardiographic Features of Arrhythmogenic Right VentricularDysplasia/CardiomyopathyAccordngtoDiseaseSeverity: A NeedtoBroadenDiagnostic Criteria. Circulation. 2004; 110: 1527-1534 • HulotJ, Jouven X, Empana J ea. Natural Historyand Risk Stratification of Arrhythmogenic Right VentricularDysplasia/ Cardiomyopathy. Circulation. 2004; 110: 1879-1884 • Dalal D, Nasis K, Bomma C ea. Arrhythmogenic Right VentricularDysplasia: A United StatesExperience. Circulation. 2005; 112: 3823-3832

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