Benign RVOT Ectopy and RV dysplasia

Transcription

1 Heart Rhythm Congress Birmingham October 2009 How to distinguish between... Benign RVOT Ectopy and RV dysplasia in the child... Dr Graham Stuart

2 14yr old boy asymptomatic irregular pulse picked up by GP Is this dangerous?

3 Benign RVOT ectopy vs RV Dysplasia? Definitions Differences RV ectopy vs ARVC Differences child vs adult vs athlete Practical approach..

4 Benign RVOT ectopy vs RV Dysplasia? Definitions Differences RV ectopy vs ARVC Differences child vs adult vs athlete Practical approach..

5 Benign RVOT ectopy

6 Benign RVOT ectopy Definition Non-familial benign condition that occurs in young individuals without structural heart disease Corrado et al 2008 J Am Coll Cardiol 2008;51(7):731-9

7 Benign RVOT ectopy Aetiology / Mechanism Most adenosine sensitive Caused by camp mediated Delayed after depolarisations DADs Triggered activity 2 main types Zipes Lerman et al Clin Cardiol 2000;18:265 Berruezo et al Circulation 2004;109:1842 Iwai et al J Cardiovasc Electrophysiol 2006;17:1052.

8 Benign RVOT ectopy Type 1 Most common form 60-90% Repetitive bursts of tachycardia Interspersed with SR Frequent PVC, couplets, salvos Occurs at rest or following exercise ( usually...) Issa, Miller and Zipes 2009

9 Benign RVOT ectopy Type 2 less common form 10-20% Sustained episodes of tachycardia Precipitated by exercise or emotional stress Issa, Miller and Zipes 2009

10 Benign RVOT ectopy Type 1 Type 2 non-sustained VT in 70% 20% > 5 beats Most have runs of PVCs Kim RA et al Clinical and electrophysiological spectrum of idiopathic ventricular outflow tract arrhythmias JACC 2007;49:

11 Benign RVOT ectopy Definition Non-familial benign condition that occurs in young individuals without structural heart disease Corrado et al 2008 J Am Coll Cardiol 2008;51(7):731-9

12 Benign RVOT ectopy Lesch M et al Paroxysmal ventricular tachycardia in the absence of organic heart disease. Ann Int Med 1967;66: Pederson DH,et al Ventricular tachycardia and ventricular fibrillation in a young population Circulation 1979;60; Corrado D et al Sudden death in young competitive athletes: clinicopathological correlations. Am J Med 1990;89: Grimm W et al Reversal of tachycardia induced cardiomyopathy following ablation of repetitive monomorphic RVOT tachycardia Pace Clin Electrophysiol 2001;24(2): Niwano S et al prognostic significance of frequent premature ventricular contractions originating from the outflow tract in patients with normal left ventricular function Heart 2009;95: Syncope 10% LV dysfunction 10% sudden death...

13 Benign RVOT ectopy 17yr old cardiac arrest during removal of finger nails!

14 RV Dysplasia

15 RV Dysplasia Definition progressive, often inherited cardiomyopathy characterised by fibrofatty replacement of RV myocardium arrhythmias and sudden death...especially in young people and athletes J Am Coll Cardiol 2008;51(7):731-9 Corrado et al 2008

16 RV Dysplasia Aetiology / Mechanism Disorder of the desmosome

17 Desmosomes cell adhesion proteins specialised for cell-cell adhesion Wikipedia components Deletions = ARVC

18 RV Dysplasia Aetiology / Mechanism Disorder of the desmosome Prevalence 1:1000-1:5000 Genetic diagnosis in 40-50% Diagnosis ARVC Task Force criteria Hamilton RM Arrhythmogenic RV cardiomyopathy PACE 2009;32:S44-51

19 ARVC Task Force CRITERION MAJOR eg MINOR eg RV Dysfunction Localised RV aneurysm Mild segmental dilatation Tissue wall characterisation Repolarisation abn. Depolarisation abn. Arrhythmia Family History Diagnosis of ARVC Fibrofatty replacement of RV wall on biopsy 2 major or Epsilon waves/qrs> 110ms in V1/2 Necropsy/biopsy confirmed McKenna et al 1994 Br Heart J Inverted T waves V2/V3 (>12yrs) Late potentials 1 major + 2 minor or 4 minor LBBB VT/ > 1000/ 24hr VE s Clinical diagnosis or SD < 35yrs

20 Limitations of Task Force Diagnosis Insensitive for relatives 67 patients Hamid JACC relatives; 29 had ARVC 19/67 families = familial (28%) But additional 11% had minor cardiac abnormalities Conclusion Need to broaden criteria to reflect minor disease in relatives AD var pen

21 Limitations of Task Force Diagnosis Insensitive for relatives Hamid JACC 2002: 40: LV involvement may precede RV Sen Chowdhry et al Circulation 2007;115: In early stages RV function may be normal Marcus et al Heart Rhythm 2009;6: New diagnostic tests proposed...

22 Accordian sign Focal crinkling of RVOT 60% of mutation carriers 0% of non-carriers Dalal et al J Am Coll Cardiol 2009;53:15:

23 Immunohistochemical Biopsy Improve biopsy Test for plakoglobulin ARVC vs controls PPV 83% NPV 90% Irrespective of biopsy site Asimaki et al New Engl J Med 2009;360:

24 Benign RVOT ectopy vs RV Dysplasia? Definitions Differences RV ectopy vs ARVC Differences child vs adult vs athlete Practical approach..

25 Conventional criteria SAECG MRI RV Biopsy Normal in RVOT VT RV angiography VT is monomorphic in RVOT VT

26 RVOT VT vs ARVC QRS duration is longer in ARVC vs RVOT VT Algorith of QRSD + axis Ainsworth et al Heart Rhythm 2006;3:

27 RVOT VT vs ARVC Can 3D mapping distinguish RVOT VT and ARVC? Corrado et al J Am Coll Cardiol 2008;51(7):731-9 N= 27 ( 15 male) undergoing EPS All RVOT VT Normal echo / MRI/ angio

28 RVOT VT vs ARVC Electroanatomic Mapping Results Abnormal < 0.5mV N = 7 Correlated with biopsy ( fatty change) 43% required ICD over next 4yrs

29 Benign RVOT ectopy vs RV Dysplasia? Definitions Differences RV ectopy vs ARVC Differences child vs adult vs athlete Practical approach..

30 Benign RVOT VT epidemiology Adult literature Issa et al % of VT referred to electrophysiologist Age at presentation: 30-50yrs Female > Male? M>F 10-15yrs 90% No data!

31 ARVC Epidemiology 15% deaths < 18yrs Circulation 2003:108:

32 ARVC Epidemiology

33 ARVC Epidemiology Children referred to arrhythmia clinic with LBBBVT / +ve family history Hamilton RM PACE 2009;32:S44-51

34 Exercise tests in Children Relationship unclear Sequeri Am J Cardiol 2009;104: exercise tests in 16 children Ectopy increased in 6 decreased in 5 Endurance exercise accelerates changes in plakoglobulin def. Mice Kirchhof et al Circulation 2006;114:

35 Benign RVOT ectopy vs RV Dysplasia? Definitions Differences RV ectopy vs ARVC Differences child vs adult vs athlete Practical approach..

36 Practical Approach... Evidence free Zone!!

37 14yr old boy asymptomatic irregular pulse picked up by GP

38 Practical Approach History & Examination & family history ECHO 24hr ECG / SAECG / exercise test MRI If normal - reassure ( but follow up) If abnormal - consider 3D map guided biopsy ICC discussion re genetic testing

39 RVOT ectopy, normal echo Calvin 14yrs Jonathan 15yrs Joshua 15yrs county football PICU / S-ICD Cardiac Tx

40 12 / 12 map rvot vt

41 Miss CE Normal examination No murmurs, BP 130/70 ECG Echocardiogram

42 ECHOCARDIOGRAM Miss CE

43 ECHOCARDIOGRAM

44 Exercise Test Miss CE Stage 1

45 Exercise Test Miss CE Stage 2

46 Exercise Test Miss CE Peak Exercise

47 Exercise Test Miss CE Recovery

48 Exercise Test Miss CE Recovery

49 Loop Recorder Medtronic Reveal DX Reveal DT implanted L prepectoral 12/3/09 Lots of symptoms Weakness Feels unwell Woozy No near syncope / collapse Reveal Printouts No typical symptoms (yet) Frequent artefact (including loss of signal)

50 S-ICD JT