Benign RVOT Ectopy and RV dysplasia

Dr Graham Stuart

Heart Rhythm CongressBirmingham October 2009

How to

distinguish between.......

in the child.....

Is this dangerous?

14yr old boy asymptomaticirregular pulse picked up by GP

Benign RVOT ectopy vs RV Dysplasia ?

• Definitions• Differences RV ectopy vs ARVC

• Differences child vs adult vs athlete

• Practical approach…..

Benign RVOT ectopy vs RV Dysplasia ?

• Definitions• Differences RV ectopy vs ARVC

• Differences child vs adult vs athlete

• Practical approach…..

Benign RVOT ectopy

Benign RVOT ectopy

“Non-familial benign condition that occurs in young individuals without structural heart disease” Corrado et al 2008

Definition

J Am Coll Cardiol 2008;51(7):731-9

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.

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

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

Benign RVOT ectopy

Type 2Type 1

Most have runs of PVCs

non-sustained VT in 70% 20% > 5 beats

Kim RA et al Clinical and electrophysiological spectrum of idiopathic ventricular outflow tract arrhythmias JACC 2007;49:2035-43

Benign RVOT ectopy

“Non-familial benign condition that occurs in young individuals without structural heart disease” Corrado et al 2008

Definition

J Am Coll Cardiol 2008;51(7):731-9

Benign RVOT ectopy Lesch M et al Paroxysmal ventricular tachycardia in the absence of organic heart disease. Ann Int Med 1967;66:950-60Pederson DH,et al Ventricular tachycardia and ventricular fibrillation in a young populationCirculation 1979;60;988-92 Corrado D et al Sudden death in young competitive athletes: clinicopathological correlations.Am J Med 1990;89:588-96.Grimm W et al Reversal of tachycardia induced cardiomyopathy following ablation of repetitive monomorphic RVOT tachycardia Pace Clin Electrophysiol 2001;24(2):166-171Niwano S et al prognostic significance of frequent premature ventricular contractions originating from the outflow tract in patients with normal left ventricular function Heart 2009;95:1230-7

Syncope 10%

LV dysfunction 10%

sudden death...

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

RV Dysplasia

RV Dysplasia

“progressive, often inherited cardiomyopathycharacterised by fibrofatty replacement of RVmyocardium…arrhythmias and sudden death....especially in young people and athletes”

Corrado et al 2008

Definition

J Am Coll Cardiol 2008;51(7):731-9

RV DysplasiaAetiology / Mechanism

• Disorder of the desmosome

Desmosomescell adhesion proteins specialised for cell-cell adhesion Wikipedia 2009

5 components

Deletions = ARVC

RV DysplasiaAetiology / 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

ARVC Task Force CRITERION MAJOR eg MINOR egRV Dysfunction Localised RV aneurysm Mild segmental dilatationTissue wall characterisation

Fibrofatty replacement of RV wall on biopsy

Repolarisation abn. Inverted T waves V2/V3 (>12yrs)

Depolarisation abn. Epsilon waves/QRS> 110ms in V1/2

Late potentials

Arrhythmia LBBB VT/ > 1000/ 24hr VE’s

Family History Necropsy/biopsy confirmed

Clinical diagnosis or SD < 35yrs

McKenna et al 1994 Br Heart J

Diagnosis of ARVC

2 major or

1 major + 2 minor or

4 minor

Limitations of Task Force Diagnosis

• Insensitive for relatives– 67 patients Hamid JACC 2002

• 298 relatives; 29 had ARVC • 19/67 families = familial (28%) • But additional 11% had minor cardiac abnormalities

ConclusionNeed to broaden criteria to reflect minor disease in relatives AD var pen

Limitations of Task Force Diagnosis

• Insensitive for relativesHamid JACC 2002: 40:1445-50

• LV involvement may precede RV Sen Chowdhry et al Circulation 2007;115:1710-20

• In early stages RV function may be normalMarcus et al Heart Rhythm 2009;6:984-992

New diagnostic tests proposed......

“Accordian” sign

Focal Focal ““crinklingcrinkling””of RVOT of RVOT

60% of mutation carriers60% of mutation carriers0% of non0% of non--carrierscarriers

Dalal et al J Am Coll Cardiol 2009;53:15:1289-99

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;360:1075- 84

Benign RVOT ectopy vs RV Dysplasia ?

• Definitions• Differences RV ectopy vs ARVC

• Differences child vs adult vs athlete

• Practical approach…..

Conventional criteria

• SAECG• MRI• RV Biopsy• RV angiography• VT is monomorphic in RVOT VT

Normal in RVOT VT

RVOT VT vs ARVC

QRS duration is longer QRS duration is longer in ARVC vs RVOT VTin ARVC vs RVOT VT

Algorith of QRSD + axisAlgorith of QRSD + axis

Ainsworth et al Heart Rhythm 2006;3:416Ainsworth et al Heart Rhythm 2006;3:416--423423

RVOT VT vs ARVCCan 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

RVOT VT vs ARVC Electroanatomic Mapping

ResultsAbnormal < 0.5mV N = 7Correlated with biopsy ( fatty change)43% required ICD over next 4yrs

Benign RVOT ectopy vs RV Dysplasia ?

• Definitions• Differences RV ectopy vs ARVC

• Differences child vs adult vs athlete

• Practical approach…..

Benign RVOT VTepidemiology

Adult literature Issa et al 2009

• 10% of VT referred to electrophysiologist• Age at presentation: 30-50yrs• Female > Male

No data !

90%10-15yrs

? M>F

ARVC Epidemiology

Circulation 2003:108:3000-3005

15% deaths 15% deaths < 18yrs< 18yrs

ARVC Epidemiology

ARVC Epidemiology

Children referred to arrhythmia clinic Children referred to arrhythmia clinic with LBBBVT / +ve family historywith LBBBVT / +ve family history

Hamilton RM PACE 2009;32:S44-51

Exercise tests in Children• Relationship unclear Sequeri Am J Cardiol 2009;104:411-3

– 33 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;114:1799-806

Benign RVOT ectopy vs RV Dysplasia ?

• Definitions• Differences RV ectopy vs ARVC

• Differences child vs adult vs athlete

• Practical approach…..

Practical Approach........

Evidence free Zone !!

14yr old boy asymptomaticirregular pulse picked up by GP

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

RVOT ectopy, normal echo

Jonathan 15yrsCalvin 14yrs Joshua 15yrscounty football PICU / S-ICD Cardiac Tx

12 / 12 map rvot vt

Miss CE

• Normal examination – No murmurs, BP 130/70– ECG– Echocardiogram

ECHOCARDIOGRAM Miss CE

ECHOCARDIOGRAM

Exercise Test Miss CEStage 1

Exercise Test Miss CEStage 2

Exercise Test Miss CEPeak Exercise

Exercise Test Miss CERecovery

Exercise Test Miss CERecovery

Loop RecorderMedtronic Reveal DX

• Reveal DT implanted – L prepectoral– 12/3/09

• Lots of symptoms– Weakness– Feels unwell– Woozy– No near syncope /

collapse

Reveal PrintoutsReveal Printouts

•• No typical No typical symptoms (yet)symptoms (yet)

•• Frequent Frequent artefact (including    artefact (including    loss of signal)loss of signal)

S-ICD JT