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ARVD/C Who Needs an ICD?

John D. Fisher MD, FACC, FESC

Professor of Medicine

Albert Einstein College of MedicineBronx, New York, USA

Director, Arrhythmia Service

Montefiore Medical Center and

Albert Einstein College of Medicine

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Its good to be in France at ECAS!

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Diagnosis and Arrhythmias

We have heard from Drs. Schalij andMarcus on these topics.

Assume that the diagnosis is clear or,perhaps just tentative. What to do then?

Some vignettes.

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ACC/AHA/NASPE(HRS) Guidelines

Somewhat vague.

Class I-IIb depending on interpretation of the

patients history (and interp of the guidelines).ARVD is covered in the discussion, but not in

the actual guidelines, which mention familialconditions such as LQTS, from which onemight extrapolate to ARVD/C, especially ifassociated with a family history, syncope,documented tachycardia, (RV angio, SAECG,

biopsy).

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Case F 25

25 year old woman is admitted to thehospital after syncope that occurred while

playing tennis. Two prior syncopal spells. Emergency team found runs of

monomorphic VT, LBBB-LAD, terminatingspontaneously.

Fathers brother and a grandmother died

suddenly at age

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Case F 25 (Continued-1)

ECG in sinus rhythm: inverted T-waves V1-

2-3, and slightly wider QRS in V1-2-3. Small

high frequency (epsilon) waves at the endof the QRS.

SAECG positive in all 3 parameters.

RV angiogram: Localized dilatationparticularly apex & outflow, with

cauliflower appearance.

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Biopsy of septum near apex: much fibro-fatty infiltration.

MRI: widespread fatty infiltrationextending to the LV, consistent with

ARVD/C.

EPS: inducible VT of several differentmonomorphic LBBB morphologies.

Exercise test: Runs of VT-NS.

Is an ICD indicated?

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Case F 30

Mother has ARVD/C (MRI, biopsy, ECG, RVangio, spontaneous LBBB VT), has an ICD and

takes amio. Patient has multiple episodes of sustained VT,

LBBB-LAD, well tolerated, somewhatsuppressed on AADs, for 10 years (now 40

y.o.).

Normal ECG, echo, RV angio, and serial MRIs.Ablation failed years ago; refuses repeat.

Should she have an ICD?

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Case M 72

M 72 is in good health. He exercisesregularly and vigorously without

symptoms. Mild hypertension is well-controlled on -blockers. RV and LV EF,size, and function are normal.

A brother died in his sleep at 84.

The ECG shows somewhat flattened T-waves in V1-2-3, and one ECG wassuspected to have epsilon waves.

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Case M 72 (Continued-1)

The ECG also shows occasional VPCs of aLBBB morphology (asymptomatic).

A nuclear stress test is normal. Should other tests be done?

Does he need an ICD for a presumptive

diagnosis of ARVD/C based on his ECG?

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Case F 36

Healthy woman with very bothersomepalpitations especially with exercise.

Documented arrhythmias are limited to veryfrequent monomorphic VPCs. These areinterpreted as of RV origin.

No family history of early heart disease or

SCD. Echo read as typical MV prolapse with mild-

moderate MR.

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MRI read as diagnosticof ARVD/C.

EPS was negative (no VT induced) with a

moderate protocol (3 ES at 2 rates, 2 sites,and bursts), no isoproterenol.

No reduction in symptoms or VPCs with-blockers.No further testing at this point.

Is an ICD indicated? Contra-indicated?

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The cardiologist & EP felt that the stronginterpretation of the MRI, together with the

symptoms and ECG (VPCs) made it likelythat ARVD/C was the diagnosis.

(American doctors are very worried aboutgetting sued if there is a bad outcome that

may have been prevented by a treatmentsuch as an ICD).

An ICD was implanted.

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1 week later she developed pericarditis(ICD data & echo stable).

3.5 months after implant the pericarditis(never completely resolved) worsened.

Echo showed perforation.

ICD: EGM 1mV; no capture.

The ICD was removed.

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She was referred to a renowned hospital.

They repeated the MRI, and told her that if

it was positive for ARVD, they wouldrecommend.

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they would recommend an ICD.

The echo was normal (no MVP).

The MRI was equivocal. They offered

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They offered an ICD or ablation.

She refused both.

She was treated with mexiletine and -blockers, and has been symptomaticallyimproved for several years.

Repeat echo by original cardiologist: MVP.

Still some angst about the MRI. Worryabout whether she should have an ICD.

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Case F 36 (Continued-7): Notes

Multiple ECGs in NSR were normal. No T-wave or QRS abnormalities; no epsilon

waves. VPCs were 99% RBBB-RAD on multiple

tracings over time.

Are these typical for an RV origin or

ASRVD/C?

(VPCs seen by EMS are lost; may havebeen different).

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Case F 36 (Continued-8): Notes

Would other information have helpedmake the decision for or against an ICD?

SAECG. RV angiogram.

RV biopsy.

Frank Marcus??

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Conclusions

Sometimes the decision is easy.

Sometimes its not.

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Conclusions: Here and There

Americans are more likely to implant inequivocal cases because of litiginous climate.

This often trump concerns about paymentdenial.

ESC guidelines may offer some protection by

saying if you did whats recommended, you

did enough. I hope ECAS follows this pattern.

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All Done!

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ARVD ECAS 06