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Presenter Dr Praveen Gupta

Moderator Dr Raja Selvaraj 

MD(Ped) DNB(Card) Fellowship in Cardiac EP(Toronto)

Cardiac Electrophysiologist

Associate Professor of Cardiology

Jawaharlal Institute of Postgraduate Medical Education and Research

Pondicherry | India 605006

Date 19/12/2016

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BACKGROUND

Ventricular arrhythmias are characteristic of arrhythmogenic right ventricular

cardiomyopathy (ARVC)

Patients may have ventricular fibrillation (VF) resulting in sudden cardiac death

(SCD)

This complication is a basis for the recommendation of implantable cardioverter

defibrillator (ICD)

There is a significant complication of ICDs that are implanted for many years

Risk stratification to identify patients who need an ICD to prevent SCD would be

useful clinical information

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BACKGROUND

Of the 458 patients with nonischemic cardiomyopathy who were randomized to

ICD treatment versus no ICD implantation, the number of shocks delivered was

greatly in excess of lives saved as compared with controls without ICDs

The excess estimation of lives saved by ICD therapy may be even greater in

patients with ARVC who have normal left ventricular (LV) function and can

tolerate VT at rates of >200 beats/min

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BACKGROUND

An alternative approach to risk stratification is to study patients with a

definite diagnosis of ARVC who were not implanted with an ICD and to

analyze their presenting clinical features and subsequent risk of arrhythmic

death. This is the approach used in this study

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METHODS

Data from 88 probands with definite ARVC based on the modified Task

Force Criteria who did not receive ICDs were analyzed and their clinical

features were evaluated to determine risk stratification

These were patients from the

Multidisciplinary Study of Right Ventricular Dysplasia, funded by the

NIH

Familial Cardiomyopathy Registry of Trieste, Italy

Registry from the University Medical Center of Utrecht, the Netherlands

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METHODS

88 probands were 60 men and 28 women

Medical and family history, physical examination, electrocardiogram

(ECG), Holter monitoring ECG, and echocardiogram were performed on

all index patients

Arrhythmias recorded either on ECG or 24-/48-h Holter monitoring

Data analyzed separately for each center because there was a marked

difference in the percent of patients who received ICDs among the centers

The analysis was also performed with the combined data

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METHODS

The NIH-sponsored Multidisciplinary Study of Right Ventricular Dysplasia

Prospective study from 2000 to 2008 that enrolled 137 probands with

newly diagnosed ARVC

Twenty-eight patients (20%) did not receive an ICD

Of those, 22 were included in the study

Six other enrolled probands without an ICD met the original Task Force

Criteria but did not meet the Modified Task Force Criteria and were

excluded from this analysis

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METHODS

82 probands from the Familial Cardiomyopathy Registry, Trieste, Italy

Fifteen received ICDs

67 patients (82%) did not receive ICDs,

13 were excluded from further analysis, did not meet the Modified Task

Force Criteria, 1 patient died <2 months after enrollment,from progressive

heart failure, and 3 were lost to follow-up

54 patients did not receive an ICD because they were enrolled before the

ICDs became available, before the current guidelines were published, or

because they underwent heart transplantation

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METHODS

The University Medical Center at Utrecht had 49 probands

36 with an ICD and 13 (27%) without an ICD

One of the 13 had no follow-up

There were 12 probands with ARVC who met Modified Task Force

Criteria and who did not receive an ICD

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STATISTICAL ANALYSIS

Cox proportional hazards model regression with survival data were used to

identify variables associated with mortality

SAS/STAT version 9.4 (SAS Institute, Cary, North Carolina)

SPSS statistical package, version 20, were used to calculate the various

statistics (IBM Corp., Armonk, New York)

Two-tailed tests were used in all cases with an alpha level of 0.05

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RESULTS

During the average

follow-up of 9.1 +_ 7.7

years, there were 12

deaths (14%) in the 88

probands without an

ICD (10 men and 2

women)

There were 5 deaths

attributed to ventricular

arrhythmias

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RESULTS There were no deaths during

follow-up in the

Multidisciplinary Study of

Arrhythmogenic Right

Ventricular Dysplasia

There were 10 deaths during

follow-up in the Familial

Cardiomyopathy Registry,

Trieste, Italy

During follow-up at the

University Medical Center at

Utrecht, there was 1 arrhythmic

death

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RESULTS

Of 52 patients who had

episode of VT (sustained

or nonsustained VT),

there were 5 arrhythmic

deaths

Of 36 patients who not

have arrhythmia, there

were no arrhythmic causes

of death

One of these patients died

of congestive heart failure

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RESULTS

Relationship between index VT and

all-cause mortality was close but not

statistically significant (p ¼ 0.052),

Overall likelihood test was significant

(p ¼ 0.012).

Effect for site small (0.015) & not

significant (p ¼ 0.099)

Odds for VF & unknown VT difficult

to estimate

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RESULTS

• Among the 5 patients who

had an arrhythmic death, the

left ventricular ejection

fraction (LVEF) at baseline

was significantly lower (p ¼

0.04) than those who did not

have an arrhythmic death

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RESULTS

• No relation between mortality and syncope before diagnosis, sex , age at

diagnosis, inducibility at electrophysiology study, extent of T-wave

inversion , index QRS duration or beta-blockers for the 76 survivors and

12 who died during follow-up

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DISCUSSION

Risk of death in patients with ARVC who have decreased LVEF

Sustained or nonsustained VT risk factor

No arrhythmic deaths in patients who did not have clinical sustained or

nonsustained VT

LV function should be considered in the discussion of whether patients

may need an ICD to prevent an arrhythmic death

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STUDY LIMITATIONS

Antiarrhythmic or beta-blocker use skewed the results

A limitation, of this study is the analysis of outcomes in a highly selected

population

Family members with ARVC were not included

The reasons the ICDs were not implanted was not documented

systematically

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STUDY LIMITATIONS

Risk stratification based on spontaneous nonsustained VT and sustained

VT may change with time during the course of the disease and should be

reassessed to determine the presence of these risk factors

It is not known how frequently this should be done, but every 3 years

could be considered.

The results of this analysis need to be confirmed because the numbers of

patients are relatively few

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Take home message

Implantation of an ICD in heart failure not caused by ischemic heart disease did

not provide an overall survival benefit, although the risk of sudden cardiac death

was halved with an ICD

No difference of ICD between patients with CRT and without CRT

Younger patients have a survival benefit with ICD

Patients at higher risk more likely to benefit from ICD

Risk of death in patients with ARVC who have decreased LVEF

Sustained or nonsustained VT risk factor for death in patient with ARVC

No arrhythmic deaths in ARVC who did not have clinical sustained or nonsustained

VT

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Thank you