Risk Stratification in Arrhythmic Right Ventricular Cardiomyopathy Without Implantable Cardioverter-Defibrillators trial published in JACC, Clinical electrophysiology on 5/11/2016, published by ELSEVIER

1. 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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2. 2

3. 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
3

4. 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
4

5. 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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6. 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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7. 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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8. 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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9. 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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10. 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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11. STATISTICALANALYSIS
 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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12. 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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13. 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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14. 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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15. 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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16. 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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17. 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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18. 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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19. 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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20. 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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21. 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