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Impact of the Revision of ArrhythmogenicRight Ventricular Cardiomyopathy/DysplasiaTask Force Criteria on Its Prevalence byCMR Criteria

Emmanuelle Vermes, MD,* Oliver Strohm, MD,* Akli Otmani, MD,†Helene Childs, BHSC,* Hank Duff, MD,† Matthias G. Friedrich, MD‡

Calgary, Alberta, Canada

O B J E C T I V E S The purpose of our study was to assess the impact of revised versus original criteriaon the prevalence of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) criteria incardiac magnetic resonance (CMR) studies.

B A C K G R O U N D Recently, the ARVC/D task force criteria have been revised, aiming for a betterdiagnostic sensitivity. The implications of this revision on clinical decision making are unknown.

M E T H O D S We retrospectively evaluated the CMR scans of 294 patients referred for ARVC/D between2005 and 2010, and determined the presence or absence of major and minor CMR criteria using the originaland the revised task force criteria. Previously, major and minor abnormalities were identified by the presenceof right ventricle dilation (global or segmental), right ventricle microaneurysm, or regional hypokinesis. Therevised criteria require the combination of severe regional wall motion abnormalities (akinesis or dyskinesisor dyssynchrony) with global right ventricle dilation or dysfunction (quantitative assessment).

R E S U L T S Applying the original criteria, 69 patients (23.5%) had major original criteria, versus 19patients (6.5%) with the revised criteria. Forty-three patients (62.3%) with major original criteria did notmeet any of the revised criteria. Using the original criteria, 172 patients (58.5%) had at least 1 minorcriterion versus 12 patients (4%) with the revised task force criteria; 167 patients (97%) with minororiginal criteria did not meet any of the revised criteria. In the subgroup of 134 patients with completediagnostic work-up of ARVC, 10 patients met the diagnosis of proven ARVC/D without counting imagingcriteria. Only 4 of 10 met major criteria according to the revised CMR criteria; none met minor criteria.However, 112 of 124 patients without ARVC/D were correctly classified as negative by major and minorcriteria (specificity 94% and 96%, respectively).

C O N C L U S I O N S In our experience, the revision of the ARVC/D task force imaging criteriasignificantly reduced the overall prevalence of major and minor criteria. The revision, althoughmaintaining a high specificity, may not have improved the sensitivity for identifying patients withARVC/D. Larger studies including follow-up are required. (J Am Coll Cardiol Img 2011;4:282–7) © 2011by the American College of Cardiology Foundation

From the *Stephenson Cardiovascular MR Centre at the Libin Cardiovascular Institute of Alberta, Department of CardiacSciences, University of Calgary, Calgary, Alberta, Canada; †Department of Cardiac Sciences, University of Calgary, Calgary,Alberta, Canada; and the ‡Stephenson Cardiovascular MR Centre at the Libin Cardiovascular Institute of Alberta,Departments of Cardiac Sciences and Radiology, University of Calgary, Calgary, Alberta, Canada. The authors have reportedthat they have no relationships to disclose.

Manuscript received October 21, 2010; revised manuscript received January 20, 2011, accepted January 20, 2011.

A

task force criteria

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rrhythmogenic right ventricular cardiomyopa-thy/dysplasia (ARVC/D) is an inherited car-diomyopathy characterized by structural

and functional abnormalities due to aprogressive replacement of predominantly rightventricular myocardium by fibrofatty tissue (1–4).ARVC/D predisposes patients to complex ventric-ular arrhythmias and sudden cardiac death, typicallyamong young subjects.

See page 288

Establishing the clinical diagnosis of ARVC/Dremains challenging because of the lack of a singletest to establish a definite diagnosis. Even endo-myocardial biopsy, sometimes considered to be thegold standard for ARVC/D, is limited because theinterventricular septum as a typical sampling site isless commonly involved (5). In 1994, the TaskForce of the European Society of Cardiology andthe Scientific Council on Cardiomyopathies of theInternational Society and Federation of Cardiologyproposed a set of criteria (6). These criteria werebased on medical history, as well as on morpholog-ical, functional, and structural abnormalities, in-cluding right ventricular dilation, regional dysfunc-tion, (fibro)fatty replacement of the right ventricle(RV), electrocardiographic (ECG) changes, ar-rhythmias, and a family history of sudden cardiacdeath. These original task force criteria (TFC) wereuniversally used to identify patients with ARVC/D.They were considered highly specific, but someauthors have suggested that they may lack sensitiv-ity, especially for early and familial disease (7,8).Moreover, most of these criteria (global and struc-tural abnormalities) were based on qualitative ratherthan quantitative information and were definedbased on the experience before the wide availabilityof cardiac magnetic resonance (CMR).

Over the past 15 years, CMR has emerged as thenoninvasive diagnostic tool of choice for assessingRV anatomy, structure, and function (9,10). High-resolution cine imaging with state-of-the-artsteady-state free-precession techniques is widelyconsidered the gold standard for the assessment ofventricular volumes, myocardial mass, and systolicfunction; and CMR demonstrates high intraob-server and interobserver agreement and accuracy(11,12). The high spatial and temporal resolutionenables a detailed assessment of the RV for regionsof severely reduced wall thickness and wall motion

abnormalities.

The CMR evidence of intramyocardial fat andfibrosis in the RV has been used as supportivediagnostic information; however, because of thelimited specificity of these findings and technicallimitations of CMR in visualizing the thin RVmyocardium, the diagnostic utility of intramyocar-dial fat and fibrosis as diagnostic targets inARVC/D remains controversial (13).

Recently, a revision of the TFC has been pro-posed, incorporating quantitative assessment of RVsize and RV function (14). On the basis of as yetunpublished pilot data from a partially geneticallydefined cohort of patients, the authors proposeimaging criteria using a combination of RV dilationand severe regional wall motion abnormalities toestablish evidence for ARVC/D. The main differ-ences to the previous set of criteria include theremoval of RV microaneurysms (focal akinesis withearly diastolic bulging) (Fig. 1) and segmental RVdilation, and the use of a different and more detailedquantitative definition of RV dilation.

The purpose of our study was to assessthe impact of revised versus original crite-ria on the prevalence of ARVC/D criteriain CMR studies.

M E T H O D S

Study population. We performed a retro-spective analysis of patients referred to ourCMR center for ARVC/D between 2005and 2010. To minimize inappropriate in-dications, we included only patients re-

Figure 1. Microaneurysm in a Patient With Suspected ARVC

There is a focal outpouching of the free RV wall in early diastole (“eing,” arrow). According to the original criteria, this finding itself wacriterion while the revised criteria require a combination of regionaor dyssynchronous contraction with global RV dysfunction or dilatio

A B B

A N D

ARVC

ventri

dyspl

CMR �

reson

ECG �

EF �

LV �

RV �

TFC �

arly diastolic bulg-s considered a majorl akinesis, dyskinesis,n. See Online Video

1. ARVC � arrhythmogenic right ventricular cardiomyopath

R E V I A T I O N S

A C R O N YM S

/D � arrhythmogenic right

cular cardiomyopathy/

asia

cardiac magnetic

ance

electrocardiographic

ejection fraction

left ventricle

right ventricle

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bctoeveca

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cC1fa

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ferred from cardiologists specialized in electrophys-iology. All reports included a quantitativeassessment of the RV and, therefore, allowed thepost-hoc application of both sets of criteria.

We examined the CMR studies for the presenceor absence of major and minor criteria for CMRusing both the original TFC and the revised TFC(Table 1). When analyzing the data using therevised TFC, RV volumetric criteria and RV func-tion were assigned strictly quantitatively, using thequantitative cutoff value from the revised TFC(Table 1).CMR protocol. CMR was performed using a 1.5-Tscanner (Magnetom Avanto, Siemens Medical So-lutions, Erlangen, Germany). The left ventricle(LV) and RV function were assessed using standardECG-gated cine steady-state free-precession se-quences (typical repetition time [TR] � 67, echodelay time [TE] � 1.15, field of view � 340 �276). The RV function was assessed in a contiguousstack of short-axis slices of the RV perpendicular tothe anatomic RV long axis (slice thickness 6-mm,no gap) to cover the entire RV, and in sagittal

Major and Minor Imaging Criteria According to Original andriteria

ria Revised Criteria

nd reducedorrysms orl RV

Regional RV akinesia or Regional dyskinesiaor Dyssynchronous RV contraction andRVEDVI/BSA �110 ml/m2 (male) orRVEDVI/BSA �100ml/m2 (female) orRVEF �40%

on and/orrmal LV) orkinesia orV dilation

Regional RV akinesia or Regional dyskinesiaor Dyssynchronous RV contraction andRVEDVI/BSA �100 to �110 ml/m2

(male) or RVEDVI/BSA �90 to �100 ml/m2 (female) or RVEF �40% to �45%

ight ventricle; RVEDVI/BSA � right ventricular end-diastolic volume indexed toright ventricular ejection fraction.

acteristics

Variables Patients (n � 294)

43 � 16

146 (49.6)

VC/D in a first-degree relative and/orythmia

169 (57.4)

125 (42.6)

57.6 � 6.1

54.6 � 7.6

88.2 � 20.3

c right ventricular cardiomyopathy/dysplasia; LVEF � left ventricular ejection

ens as in Table 1.

orientation (slice thickness 8-mm, no gap). LVfunction was assessed using multiple long-axisviews.Image analysis. All CMR studies were interpretedy at least 2 experienced readers (case review dis-ussions), blinded to the results of other diagnosticests. For viewing and for the quantitative analysisf RV volumes and function, certified CMR imagevaluation software was used (cmr42, Circle Cardio-ascular Imaging, Calgary, Alberta, Canada). Thendocardial and epicardial LV contours and endo-ardial RV contours were drawn for each diastolicnd systolic frame.Major criteria. Table 1 summarizes the differencesor major and minor criteria between original andevised TFC. Important to our sample, localizedV microaneurysm and severe segmental RV dila-

ion, originally classified as a major criterion, are notonsidered a criteria in the revised TFC, but may,owever, qualify as supportive (RV akinesis).

Minor criteria. Mild segmental RV dilation andregional RV hypokinesis, originally classified asminor criteria, are not considered criteria in therevised TFC.Statistical analysis. Continuous parameters are ex-pressed as mean � SD. Criteria were expressed asdichotomous data. Differences between groups wereassessed by the McNemar test. A p value �0.05 wasonsidered significant. Sensitivity and specificity ofMR criteria were calculated for the subgroup of34 patients with complete information regardingamily history, ECG changes, tissue characteristics,nd arrhythmias.

R E S U L T S

During the observed period, 308 patients werereferred. The RV volumes and ejection fraction(EF) were not assessable for 14 patients owing toimage quality, mostly related to complex arrhythmiapatterns. The study population thus consisted of294 patients (see Table 2 for patient characteristics).The most frequent reason for the referral was apositive family history (ARVC/D and/or suddencardiac death) in first-degree relatives and/or doc-umented arrhythmias.

The prevalence of major and minor criteria ac-cording to the original and revised TFC is shown inFigure 2.Major criteria. Applying the original TFC, 69 pa-ients (23.5%) had major criteria versus 19 patients6.5%) with the revised TFC (Fig. 2). The differ-

Table 1. Definition ofRevised Task Force C

Original Crite

Major criteria

Severe RV dilation aRVEF (normal LV)Localized RV aneuSevere segmentadilation

Minor criteria

Mild global RV dilatireduced RVEF (noRegional RV hypoMild segmental R

LV � left ventricle; RV � r

Table 2. Patient Char

Age, years

Female sex, n (%)

Indications, n (%)

Family history of ARdocumented arrh

Rule out ARVC/D

LVEF (%)

RVEF (%)

RVEDVI/BSA, ml/m2

ARVC/D � arrhythmogeni

nce was mainly due to findings of regional wall

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motion abnormalities or microaneurysms in theabsence of RV dilation, qualifying as a criterionaccording to the original but not the revised criteria.

Using the original criteria, localized RV microa-neurysm was the most frequent major abnormality(64 of 69 patients), 2 patients showed severe seg-mental RV dilation, and 3 patients had severe RVdilation and reduced RVEF. With the revisedTFC, the most frequent major abnormality was thecombination of regional akinesis with moderate tosevere RV dilation or RV dysfunction. One patientshowed regional dyskinesis, and none had dyssyn-chronous RV contraction.

Of the 69 patients with major criteria accordingto the original TFC, only 16 patients (23.1%) hadmajor criteria according to the revised TFC (p �0.001). Forty-three patients (62.3%) did not meetany criteria at all (Fig. 3).Minor criteria. With the original TFC, 172 patients58.5%) had minor criteria with the majority ofatients having 1 minor criterion (n � 133), 38atients with 2, and 1 patient with 3 minor criteria.egional hypokinesia was the most frequent minor

bnormality among patients with 1 minor criterionn � 132). Using the revised TFC left only 12atients (4%) with minor criteria (Fig. 2). The mostrequent minor abnormality was the conjunction ofegional akinesis with mild RV dilation or RVysfunction; only 1 patient had regional dyskinesis,nd none had dyssynchronous RV contraction.

Of the 172 patients initially classified as havinginor criteria, only 2 patients (1.1%) still haveinor criteria using the revised TFC (p � 0.001),

nd 167 patients (97%) were reclassified to normalccording to the revised TFC (Fig. 4).Correlation of original and revised criteria for CMR inclinically proven ARVC/D patients. Detailed informa-tion about family history, ECG changes, tissuecharacteristics, and arrhythmias were obtained for134 patients. Ten patients met the definite diagno-sis of proven ARVC without counting imagingcriteria. Applying the original TFC for imaging, 10patients had abnormalities (9 patients with majorcriteria, 1 patient with 1 minor criterion), whereasusing the revised TFC, 4 patients had major crite-ria, yet 6 patients had no criteria at all. If we addmicroaneurysms as a criterion to the modified TFcriteria, the sensitivity increases to 50% (5 of 10patients).Correlation of original and revised criteria for CMR inpatients without ARVC/D. One hundred and twenty-our patients did not meet the criteria for

RVC/D. Applying the old imaging criteria, 4

atients were classified as ARVC only because ofhe imaging findings. Three of them had at least 1inor criterion, and 1 patient had a major criterion.he complete chart of each patient (including

amily history, symptoms, ECG, late potentials,olter monitoring, and follow-up) has been re-

iewed by a cardiologist specialized in electrophys-ology. These 4 patients have been consideredithout ARVC, and they have been included in theroup of 124 patients without ARVC/D. Using theevised TFC, the majority of patients had noriteria (n � 112; 90.5%), 7 patients had major

Figure 2. Proportional Incidence of Original and Revised ARVC

The graph shows the different incidence of patients with major critminor criteria, or without criteria according to the original cardiac mresonance criteria (green bars) and the revised criteria (orange barAbbreviation as in Figure 1.

Figure 3. Reclassification of Patients Initially Classified asHaving Major Criteria

Of the total of 69 patients with a major criterion according to the oclassification, 16 (23.2%) also had a major criterion according to therecommendations, 10 patients (14.5%) were reclassified as having a

Criteria

eria,agnetics).

riginalrevisedminor

criterion, and 43 patients (62.3%) did not have any criteria at all.

iwimt

normal acc

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criteria (5.5%), and 5 patients had minor criteria(4%). Specificity of major and minor CMR criteriafor ARVC/D in our sample was higher for therevised criteria (94% and 96%, respectively) than forthe original criteria (78% and 39%, respectively).Follow-up. With a mean follow-up of 2.2 � 1.2years, 293 patients were still alive; 1 patient died ofcancer. Of 134 patients with complete diagnosticwork-up of ARVC, with a mean follow-up of2.25 � 1.25 years, 7 patients underwent insertion ofmplantable cardioverter-defibrillator, all of themith proven ARVC. Using the original TFC for

maging, 5 patients had major criteria and 2 had ainor criterion; applying the revised TFC, 4 pa-

ients had major criteria and 3 had no criteria.

D I S C U S S I O N

To our knowledge, this is the first study to comparethe original and the revised task force imagingcriteria for ARVC/D. In our sample, we observed asignificant decrease in the prevalence of minor andmajor CMR criteria for ARVC/D with the revisedTFC, with a potential increase of specificity anddecrease of sensitivity.

In a large cohort of 294 patients referred forCMR for suspected ARVC/D, only 31 patients(10.5%) had criteria according to the revised TFC,compared with 241 patients (82%) according to theoriginal TFC. Only 37.7% of our patients initiallyclassified as having major criteria and 2.7% ofpatients initially classified as having at least 1 minorcriterion still met imaging criteria for ARVC/D.

The revision of the classification for major and

Reclassification of Patients Initially Classified asinor Criteria

ients initially classified as having at least 1 minor criteria, 3.7%) were reclassified as having a major criterion, 2 (1.1%) contin-e a minor criterion, and 167 patients (97%) were reclassified toording to the revised task force criteria.

minor structural abnormalities represents a major

change. Previously, major and minor structuralabnormalities were established with the presence ofRV dilation (visual assessment of global or segmen-tal dilation), localized microaneurysm, and regionalhypokinesis. Dilation of the RV was initially de-fined by echocardiography or angiography with noquantitative limits to differentiate mild from mod-erate to severe RV dilation. The newly suggestedmajor and minor criteria are based on data from 108subjects with newly diagnosed ARVC/D (14). TheCMR data from 44 volunteers were compared withresults obtained from 462 normal subjects. Thecombination of regional wall motion abnormalities(akinesis, dyskinesis, or dyssynchrony) with RVdilation or RV dysfunction (with quantitative as-sessment) had a sensitivity of 89% for major criteriaand 78% for minor criteria. In our study, to excludethe bias due to using different cutoff values to assessRV dilation and RV dysfunction, we used the samequantitative cutoff value for both original and re-vised criteria. However, only 31 patients (10.5%)had a positive criterion as defined by RV dilation(19 with moderate to severe RV dilation and 12with mild RV dilation) in combination with re-gional or global RV dysfunction. Of 64 patientswith localized microaneurysms according to theoriginal TFC, only 24 still had a criterion in thecombination with RV dilation or RV dysfunction.Recently, Cox et al. (15) analyzed 105 patients withproven ARVC/D (using the 1994 TFC), includinga subgroup of 64 patients with CMR data. Fifteenof their patients (24%) revealed major or minorcriteria using the new imaging TFC. In a lessstrictly defined population (89 family members), 26subjects had CMR with only 1 patient (3.8%)showing a structural abnormality using the revisedimaging TFC.

Although our sample size of verified ARVC issmall, our data indicate that the new criteria mayhave a limited sensitivity, with only 4 of 10 patientswith a definite diagnosis of ARVC/D having majorcriteria. The reason for that may be that therequired combination of regional wall motion cri-teria with global RV dilation/dysfunction may misspatients with purely or mainly regional RV abnor-malities. Therefore, the revision of the criteria mayhave missed the goal of increasing the sensitivity ofsuch criteria, yet have led to an increase of speci-ficity. If confirmed for a larger population of pa-tients with verified ARVC, this observation mayhave important clinical implications.

A significant limitation of our study is the lack of

Figure 4.Having M

Of 172 patpatients (1ued to hav

a true “gold standard” for the definite diagnosis of

FdS7C

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ARVC/D and limited follow-up data. However,the chart of each patient has been reviewed, and thediagnosis of ARVC/D has been confirmed bycardiologists specialized in electrophysiology re-garding the family history, symptoms, ECG abnor-malities, arrhythmias, tissue characteristics, andfollow-up. Prospective studies will have to be per-formed using the revised TFC and hard end pointssuch as appropriate implantable cardioverter-defibrillator shocks or cardiovascular death.

Our single-site experience may not reflect that ofothers. The high incidence of microaneurysms asmajor criteria according to the original criteria mayreflect a local tendency to “overcall” this and,therefore, overestimate the negative impact of therevision on the sensitivity. We did not utilize lategadolinium enhancement imaging to assess themyocardium for areas of fibrous or fibrofatty degen-eration. Although this was not part of the originalor revised criteria and is challenging in the thin wallof the RV, the detection of such areas in the LVmay prove useful.

Finally, we have investigated the value of imaging

Society and Federation of Cardiology.Br Heart J 1994;71:215–8.

1

1

1

ing in arrhythmogelar dysplasia: insigh

the diagnostic accuracy would also have to beassessed in the context of combined criteria.

C O N C L U S I O N S

In CMR studies performed for assessing patientsfor ARVC/D, the introduction of the revised TFCfor ARVC/D may lead to a decrease of the preva-lence of major and minor imaging criteria. Therevision may, therefore, not have improved theirsensitivity but, instead, have improved their speci-ficity for identifying patients with ARVC/D. Pro-spective studies should be performed to study theprognostic value of the new imaging criteria.

Reprint requests and correspondence: Dr. Matthias G.riedrich, Stephenson CMR Centre at the Libin Car-iovascular Institute of Alberta, Departments of Cardiacciences and Radiology, University of Calgary, SSB Suite00, Foothills Medical Centre, 1403-29 Street NW,algary, Alberta T2N 2T9, Canada. E-mail: matthias.

criteria using them as independent markers. Thus, friedrich@ucalgary.ca.

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2. Pinamonti B, Sinagra G, Salvi A, etal. Left ventricular involvement inright ventricular dysplasia. Am Heart J1992;123:711–24.

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6. McKenna WJ, Thiene G, Nava A, etal. Diagnosis of arrhythmogenic rightventricular dysplasia/cardiomyopathy.Task Force of the Working GroupMyocardial and Pericardial Disease ofthe European Society of Cardiologyand of the Scientific Council on Car-diomyopathies of the International

7. Hamid MS, Norman M, Quraishi A,et al. Prospective evaluation of rela-tives for familial arrhythmogenic rightventricular cardiomyopathy/dysplasiareveals a need to broaden diagnosticcriteria. J Am Coll Cardiol 2002;40:1445–50.

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0. Tandri H, Friedrich MG, Calkins H,Bluemke DA. MRI of arrhythmo-genic right ventricular cardiomyopa-thy/dysplasia. J Cardiovasc MagnReson 2004;6:557–63.

1. Tandri H, Castillo E, Ferrari VA, etal. Magnetic resonance imaging ofarrhythmogenic right ventricular dys-plasia: sensitivity, specificity, and ob-server variability of fat detection versusfunctional analysis of the right ventri-cle. J Am Coll Cardiol 2006;48:2277–84.

2. Tandri H, Macedo R, Calkins H, etal. Role of magnetic resonance imag-

nic right ventricu-ts from the North

American Arrhythmogenic RightVentricular Dysplasia (ARVD/C)study. Am Heart J 2008;155:147–53.

13. Tandri H, Calkins H, Marcus FI.Controversial role of magnetic reso-nance imaging in the diagnosis ofarrhythmogenic right ventricular dys-plasia. Am J Cardiol 2003;92:649.

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Key Words: arrhythmogenicright ventricularcardiomyopathy/dysplasia ycardiac magnetic resonance yTask Force Criteria.

A P P E N D I X

For a supplementary video, please see the

online version of this article.