Arrhythmogenic RV Cardiomyopathy
Multimodality Imaging
Natesa G. Pandian
Tufts – New England Medical Center
Boston, Massachusetts
Disclosure: No conflicts
19 year old male college
student
2 month history of
palpitations with exertion
Holter monitoring advised
You can’t afford to
miss
42 year old lawyer
Used to be a college athlete
Continues to play tennis
Father died at 46
Had a syncopal episode
Diagnosed to have ARVC on CMR
Holter showed some PVCs
Started on betabeta-blockers
Came to our center for a second opinion
CMR in our center: Normal
Detailed history:
More like vasovagal syncope
Father died after 2 days of CP
Holter: Normal except for isolated PVCs
Exercise Echo: Normal; 15 min exercise
Lipids: Mild hyperlipidemia
Advice: No ARVC
Change diet, Statin
Resume tennis and activity
You can’t afford to
miss
You can’t afford to
mislabel
What is
ARVD ?
ARVD/C
• Patients are typically < 40 yrs
• In many, the disease is familial
• PrePre-/syncope, palpitations or sustained
or nonnon-sustained VT
• Early, asymptomatic disease is most
common cause of sudden death at
presentation
ARVD may account for as
many as 5% of unexpected
sudden deaths under the age
of 65 and 3-4% of sudden
death during sports.
sports.
Management
• Medical
– May help in some
• Surgical
– Right ventriculotomy or isolation surgery
– Ablation
• ICD implantation
• Genetic counseling
• Cardiac Transplantation
– Patients with RV or biventricular failure
Management
• Medical
– May help in some
• Surgical
– Right ventriculotomy or isolation surgery
– Ablation
• ICD implantation
• Genetic counseling
• Cardiac Transplantation
– Patients with RV or biventricular failure
How to
detect or exclude
ARVD ?
Criteria for the Diagnosis of ARVD
2 major criteria, 1 major and 2 minor criteria, or four minor criteria
Global and/or regional dysfunction and
structural alterations
Major
Severe dilatation and reduction of RV with
no (or only mild) LV impairment
Localized right ventricular aneurysms
Severe segmental dilatation of the RV
Minor
Mild global RV dilatation and/or low
RV EF with a normal LV
Mild segmental dilatation of the RV
Regional right ventricular hypokinesis
ECG depolarization/conduction abn
Major
Epsilon waves/localized prolongation
(QRS>110 ms) in rt precordial leads (V1-V3)
Minor
Late potentials on signal-averaged ECG
Tissue characterization of the walls
Major
Fibrofatty replacement of myocardium on
endomyocardial biopsy
Family History
Major
Familial disease confirmed at autopsy or
surgery
Minor
Family history of premature sudden death
(> 35 yrs) caused by suspected ARVD/C
Family history (clinical )
ECG repolarization abnormalities
Minor
Inverted T in the right precordial (V2-V3)
in
pts >12 years and without RBBB
Arrhythmias
Minor
Sustained or nonsustained LBBB type VT on
ECG, Holter, or during exercise stress testing
Frequent PVCs (> 1,000 per 24 hrs on Holter)
Diagnostic Tools
Suspicion, then
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ECG
Holter
SA ECG
ECHO
MRI
Angiography
Biopsy
North American ARVD Registry: 22 Enrolling Centers
Original EC:
11 US and
1 Canadian
Additional EC
7 US and
3 Canadian
“Triangle of RV Dysplasia“
Dysplasia“
Subtricuspid, apical and RVO free walls
RV Angiography
Invasive
Several criteria
Cardiomegaly
• Localized akinetic/dyskinetic areas
• Bulges/outpouchings
• Dilatation of the infundibulum
• Trabecular hypertrophy
• Disarray with deep fissures
• Elevated RVEDP
•
RV Angiography in ARVD
Cardiac CT
Mostly case reports
No solid systematic studies
CMR
for ARVC
However,
In pts with minimal abnormalities, the
sensitivity/specificity of MRI not defined
Variable degrees of epicardial fat into the
medial layer of the RV in normals
RV free wall is < 44-5 mm thick and the
resolution of the MRI to detect thinning of
several millimeters is questionable
Limited experience in Dx of ARVD by MRI
Misdiagnosis of ARVD/C
Bomma et al J CV EP 2004; 15:300
89 with the dx of “ARVD”
ARVD” who requested a rere-evaluation
ReRe-evaluation:
History, physical exam, and noninvasive testing . Invasive
testing, which included EP testing, RV angiography; biopsy
performed when indicated.
Sixty of the 65 pts (92%) who had undergone CMR at an
outside institution reported to have an abnormal MRI c/w
ARVD. Among these, in 46, the only abnormality identified
was the finding of intramyocardial fat/wall thinning in 46.
Misdiagnosis of ARVD/C
Bomma et al J CV EP 2004; 15:300
89 with the dx of “ARVD”
ARVD” who requested a rere-evaluation
ReRe-evaluation:
History, physical exam, and noninvasive testing . Invasive
testing, which included EP testing, RV angiography; biopsy
performed when indicated.
Sixty of the 65 pts (92%) who had undergone CMR at an
outside institution reported to have an abnormal MRI c/w
ARVD. Among these, in 46, the only abnormality identified
was the finding of intramyocardial fat/wall thinning in 46.
On rere-evaluation, these findings were not confirmed.
None of the 46 pts ultimately were diagnosed with ARVD.
Entire group: only 24/89 (27%) met the Task Force criteria.
Role of magnetic resonance imaging in ARVD:
The North American ARVD Study
Tandri et al, Multidisciplenary Study of ARVD Investigators. Am Heart J. 2008; 155:289
40 pts who met the Task Force criteria exclusive of CMR
Results
RV fat infiltration:
LV fat infiltration:
RV regional dysf:
Qual RV dysfn:
Quant Abn RVEF:
RVEF <50%:
Sensitivity
Specificity
24 (60%)
6 (15%)
32 (80%)
26 (60%);
24/28 (85%).
73%
95%
What are the echo features ?
How to look for them ?
Echo for ARVC
The echo diagnosis of ARVD is possible only
in the absence of other causes of dilatation
of the right ventricle such as:
• congenital heart disease
• right ventricular infarction
• volume overload due to TR
• pulmonary embolism
• pulmonary hypertension
Desired Views
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Parasternal long axis
Parasternal short axis
RV Inflow
RV Outflow
Apical 4 chamber
Apical 5 chamber
Apical 2 chamber
Apical 2 chamber of RV
Subcostal long axis
Subcostal short axis
Parasternal Long Axis
RV Inflow View
• Structures of interest include:
The inferoposterior wall of the RVIT under
the tricuspid valve is the most important
structure in this view
• Often affected in ARVD with WMA,
thinning or aneurysms
• Optimize depth/zoom to ensure
adequate visualization
RV Inflow View
RV Outflow view
Echo: RVOT Dilation
Parasternal LAX
Parasternal SAX
Parasternal Short Axis View
Apical 2 Chamber LV, RV
Subcostal view
Echocardiography
¾
RV Global and Dysfunction
¾
RV basal PW, RVOF reg abn
¾
Excessive/Abnormal Trabeculations
¾
Hyperreflective Moderator Band
¾
Sacculations, Aneurysm
ARVC abnormalities are a spectrum
Multidisciplanary approach essential
Diagnosis for ARVC
Classic cases can be easily
identified by echo and CMR
In subtle cases, take all the help
you need, including Echo, CMR,
EPS and Biopsy
Thank you