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Arrhythmogenic right ventricular cardiomyopathy (ARVC)

**new task force criteria here

a) SCMR official document standardized acquisition guidelines – relevant extract

1. LV structure and function module – consider 5–6 mm slice thickness
   a. Careful RV as well as LV volumetric analysis.
2. Transaxial or oblique transaxial SSFP cine images covering the RV including RVOT. An RV vertical long axis view aligned with tricuspid inflow is recommended.
3. a. Careful RV as well as LV volumetric analysis.
4. Optional sequences
   a. Selected transaxial or oblique transaxial black blood images (double inversion recovery T1-weighted fast spin echo).
   b. Repeat same geometry with fat suppression
   c. Late gadolinium enhancement module in same orientations as above. Consider T1 nulling for RV.
   d. Consider use of anterior surface coil only to improve resolution without "wrap around" artifacts.
   e. Consider prone position in overweight patients in order to minimize distance between the surface coil and RV. 
   
   Major criteria for diagnosing ARVC include severe dilation and reduction of right ventricular ejection fraction with no or mild left ventricular involvement; localized right ventricular aneurysms (akinetic or dyskinetic areas with diastolic bulgings); severe segmental dilation of right ventricle.
   
   Minor criteria include mild global right ventricular dilation or ejection fraction reduction with normal left ventricle; mild segmental dilation of right ventricle; regional right ventricular hypokinesia. All of these are demonstrable by CMR as performed above.
   CMR also allows assessment of fatty infiltration and myocardial fibrosis. However, the latter two findings are not part of current guidelines. Note – there is variability of the structure and shape of the RV amongst normals, so there is a tendency for inexperienced observers to overdiagnose RV wall motion abnormalities. For example, relative end-systolic bulging of a thin but contractile region of the RV free wall adjacent to the moderator band can be a normal finding and basal short axis cines may give the impression of inferior wall dyskinesis due to normal through-plane motion. Careful study of RV cines in volunteers and a range of patients is recommended before attempting identification of ARVC.

b) SCMR official document reporting recommendations – relevant extract

It is recommended that each report identify major and minor criteria associated with ARVC.  This should include a statement regarding: 
a) Global right ventricular performance (RVEF); 
b) RV dilation; 
c) Location of Regional RV wall motion abnormalities (infundibulum, body or apex of right ventricle). 
When acquired: 
a) Fatty infiltration of the right ventricle, and 
b) Occurrence of fibrosis by LGE should be provided. 

c) Standardized web based images

No case yet

d) Case of the Week example(s)

Number 10-21: Role of CMR in the diagnosis of ARVC
History: A 78 year old patient was admitted to the Accident and Emergency department with a syncope. The ECG showed a broad complex tachycardia (left bundle-branch morphology with superior axis) with a rate of 230 bpm. The patient received DC cardioversion under sedation, which resulted in sinus rhythm.

Number 10-20: Electrophysiologic and CMR features of ARVC
History: A 19-year old caucasian female with documented ventricular tachycardia was referred to electrophysiology service.

Number 07-14: ARVC with LV involvement
History: A 59 year old with an 8 year history of asymptomatic ARVC diagnosed through family screening. A disease causing mutation in plakophilin was found. Type 1 diabetes since childhood. Recent echo showed a possible new LV apical aneurysm. Coronary angiography normal.

Number 07-15: Sarcoidosis Masquerading as ARVD/C
History: A 46 year old African American presenting with VT of LBBB morphology.

Number 07-17: ARVC mimic - congenital partial absence of the pericardium
History: Family screening implemented for ARVC after a 1st degree relative died of cardiomyopathy with ventricular dilatation. In this individual, and abnormal ECG with right pre-cordial T wave inversion and a shift of the transition zone to V5 was found suggesting possible ARVC. Echo normal but with poor windows.

Number 08-19 Diagnosing RV dilatation by CMR
History: ** Case of the year winner, 2008

e) Expert opinion – ‘How we do’

"How I Do" CMR of ARVC/D (David A. Bluemke, Johns Hopkins)

f) Relevant Online Talks

Free talks

Diagnostic challenges: CMR for ARVC
By James Moon - The Heart Hospital, London
Recorded at HCM working group of the Spanish cardiology society 2008

CMR in ARVC
By James Moon - The Heart Hospital, London
Recorded at The 2008 UK Cardiomyopathy Association meeting - ARVC:

ARVC and sudden death
By Matthias Friedrich
Recorded at EuroCMR 2008

Members only talks - general

Update on CMR of Suspected ARVC/D
By Harikrishna Tandri - Johns Hopkins University
Recorded at SCMR 2010

Cardiomyopathies and Right Ventricular Dysplasia
By Ian Paterson - University of Alberta
Recorded at SCMR 2008: Technicians Workshop

g) Relevant papers (starting point):

Sen-Chowdhry S et al. Circulation 2007;115:1710-20;
Sen-chowdhry S et al.JACC 2006;48:2132-40
Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia
Aditya Jain, Harikrishna Tandri, Hugh Calkins, David A Bluemke
Journal of Cardiovascular Magnetic Resonance 2008, 10:32 (20 June 2008)
[Abstract] [Full Text] [PDF]