David A. Bluemke, M.D., Ph.D.

1. David A. Bluemke, M.D., Ph.D. How to Perform MRI for Arrhythmogenic Right Ventricular Dysplasia/ Cardiomyopathy (ARVD/C) Associate Professor, Clinical Director, MRI Departments of Radiology and Medicine Johns Hopkins University School of Medicine Baltimore, Maryland

2. Disclosures • Off-label: gadolinium MRI of the heart • Sponsorship: JHU ARVD Center, NHLBI N01-CM-27018, Donald W. Reynolds Foundation Acknowledgements • João Lima, MD, Hugh Calkins, MD, Henry Halperin, MD, Saman Nazarian, MD • Frank Marcus, MD • Harikrishna Tandri, MD, Chandra Bomma, MD, Ernesto Castillo, MD • Crystal Tichnell, JHH ARVD center

3. ARVD/C – Protocol Summary • Axial & short axis “T1” images, with blood suppression (double IR FSE/ TSE) - 5 mm slice thickness, ETL 24-28 - to avoid wrap-around, use anterior coils only - 10-12 slices axial, 5 slices short axis over the heart. • Same as (1), but axial only, with fat suppression • SSFP Cine: axial and short axis, long axis cine - 10-12 short axis cine images, 8 axial images, 4 chamber cine • Delayed gadolinium images - 5 short axis images, 6-8 axial images Note: since the protocol is long, the minimum # of slices in each plane is given above.

4. Black blood double IR TSE/ FSE images • Either 1 RR or 2 RR is fine, blood suppression pulse for dark blood • TE 20-30 ms, ETL 24-32, 256x256, ZIP to 512 • 5x3 mm, 1 NEX, breath-holding • Anterior coil only to avoid wrap, FOV 24-28 short axis – shows LV and the inferior RV wall Axial – shows free wall of the RV

5. Repeat the axial images with fat sat • Axial “T1” images, blood/ fat suppression • TE min, ETL 24-32, 256x256, ZIP • 5x3 mm (same slice locations as non fatted images) • Anterior coil, FOV 24-28 Fat suppression reduces artifacts especially for the RV free wall The axial plane for fat sat is sufficient.

6. Common protocol questions: • What about prone imaging? • not necessary with breath-hold imaging. • difficult for patients to sustain for the duration of this protocol (45 + minutes). • 2. Why is there some much “axial” imaging? • Axial imaging provides an excellent view of the anterior RV wall and RVOT. It is easy for the technologist. • HLA (long axis) images do not image the RVOT

7. Common protocol questions: 3. We have a double IR single shot sequence (ssfse, HASTE) that is much faster – should I use this? NO! As seen below, these images blur RV detail and are not used for ARVD/C “HASTE”

8. Axial/ Short Axis Cine SSFP Images • Axial: 6 mm, skip 2 mm, FOV 36 cm, same slice locations as the black blood images for axials. 8-10 images from the diaphragm to the aortic root. • Obtain a 10-12 short axis cines to quantitate LV and RV function (short axis not shown). 37% of normal volunteers have a normal “anterior” bulge. The remainder have a “round” shaped RV. 17% of normal volunteers, triangular shape RV

9. Last Step: IR prepped delayed Gad • Same pulse sequence as for infarct (viability) imaging • 8-10 axial images, 5 short axis images (same locations as black blood images) • We perform short axis first; then reduce the TI (inversion time) by 25 msec for axial images. 30-80% of (advanced) cases have LV, as well as RV enhancement

10. ARVD/C MRI Reports • MRI criteria: a) enlargement of the RV, b) regional RV wall motion abnormalities or aneurysms. Double reading of all cases is recommended. • Presence of fat and fibrosis (delayed gad) can help, but are not official diagnostic criteria. • Major criterion: Severe abnormalities: can be seen by the first year resident. • Minor criterion: Mild-moderate abnormalities: you are not sure, probably present and you want to document these. • MRI Impression, choose one of the following: • 1. Normal MRI • 2. Nonspecific findings (minor criterion) • 3. MRI consistent with ARVD/C (major criterion) 2nd Opinions can be obtained at www.ARVD.com