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Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging

Harikrishna Tandri, MD^*, Manoj Saranathan, PhD, E. Rene Rodriguez, MD^*, Claudia Martinez, MD^*, Chandra Bomma, MD^*, Khurram Nasir, MBBS^*, Boas Rosen, MD^*, João A.C. Lima, MD^*, Hugh Calkins, MD^* and David A. Bluemke, MD, PhD^*,

^* Division of Cardiology
Department of Radiology, The Johns Hopkins University, Baltimore, Maryland

View larger version (47K): [in a new window]   Figure 1 (A) This panel shows an axial delayed enhanced image from an arrhythmogenic right ventricular dysplasia patient. The signal intensity of the right ventricular (RV) myocardium is increased, similar to that of epicardial fat. (B) This panel shows the same level of the myocardium as panel A, using the same pulse sequence with chemical shift fat suppression. The enhanced RV anterior wall is well-appreciated (arrows), and fat in the anterior chest wall has low signal intensity (arrowhead). (C) This panel shows the same pulse sequence as panel B except that body coil was used for signal transmission and reception. Although the signal-to-noise ratio is decreased compared with surface coil imaging in panels A and B, the signal intensity is more uniform across the field of view. This confirms that the RV signal is increased (arrows) because of increased myocardial signal rather than proximity to the phased array surface coil.

View larger version (129K): [in a new window]   Figure 2 Four-chamber delayed enhanced image from a patient with arrhythmogenic right ventricular dysplasia who had extensive lymphocytic myocarditis on biopsy. The right ventricular (RV) wall and the RV portion of the septum are diffusely enhanced. Further, there is a small area of enhancement in the lateral wall of the left ventricle (LV), possibly indicating LV involvement.

View larger version (130K): [in a new window]   Figure 3 The top left and right panels represent the end-diastolic and end-systolic frames of a short-axis cine magnetic resonance image (MRI) showing an area of dyskinesia on right ventricular free wall characterizing a focal ventricular aneurysm (arrows). The bottom left panel displays the delayed-enhanced MRI with increased signal intensity within the right ventricular myocardium (arrows), at the location of RV aneurysms. The bottom right panel shows the corresponding endomyocardial biopsy. Trichrome stain of the right ventricle at high magnification shows marked replacement of the ventricular muscle by adipose tissue. The adipose tissue cells (arrowhead) are irregular in size and infiltrate the ventricular muscle. There is also abundant replacement fibrosis (arrow). There is no evidence of inflammation.

View larger version (17K): [in a new window]   Figure 4 (A) Correlation between the extent of delayed enhancement and right ventricular ejection fraction. (B) Correlation between the extent of delayed enhancement and right ventricular end diastolic volume.