Marfan Syndrome beyond Aortic Root—Phenotyping Using Cardiovascular Magnetic Resonance Imaging and Clinical Implications

Figure 1. (Panel A): CMR balanced steady-state free precession (b-SSFP) measurement of the aortic root. Both sinus-to-sinus (left-sided image) and sinus-to-commissure (right-sided image) measurements are shown. (Panel B): CMR gadolinium-enhanced (GE) angiogram of the entire thoracic aorta.

Figure 2. CMR balanced steady-state free precession (b-SSFP) cines in systole showing mitral annular disjunction (18 mm between arrows): (a). Left ventricular outflow tract (LVOT) view, (b). heart-long-axis (HLA) view, (c). ventricle long-axis (VLA) view.

Figure 3. CMR b-SSFP still images in systole showing severe mitral valve regurgitation into a severely dilated left ventricle: (a). LVOT view, (b). HLA view, (c). VLA view.

Figure 4. Late gadolinium images at LVOT view (left-sided image) and basal short-axis view (right-sided image) showing focal sub-epicardial/mid-wall enhancement (small arrows).

Figure 5. ADAS-LV scar segmentation analysis showing VT corridors: panels a and b showing a potential VT corridor at the layer 60% distance from the endocardial surface. Scarring is coded as red areas, healthy tissue as blue areas, and border zone as yellowish area. Panels c and d showing a VT channel at the base of posteromedial papillary muscle.

Figure 6. b-SSFP still images at end-diastole (left-sided images) and end-systole (right-sided images) showing dilated LV with severely reduced ejection fraction in the absence of volume load from valvular dysfunction. (Panel A): HLA view at end-diastole and end-systole with arrows showing MAD. (Panel B): Short-axis basal slice at end-diastole and end-systole.

Figure 7. Late gadolinium images showing linear mid-wall LGE at the septum (and basal inferior/inferolateral walls (panels a,b).