Advances in Cardiovascular Multimodality Imaging in Patients with Marfan Syndrome

Figures

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  Figure 1. (A) A long parasternal view in 2D transthoracic echocardiography of a young adult reveals severe dilatation of the aortic root, displaying the characteristic “onion shape”. (B) A focused long parasternal view in 2D transthoracic echocardiography of the same patient demonstrates an aneurysm of the ascending aorta.

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  Figure 2. (A,B) 2D transthoracic echocardiography (TTE) images of a young adult with Marfan syndrome. Image (A) displays mitral valve prolapse in the long parasternal axis view, while image (B) shows the mitral valve prolapse in the four-chamber view.

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  Figure 3. (A,B) show mitral annular disjunction (MAD) in 2D transthoracic echocardiography (TTE) of a young patient with Marfan syndrome, respectively, captured in the long parasternal view and the four-chamber view. The MAD measures 0.6 cm and 0.7 cm, respectively.

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  Figure 4. A 16-year-old girl with Marfan syndrome assessed with CMR. Box (A) and Box (B) show aortic assessments using two different sequences: Box A shows the 3D balanced steady-state free precession (bSSFP) sequence, while Box B shows the contrast-enhanced MR angiography (CEMRA) sequence. Both techniques provide comparable measurements of aortic diameters at the level of the mean pulmonary artery. Box (C) shows magnification of 3D volume rendering of aorta assessed with CEMRA. The patient was characterized by normal biventricular size, wall thickness, and systolic function (assessed by bSSFP sequences, Box (D) and by normal tissue characterization at T1 mapping Box (E), T2 mapping Box (F), LGE Box (G) and ECV Box (H) sequences).

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  Figure 5. A 34-year-old woman with Marfan syndrome who underwent emergency CT examination on suspicion of aortic dissection. The asterisk and arrow panels (B,C) show the breach and the false lumen, respectively. Panel (A) shows the 3D reconstruction in volume rendering.

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  Figure 6. A case of aortic prosthetic graft infection in a patient with Marfan syndrome evaluated with 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT): coronal (A) and axial (B) images show hypermetabolism around the aortic prosthetic graft (orange arrows) and site of infection process.