Figure 11:

(A) Mitral annular disjunction (MAD) is characterized by detachment of the roots of the mitral annulus from the ventricular myocardium with atrial displacement of the posterior mitral valve leaflet. In this example, the posterior mitral valve leaflet is displaced from the ventricular myocardium and is displaced into the left atrium, as seen on 2D TTE apical four-chamber view. The MAD distance, or width, is displayed by the white dotted arrow. (B) Tissue Doppler echocardiography showing peak systolic lateral mitral annular velocity > 16 cm/s, known as the “Pickelhaube sign” (white arrow), a novel echocardiographic marker that has recently been associated with a more malignant phenotype of MVP (34). (C) Cardiac MRI three-chamber view demonstrating gap (black dotted arrow) between the posterior mitral valve leaflet insertion point and the ventricular myocardium as well as bileaflet MV prolapse.