A tale of two MADs: a case series

Mitral annular disjunction (MAD), consisting in a systolic separation between the posterior atrial wall-leaflet junction and the basal left ventricular wall, is a disputed imaging entity. MAD was initially associated with sudden cardiac death and ventricular arrhythmias in patients with mitral valve prolapse, whereas in more recent studies, it has been presented as a normal variant of the mitral annulus.

In the present series of two cases, we show a case featuring a young woman with syncope that showed a microscopic MAD on echocardiography but, after a thorough multimodality assessment, was diagnosed with a coronary anomaly responsible for her presentation. In the second case, a young man presenting with aborted sudden cardiac death was found to have a macroscopic MAD in the context of mitral valve prolapse with numerous high-risk arrhythmic features.

The need for assessing the diverse significance of MAD in the clinical and imaging context of each patient is underscored. Assessment of MAD should be complemented by other imaging and clinical parameters: the circumferential and longitudinal extent of MAD, the presence of repolarization abnormalities or ventricular arrhythmias, bi-leaflet prolapse, systolic curling, the Pickelhaube sign, left heart remodelling, and the presence of myocardial fibrosis, among others.