Arrhythmic mitral valve prolapse

Mitral annular disjunction identified peripartum: A case highlighting key features of a recently classified syndrome

“…The Padua hypothesis theorises that the increased pulling of the posteromedial papillary muscle in mid‐systole by the mitral leaflets draws the adjoining postero‐basal left ventricular wall harshly to the apex 12 . This exaggerated force eventually leads to tissue damage and the formation of scar tissue, 12 which is evident with MAD in post mortem studies, 1,13 via other imaging modalities 2,3,6 and also histologically 1,3 . The interface between scar tissue and unaffected tissue, along with the repetitive mechanical stretch, is the hypothesised origin of the ventricular arrhythmias in MAD 13 …”

“…Pickelhaube's sign is a high velocity tissue Doppler spike of >16 cm/s above the baseline in mid‐systole at the lateral mitral annulus 5,6 . (an example is shown in Figure 4).…”

“…(an example is shown in Figure 4). Pickelhaube's sign quantifies the obscure hypermobility of the mitral annulus that is a typical feature of MAD, which may predispose patients to ventricular arrhythmias 6 . Quantifying the paradoxical systolic diameter increase is another TTE measurement for MAD (shown in Figure 5).…”

“…MAD causes the PM annulus to move with the left atrium during the cardiac cycle instead of the standard movement with the left ventricle 8,13 . This produces a greater annular diameter in end‐systole, in contrast to normal where the end‐diastolic diameter is greater 6,13 …”

Electrophysiological Substrate in Patients with Barlow’s Disease

“…The presence of mitral annular disjunction (MAD), defined as the detachment of the mitral annulus compared to the ventricular myocardium during cardiac systole, and the presence of the Pickelhaube sign, defined as an high-velocity positive systolic wave at tissue Doppler evaluation of lateral mitral annulus, also characterised a cohort of patients with a high risk of malignant VAs. [ 32 – 34 ] The clinical role of MAD is not yet fully understood. In particular, Dejgaard et al showed that MAD was associated with VAs, even in absence of detectable MVP, thus suggesting that MAD itself is an arrhythmogenic entity.…”