Young female with history of palpitation and unexplained syncope and ECG changes of T inversion in inferior and lateral leads has been associated with malignant ventricular arrythmias and sudden cardiac death [SCD] termed as A-MVP. Curling, mitral annulus disjunction (MAD) and myocardial fibrosis may account for arrhythmogenesis. Option D  LV fibrosis is diagnosed by late gadolinium enhancement in cardiac magnetic resonance imaging (MRI) and not by TEE.

Features of A-MVP in TEE are

1. MAD has been increasingly recognized as a major annular abnormality in MVP. It may contribute to myxomatous degeneration of the mitral leaflets and is linked with life-threatening ventricular arrhythmias and SCD. A strong correlation was shown between MAD and systolic curling motion, characterized by hyperdynamic annular motion with abnormal papillary muscle traction and contraction pattern in the adjacent myocardium. Dejgaard, et al have provided support for the possible role of MAD in arrhythmogenesis, demonstrating a high incidence of life-threatening ventricular arrhythmias in patients with MAD. 

2. Bileaflet MVP, frequently combined with myxomatous change in the leaflets and leaflet elongation and thickness, has been suggested as a key feature indicating malignant MVP

3. Another form of echocardiographic evidence for abnormal prolapse-induced LV mechanics is provided by mitral annular velocities quantified by pulse-wave tissue Doppler echocardiography, reported as a potential risk marker for malignant ventricular arrhythmias. In 21 patients with myxomatous bileaflet MVP, a spiked systolic high-velocity signal of the lateral mitral annulus ?16cm/s was named the “Pickelhaube sign” by Tajik and colleagues after a helmet spike. Patients with this sign had more malignant ventricular arrhythmias and myocardial fibrosis detected by cardiac MRI -late gadolinium enhancement in 93% of 30 MVP cases with complex ventricular arrhythmias: 83% in the papillary muscles and 73% in the infero-basal wall.

References

1.Nagata Y, Bertrand PB, Levine RA. Malignant mitral valve prolapse: risk and prevention of sudden cardiac death. Curr Treat Options Cardiovasc Med 2022;24:61-86. 

2.Ghelani R, Chow JJ, Miyazawa A, et al. The arrhythmic mitral valve prolapse syndrome: A new insight for understanding the arrhythmogenic substrate? JACC Case Rep 2025;30:103419.

3.Perazzolo Marra M, Cecere A, Cipriani A, et al. Determinants of ventricular arrhythmias in mitral valve prolapse. JACC Clin Electrophysiol 2024;10:670-81.

4.Wejner-Mik P, Michelena HI, Mizia-Stec K, et al. Current understanding of mitral valve prolapse syndrome and related arrhythmia: State-of-the-Art Review. Kardiol Pol 2026.