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Arrhythmic MVP Phenotype — EECC MCQ

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HardValvular Heart DiseaseArrhythmic MVP PhenotypeEECC

A 55-year-old man with mitral valve prolapse (MVP) has a mid-systolic click and late systolic murmur. He is asymptomatic with normal LVEF and mild MR. A recent study described 'arrhythmic MVP' as a distinct entity. What are the high-risk features?

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Correct answer: C — Arrhythmic MVP is characterised by complex ventricular arrhythmias (PVCs, NSVT), bileaflet prolapse, mitral annular disjunction (MAD), and inferolateral LGE on CMR — this phenotype carries a small but real SCD risk even without severe MR

Arrhythmic MVP is an emerging high-risk phenotype characterised by: (1) bileaflet (or multi-segment) prolapse; (2) mitral annular disjunction (MAD) — separation between the LA wall and LV myocardium at the mitral annulus, creating abnormal wall motion; (3) inferolateral papillary muscle/LV fibrosis on CMR (LGE pattern); (4) complex ventricular ectopy (frequent PVCs, NSVT, often from the inferolateral papillary muscles — the Purkinje fibres adjacent to the fibrosis serve as arrhythmic substrate); (5) T-wave inversion in inferior leads. This phenotype is associated with SCD, particularly in young women. The 2022 ESC VA/SCD Guidelines recognise arrhythmic MVP as a risk entity. Management includes: beta-blocker therapy, Holter monitoring, CMR for LGE and MAD assessment, and ICD consideration for survivors of cardiac arrest or high-risk features.

Reference: ESC (2022): VA/SCD Guidelines; Basso et al. Arrhythmic MVP