Real-World Implementation of EHRA Consensus on Risk Stratification and Treatment in Arrhythmic Mitral Valve Prolapse (AMVP) Survey

Arrhythmic mitral valve prolapse (AMVP) is defined by the EHRA consensus as MVP with frequent (≥5% PVC burden) or complex ventricular arrhythmias (VA) after excluding other causes. Although the annual risk of sudden cardiac death (SCD) is low (<1%), MVP is present in 4–7% of SCD cases in young victims.

The EHRA consensus proposes a risk stratification framework and a grey zone for ICD implantation beyond standard indications, based on phenotypic risk features and advanced rhythm and imaging assessment. Retrospective application of these criteria suggested substantially higher ICD implantation rates.

Evaluating real-world implementation of these recommendations remains highly relevant, as predicting malignant ventricular arrhythmias and SCD in AMVP—often affecting young, otherwise healthy individuals—remains challenging.

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1.Please confirm that you have read the above and agree to participate in this survey.

Yes

No

2.In which country is your centre located?

3.What is the type of your hospital or medical centre?

Non-university / public hospital

University or academic hospital

Private centre

4.Does your centre have a dedicated electrophysiology department?

Yes

No

5.Is cardiac magnetic resonance (CMR) imaging available at your centre?

Onsite

Offsite with fast-track access

Not available

8.Out of all AMVP cases, what percentage of annual AMVP cases at your centre raise concern for sudden cardiac death and ultimately receive an ICD for primary prevention?

9.What is the estimated annual number of catheter ablation procedures performed for ventricular arrhythmias in the context of AMVP at your centre?

None

< 5

5-10

>10

10.How aware are you of contemporary EHRA AMVP consensus recommendations?

Fully familiar and actively applying

Aware, selectively applying

Aware, not yet applying

Not aware

11.How would you best describe the typical MVP patients referred to your practice?

Highly selected patients with severe symptoms (syncope) or markedly abnormal Holter findings (e.g. NSVT) or sustained ventricular arrhythmia.

Selected patients with symptoms (e.g., palpitations or presyncope) or mildly abnormal Holter findings (e.g., frequent PVCs).

All patients with MVP undergoing Holter monitoring.

12.Do all patients with MVP undergo a risk stratification including focused history, 12-lead ECG, 24h Holter and detailed TTE?

Yes, all of them

None of them

No, only symptomatic patients

15.Which CMR protocol elements are used for AMVP evaluation at your centre? (multiple answers allowed)

Cine LV/RV & MV apparatus views

LGE with papillary muscles & MV apparatus focus

T1 mapping/ECV

Strain analysis

I don’t know / at the radiologist’s discretion

I do not have access to CMR

16.What are the main barriers to CMR utilisation in AMVP at your centre? (multiple answers allowed)

17.To what extent do you collaborate with cardiac imaging specialists in the management of patients with AMVP?

Routinely, as part of standard patient management

In selected challenging cases

Rarely

Never

18.Which of the following would lead you to recommend ILR implantation in AMVP patients? (multiple answers allowed)

19.When would you recommend exercise stress testing in MVP? (multiple answers allowed)

In all patients with MVP

In AMVP patients with high-risk features (history of haemodynamically tolerated VT, unexplained syncope and/or NSVT)

To assess for adrenergic-dependent rhythm disturbances

To assess exercise tolerance in AMVP patients who want to engage in sports

20.What is the role of diagnostic invasive electrophysiological study in clinical decision-making for AMVP patients at your centre?

No role

Yes, in all AMVP patients

Yes, in selected patients with phenotypic risk features (LGE in MV apparatus, MAD)

Yes, if PVC ablation was attempted anyways

21.When PVCs/VT arise from the ventricular outflow tract in patients with MVP, how do you ap-proach treatment?

Treat as idiopathic outflow-tract arrhythmia

Treat as MVP-related arrhythmia, affecting monitoring and therapy decisions

22.In the scenario of AMVP with a history of unexplained syncope and sustained VT or fast/polymorphic non-sustained VT from the mitral apparatus, what would you recommend for the patient next? (multiple answers allowed)

Additional imaging such as CMR with the question of LGE

Schedule for VT ablation

ILR implantation

ICD implantation

23.In the scenario of unexplained syncope or haemodynamically tolerated VT or nsVT would the presence of at least two phenotypic risk features (TWI inferior, polymorphic PVCs, MAD, myxoma-tous leaflets, LA dilatation, reduced LV-EF) influence your next therapeutic decision (ICD, ILR, VT ablation)

Yes

No

24.In the scenario of AMVP with significant symptomatic mitral regurgitation and history of unex-plained syncope and sustained VT originating from the mitral apparatus, what would you recommend for the patient

Schedule for surgery with a close arrhythmia monitoring

Implant an ICD and schedule for surgery

Implant an ICD first, only in the case of LGE in CMR, and schedule for surgery

Perform a VT ablation and schedule for surgery

26.What are the main barriers to implementing consensus recommendations for AMVP management at your centre?

Evidence uncertainty

Unclarity of the recommendations

Access to CMR/expert interpretation

Reimbursement/coverage/resources

27.What follow-up pathways are used for AMVP patients after evaluation at your centre?

At your centre for all patients with MVP

At your centre only for patients with phenotypic risk features (TWI in the inferior leads, multi-ple polymorphic PVCs, MAD, redundant MV leaflets, enlarged LA, LV-EF≤ 50%, LGE).

Ambulatory cardiologist