EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction complex

EHRA expert consensus statement on arrhythmic mitral valve prolapse and mitral annular disjunction complex

in collaboration with the ESC Council on valvular heart disease and the European Association of Cardiovascular Imaging endorsed by the Heart Rhythm Society, by the Asia Pacific Heart Rhythm Society, and by the Latin American Heart Rhythm Society

REVIEWED BY Alison White, AFASA | ASA SIG: Cardiac

REFERENCE | Authors: Sabbag A, Essayagh B, Ramírez Barrera JD, Basso C, Berni A, et al.

WHY THE STUDY WAS PERFORMED

Patients with arrhythmic mitral valve prolapse (AMVP) represent a small cohort of patients in the general population who present with a heightened risk profile of sudden cardiac death (SCD). Given the serious nature of the anatomical derangement of mitral valve prolapse (MVP), mitral annular disjunction (MAD) and the potential for the development of life-threatening ventricular arrhythmias, the aims of this study included defining a comprehensive imaging protocol for transthoracic echocardiography to assess the presence and complexity of AMVP.

HOW THE STUDY WAS PERFORMED

This consensus statement reviewed and summarised the current literature published regarding the AMVP complex. Expert statements, guidelines and evidence from the literature were collected and the information was summarised to arrive at the position of practical strategies and tips on how to define and identify AMVP complex, as well as provide guidance on risk stratification and management of this complex anatomical and arrhythmic disorder.

MAD can be observed at different locations on the mitral annulus. However, MAD is associated with increased risk of VAs when observed at the posterior LV wall.

WHAT THE STUDY FOUND

• A clear definition of MVP was agreed upon with MVP defined as a systolic displacement of one or both mitral leaflets ≥ 2 mm above the plane of the mitral annulus in the sagittal (parasternal long-axis view in echocardiographic imaging) view of the mitral valve.

• Diagnostic criteria for MAD were defined and the use of echocardiography to determine the presence of MAD was recommended as an accessible and relevant screening tool. Importantly, this paper decreases the ‘guess’ work previously associated with the echocardiographic assessment of MAD, due to the clear summary of diagnostic criteria produced in Box 1 on page 1985.

• The accurate quantification of mitral regurgitation (MR) in AMVP patients is crucial as an increase in mortality in AMVP patients has been associated with increasing MR severity.

• Further research is required on large cohorts of patients to further quantify and determine the mechanisms of VAs in MVP patients; how the progression of MVP and associated progression of the severity of MR impacts the management strategies in patients with potential for VAs; the clinical outcomes of screening for VAs to better define low, medium and high risk categories of patients.

RELEVANCE TO CLINICAL PRACTICE

• Patients with suspected MVP should be carefully screened as there is a characteristic phenotype of MVP linked to SCD, where patients with MVP with malignant ventricular arrhythmias (VAs) have an increased risk of deterioration to SCD as the VAs progress in severity.

• MAD will be more accurately identified by cardiac sonographers given the clear summary of diagnostic criteria for MAD that this paper provides.

• A critical component of this paper was the publication of a clear and detailed echocardiographic protocol which is guideline-based and standardised. This protocol is found on page 1995 of the paper and should be mandatory reading for all cardiac sonographers, both newly qualified and experienced. In doing so, misdiagnosis of MVP and MAD can be avoided, and thus the accuracy of determination of patients at risk of SCD can be improved.