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 cby the Heart Rhythm Society, by the Asia Pacific Heart Rhythm Society, and by the Latin American Heart Rhythm Society

Abstract

Graphical Abstract

Risk stratification scheme. Risk stratification aiming at assessing the risk of VAs and SCD in patients with MVP, involving two phases based on the clinical and imaging context and the uncovered arrhythmia. In the absence of ventricular tachycardia, phenotypic risk features will trigger the intensity of screening for arrhythmia. Green boxes indicate green heart consensus statements and yellow boxes indicate yellow heart consensus statements. High risk - sustained VT, polymorphic NSVT, fast (>180 bpm) NSVT, VT/NSVT resulting in syncope. ICD = implantable cardioverter defibrillator; LA = left atrium; LGE = late gadolinium enhancement; LV-EF = left ventricular ejection fraction; MAD = mitral annular disjunction; MV = mitral valve; PVCs = premature ventricular contractions; TWI = T-wave inversion; VT = ventricular tachycardia. #Additional ECG monitoring method may be used such as loop recorders.

Keywords: Mitral annular disjunction; Mitral valve prolapse; Risk stratification‌; Sudden cardiac death; Ventricular arrhythmia.

Graphical Abstract

Risk stratification scheme. Risk stratification aiming at assessing the risk of VAs and SCD in patients with MVP, involving two phases based on the clinical and imaging context and the uncovered arrhythmia. In the absence of ventricular tachycardia, phenotypic risk features will trigger the intensity of screening for arrhythmia. Green boxes indicate green heart consensus statements and yellow boxes indicate yellow heart consensus statements. High risk - sustained VT, polymorphic NSVT, fast (>180 bpm) NSVT, VT/NSVT resulting in syncope. ICD = implantable cardioverter defibrillator; LA = left atrium; LGE = late gadolinium enhancement; LV-EF = left ventricular ejection fraction; MAD = mitral annular disjunction; MV = mitral valve; PVCs = premature ventricular contractions; TWI = T-wave inversion; VT = ventricular tachycardia. #Additional ECG monitoring method may be used such as loop recorders.

Figure 1

The mitral valve with and without mitral valve prolapse. Transthoracic echocardiographic long-axis view in end-systole zoomed in on the mitral valve with (A) the location of both mitral leaflets within <2 mm under the plane of the mitral annulus (red line) refutes the diagnosis of mitral valve prolapse. (B) A displacement of both leaflets >2 mm (red arrow) above the plane of the annulus (red line) defines mitral valve prolapse. Note the absence of detachment of the posterior leaflet from the left ventricular myocardium (no mitral annular disjunction).

Figure 2

Mitral valve prolapse with and without mitral annular disjunction (MAD) on transthoracic echocardiography (TTE). TTE long-axis view in end-systole displaying bileaflet mitral valve prolapse with (A) MAD (yellow line) of 11 mm length vs. (B) without MAD. The red line indicates the plane of the mitral annulus.

Figure 3

Mitral valve prolapse and mitral annular disjunction (MAD) on transthoracic echocardiography (TTE) and transoesophageal echocardiography (TEE). (A) TEE 3D view, displaying a large prolapse of P2 precisely assessed by ‘photo-realistic’ tools now available. Severe mitral regurgitation is associated as shown on 120° TEE (B) and TTE apical four-chamber views (C), with severe left atrial dilatation. A 10 mm MAD is diagnosed both on these TEE (D) and TTE (E) views.

Figure 4

Mitral valve prolapse and mitral annulus disjunction (MAD) on cardiac magnetic resonance (CMR). CMR long-axis view in end-systole displaying mitral valve prolapse with MAD (yellow arrow). The red line indicates the plane of the mitral annulus.

Figure 5

Twelve-lead electrocardiogram (ECG) showing T wave inversion in infero-lateral leads. An ECG of a 44-year-old female who presented with ventricular fibrillation and was found to have mitral valve prolapse. The tracing is remarkable for T wave inversion in leads II, III, AVF, V4-V6.

Figure 6

24 h Holter monitor showing complex ventricular ectopy and non-sustained ventricular tachycardia (NSVT). 24 h Holter monitoring of two patients with mitral valve prolapse, mitral annular disjunction, and palpitations. It shows a right bundle branch block with non-sustained ventricular tachycardia, likely from the posteromedial papillary muscle.

Figure 7

Left ventricular mechanical dispersion associated with mitral annular disjunction (MAD). First panel: Transthoracic echocardiographic long-axis view in end-systole (A) displaying mitral valve prolapse with MAD (yellow arrow). Transthoracic echocardiographic apical four-chamber (B) three-chamber (C) and two-chamber (D) views for the measurement of global longitudinal strain (E). Note the disjunction inducing mechanical dispersion and post-systolic shortening of the posterior-basal segment of the left ventricle (in red). The patient has been seen for ventricular premature complexes. (F) The disjunction is associated with fibrosis in the basal inferolateral segments of the left ventricle and mild mitral regurgitation. Second panel: Transthoracic echocardiographic long-axis view in end-systole (G) displaying mitral valve prolapse with MAD (yellow arrow). Note here the disjunction involving all the insertion of the posterior leaflet on the annulus on transthoracic echocardiographic apical three-chamber (H) and four-chamber views (I, J, K). The depth of the disjunction is only mild yet the circumferential extension is extensive. (L) It leads to mechanical dispersion, post-systolic shortening of the posterior-basal segment of the left ventricle. The patient has been seen for palpitations and > 10% ventricular premature complexes.

Figure 8

Typical examples of premature ventricular contractions arising from the mitral apparatus. Premature ventricular complexes (PVCs) arising from the posterior papillary muscle (PPM) are characterized by right bundle branch block (RBBB) morphology, a predominantly negative QRS in V5, QRS >130 ms and superior axis with both leads II and III negative. Foci in the anterior papillary muscle (APM) also yield a RBBB morphology, typically with a late R/S transition (V3 to V5), QRS >130 ms and lead II discordantly negative while lead III is positive. Ectopy originating from the mitral annulus result in RBBB morphology with positive precordial concordance. PVCs form the anterior mitral annulus (AMA) are defined by an inferior QRS axis while those originating from the posterior mitral annulus (PMA) result in a superior axis. Image courtesy of Jérôme Hourdain, MD, Hôpital la Timone, Marseilles, France.