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Prevalence of Diastolic and Systolic Mitral Annular Disjunction in Patients With Mitral Valve Prolapse - 02/01/25

Doi : 10.1016/j.echo.2024.10.004 
Giorgio Fiore, MD a, Vincenzo Rizza, MD a, Giacomo Ingallina, MD a, Francesco Ancona, MD a, Stefano Stella, MD a, Federico Biondi, MD a, Paola Cunsolo, MD a, Carlo Gaspardone, MD a, Davide Romagnolo, MD a, Annamaria Tavernese, MD a, Martina Belli, MD a, Davide Margonato, MD a, Anna Palmisano, MD c, Antonio Esposito, MD b, c, Francesco Maisano, MD b, d, Francesco Fulvio Faletra, MD e, Eustachio Agricola, MD a, b,
a Unit of Cardiovascular Imaging, IRCCS Ospedale San Raffaele, Milan, Italy 
b Vita-Salute San Raffaele University, Milan, Italy 
c Experimental Imaging Center, IRCCS Ospedale San Raffaele, Milan, Italy 
d Department of Cardiac Surgery, IRCCS Ospedale San Raffaele, Milan, Italy 
e Senior Imaging Consultant, IRCCS-ISMETT UPMC, Palermo, Italy 

Reprint requests: Eustachio Agricola, MD, Associate Professor of Cardiology, San Raffaele Vita-Salute University, Head of Cardiovascular Imaging Unit, Cardio-Thoracic-Vascular Department, San Raffaele Hospital, IRCCS, Via Olgettina 60, Milan 20132, Italy.Associate Professor of CardiologySan Raffaele Vita-Salute UniversityHead of Cardiovascular Imaging UnitCardio-Thoracic-Vascular DepartmentSan Raffaele HospitalIRCCSVia Olgettina 60Milan20132Italy

Abstract

Backgrounds

Mitral annular disjunction (MAD) is commonly evaluated at end systole. However, a systolic-only disjunction is merely apparent, and 2 distinct phenotypes have been identified: True-MAD (atrial displacement of the posterior leaflet in diastole and systole) and Pseudo-MAD (apparent displacement in systole only). The prevalence of True-MAD and Pseudo-MAD in mitral valve prolapse (MVP) is not known. The aim of this study was to assess the prevalence of True-MAD and Pseudo-MAD in myxomatous MVP patients by transthoracic echocardiography (TTE) and to validate TTE compared to cardiac magnetic resonance (CMR; reference standards).

Methods

Consecutive patients who underwent TTE for MVP were included. Mitral annular phenotype was evaluated in the TTE parasternal long-axis view. Accuracy (against CMR) and intra-/interrater reliability of TTE were also assessed.

Results

Six hundred three consecutive patients were included. The prevalence of True-MAD and Pseudo-MAD was 7% (42) and 37% (221) (P < .05), respectively. Accordingly, 221 of 263 (84%) patients classically classified as “MAD” would have been reclassified as Pseudo-MAD. Pseudo-MAD prevalence and systolic length increased with higher mitral regurgitation (MR) severity (23% for mild MR, 36% for moderate MR, 44% for severe MR [P < .05]; 6 ± 2 mm for mild MR; 8 ± 2 mm for moderate MR; 10 ± 2 mm for severe MR [P < .05]), while True-MAD prevalence was consistent across MR grades. Pseudo-MAD was linked to systolic curling and Pickelhaube. Transthoracic echocardiography showed an overall accuracy of 0.89 (Cohen k 0.80), a substantial interrater agreement of 0.87 (k = 0.76), and an almost perfect intrarater agreement of 0.93 (k = 0.85).

Conclusions

True-MAD, unlike Pseudo-MAD, is rare in patients with MVP. Pseudo-MAD is associated with the grade of MR and other echocardiographic features of advanced myxomatous degeneration. Transthoracic echocardiography is an accurate and reliable first-line method to assess mitral annulus morphology in MVP.

Le texte complet de cet article est disponible en PDF.

Central Illustration

Patients with MVP were subclassified by TTE based on the mitral annulus phenotype in systole and diastole. True-MAD was defined as a clear separation between the central part of the posterior leaflet (P2) and the ventricular myocardium in both diastole and systole. Pseudo-MAD was defined as the presence of only systolic apparent disjunction. Finally, No-MAD was defined as normal leaflet insertion through the entire cardiac cycle. Transthoracic echocardiography was validated against the reference standard CMR. Pseudo-MAD, unlike True-MAD, was highly prevalent in the overall cohort of patients (37%), and its prevalence increased with higher degrees of MR severity (P < .05), while True-MAD prevalence remained almost the same in all MR severity grades. Values are expressed as percentages. ∗P < .05 mild MR vs moderate MR. †P < .05 mild MR vs severe MR. ‡P < .05 moderate MR vs severe MR.



Central Illustration : 

Patients with MVP were subclassified by TTE based on the mitral annulus phenotype in systole and diastole. True-MAD was defined as a clear separation between the central part of the posterior leaflet (P2) and the ventricular myocardium in both diastole and systole. Pseudo-MAD was defined as the presence of only systolic apparent disjunction. Finally, No-MAD was defined as normal leaflet insertion through the entire cardiac cycle. Transthoracic echocardiography was validated against the reference standard CMR. Pseudo-MAD, unlike True-MAD, was highly prevalent in the overall cohort of patients (37%), and its prevalence increased with higher degrees of MR severity (P < .05), while True-MAD prevalence remained almost the same in all MR severity grades. Values are expressed as percentages. ∗P < .05 mild MR vs moderate MR. †P < .05 mild MR vs severe MR. ‡P < .05 moderate MR vs severe MR.


Central IllustrationPatients with MVP were subclassified by TTE based on the mitral annulus phenotype in systole and diastole. True-MAD was defined as a clear separation between the central part of the posterior leaflet (P2) and the ventricular myocardium in both diastole and systole. Pseudo-MAD was defined as the presence of only systolic apparent disjunction. Finally, No-MAD was defined as normal leaflet insertion through the entire cardiac cycle. Transthoracic echocardiography was validated against the reference standard CMR. Pseudo-MAD, unlike True-MAD, was highly prevalent in the overall cohort of patients (37%), and its prevalence increased with higher degrees of MR severity (P < .05), while True-MAD prevalence remained almost the same in all MR severity grades. Values are expressed as percentages. ∗P < .05 mild MR vs moderate MR. †P < .05 mild MR vs severe MR. ‡P < .05 moderate MR vs severe MR.

Le texte complet de cet article est disponible en PDF.

Highlights

Anatomical substrate of MAD implies its presence through the entire cardiac cycle.
Pseudo-MAD is the apparent systolic-only disjunction of the PML.
True-MAD is a real PML insertion on the atrial wall in both diastole and systole.
Pseudo-MAD is more common than True-MAD.
Pseudo-MAD is associated to MR grade and features of the arrhythmic MVP complex.

Le texte complet de cet article est disponible en PDF.

Keywords : Pseudo-MAD, MAD, Ventricular arrhythmias, Mitral annulus, Arrhythmic mitral valve prolapse, Mitral regurgitation

Abbreviations : 3D, ANOVA, CCT, CMR, LGE, LV, MAD, MVP, MR, OR, PLAX, PML, SCD, TEE, TTE


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© 2024  American Society of Echocardiography. Publié par Elsevier Masson SAS. Tous droits réservés.
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Vol 38 - N° 1

P. 1-11 - janvier 2025 Retour au numéro
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