Background Arrhythmic mitral valve prolapse (AMVP) is a cause of sustained ventricular tachyarrhythmias (VAs) and sudden cardiac death (SCD), but the arrhythmias remain only partially understood. Objectives In this worldwide collaboration, this study aimed to characterize the VAs occurring in AMVP patients, explore factors associated with various types of sustained VA, and describe common triggering mechanisms. Methods In this multicenter retrospective cohort study, we collected patients with AMVP and documented VA. Clinical and imaging data, and detailed data of the arrhythmic events were collected. In addition, electrocardiograms or intracardiac tracings capturing the arrhythmic events were analyzed by a core laboratory. Results We included 225 patients from 35 centers (age 44 ± 17 years, 57% female). Late gadolinium enhancement (LGE) was found in 61%. We collected 278 arrhythmic events, of which ventricular fibrillation (VF) was the most frequent (65%), followed by sustained monomorphic ventricular tachycardia (SMVT; 26%). Triggers were most commonly exercise or stress (37%), but 31% had no discernable trigger. SMVT was associated with increasing age (P = 0.03), family history of SCD (P = 0.03), history of syncope (P = 0.05), and myocardial LGE (P = 0.003). Of the 278 events, 140 (50%) had available tracings, where 25% of events were triggered by a short-coupled premature ventricular contraction. Pause-dependent initiation was most frequent (49%), and more likely to lead to VF than to SMVT (P = 0.01). Conclusions The dominant VA in AMVP was VF, although SMVT was also common and associated with older age, family history of SCD, syncope, and myocardial LGE. The most common initiation was pause dependent leading to VF.
Avi Sabbag, Nina Ajmone-Marsan, Fatima Ezzeddine, Guido Ascione, Nicolò Azzola Guicciardi, et al.. Characterizing Sustained Arrhythmias in Patients With Arrhythmic Mitral Valve Prolapse. JACC: Clinical Electrophysiology, 2025, ⟨10.1016/j.jacep.2025.10.004⟩ (lien externe). ⟨hal-05375339⟩ (lien externe)
Citations
Sabbag, A., Ajmone-Marsan, N., Ezzeddine, F., Ascione, G., Guicciardi, N. A., Baudinaud, P., Beinart, R., Ben Kilani, M., Bergonti, M., Bhardwaj, P. K., Boveda, S., Czimbalmos, C., Cristin, L., Conte, G., del Carpio, F., Delling, F., de Riva, M., Deharo, J.-C., Escande, W., … Aabel, E. (2025). Characterizing Sustained Arrhythmias in Patients With Arrhythmic Mitral Valve Prolapse. In JACC: Clinical Electrophysiology. https://dx.doi.org/10.1016/j.jacep.2025.10.004
Sabbag, Avi, et al. “Characterizing Sustained Arrhythmias in Patients With Arrhythmic Mitral Valve Prolapse.” JACC: Clinical Electrophysiology, Nov. 2025, https://dx.doi.org/10.1016/j.jacep.2025.10.004.
Sabbag, Avi, Nina Ajmone-Marsan, Fatima Ezzeddine, et al. 2025. “Characterizing Sustained Arrhythmias in Patients With Arrhythmic Mitral Valve Prolapse.” In JACC: Clinical Electrophysiology. https://dx.doi.org/10.1016/j.jacep.2025.10.004.
Sabbag, A. et al. (2025) “Characterizing Sustained Arrhythmias in Patients With Arrhythmic Mitral Valve Prolapse,” JACC: Clinical Electrophysiology. Available at: https://dx.doi.org/10.1016/j.jacep.2025.10.004.
SABBAG, Avi, AJMONE-MARSAN, Nina, EZZEDDINE, Fatima, ASCIONE, Guido, GUICCIARDI, Nicolò Azzola, BAUDINAUD, Pierre, BEINART, Roy, BEN KILANI, Mouna, BERGONTI, Marco, BHARDWAJ, Priya Kumar, BOVEDA, Serge, CZIMBALMOS, Csilla, CRISTIN, Luca, CONTE, Giulio, DEL CARPIO, Freddy, DELLING, Francesca, DE RIVA, Marta, DEHARO, Jean-Claude, ESCANDE, William, FONT, Jonaz, FOLTRAN, Deborah, GUERRA, Federico, GUENANCIA, Charles, HAUGAA, Kristina, HADDAD, Christelle, HERMIDA, Alexis, HUTTIN, Olivier, JACON, Peggy, KREBSOVÁ, Alice, LAREDO, Mikael, LIM, Han, LÓPEZ-SANTI, Pilar, MAURY, Philippe, MANSOURATI, Jacques, MILMAN, Anat, MAZOR, Efrat, NAGY, Andrea, PRZYBYLSKI, Andrzej, SABA, Magdi, SOHNS, Christian, STICHERLING, Christian, SIONTIS, Konstantinos, TER BEKKE, Rachel M.A., TIXIER, Romain, ACHA, Moshe Rav, RICHARD-VITTON, Robin, WINKEL, Bo Gregers, OLLITRAULT, Pierre and AABEL, Eivind, 2025. Characterizing Sustained Arrhythmias in Patients With Arrhythmic Mitral Valve Prolapse [en ligne]. November 2025. Disponible à l'adresse : https://dx.doi.org/10.1016/j.jacep.2025.10.004