Benjamin Essayagh1, Avi Sabbag2, Clémence Antoine3, Giovanni Benfari4, Roberta Batista3, Li-Tan Yang3, Joseph Maalouf3, Prabin Thapa3, Samuel Asirvatham3, Hector I Michelena3, Maurice Enriquez-Sarano5. 1. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA; Department of Cardiovascular Medicine, Simone Veil Hospital, Cannes, France. 2. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Davidai Arrhythmia Center, Chaim Sheba Medical Center, Tel Hashomer, Israel. 3. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA. 4. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA; Department of Cardiovascular Medicine, University of Verona, Verona, Italy. 5. Department of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: sarano.maurice@gmail.com.
Abstract
OBJECTIVES: The aim of this study was to assess in patients with mitral valve prolapse (MVP) mitral annular disjunction (MAD) prevalence, phenotypic characteristics, and long-term outcomes (clinical arrhythmic events and excess mortality). BACKGROUND: Clinical knowledge regarding MAD of MVP remains limited and controversial, and its potential link with untoward outcomes is unsubstantiated. METHODS: A cohort of 595 (278 women, mean age 61 ± 16 years) consecutive patients with isolated MVP, with comprehensive clinical, rhythmic, Doppler echocardiographic, and consistent MAD assessment, were examined. MAD prevalence, associated MVP phenotypes, and outcomes (survival, clinical arrhythmic events) starting at diagnostic echocardiography were analyzed. To balance important baseline differences, propensity scoring matching was conducted among patients with and those without MAD. RESULTS: The presence of MAD was common (n = 186 [31%]) in patients with MVP, generally in younger patients, and was not random but was independently associated with severe myxomatous disease involving bileaflet MVP and marked leaflet redundancy (both P ≤ 0.0002). The presence of MAD was also independently associated with a larger left ventricle (P = 0.005). Age-matched cohort survival after MVP diagnosis was not worse with MAD (10-year survival 93% ± 2% for patients without MAD and 97% ± 1% for those with MAD; P = 0.40), even adjusted comprehensively for MVP characteristics (P = 0.80) and accounting for time-dependent mitral surgery (P = 0.60). During follow-up, 170 patients had clinical arrhythmic events (ventricular tachycardia, n = 159; arrhythmia ablation, n = 14; cardioverter-defibrillator implantation, n = 14; sudden cardiac death, n = 3). MAD was independently associated with higher risk for arrhythmic events (adjusted HR: 2.60; 95% CI: 1.87-3.62; P < 0.0001). The link between MAD and arrhythmic events persisted with time-dependent mitral surgery (adjusted HR: 2.54; 95% CI: 1.84-3.50; P < 0.0001), was strong under medical management (adjusted HR: 3.21; 95% CI: 2.03-5.06; P < 0.0001) but was weaker after mitral surgery (adjusted HR: 2.07; 95% CI: 1.24-3.43; P = 0.005). CONCLUSIONS: This large cohort with MVP comprehensively characterized shows that MAD is frequent at MVP diagnosis and is strongly linked to advanced myxomatous degeneration. The presence of MAD was independently associated with long-term excess incidence of clinical arrhythmic events. However, within the first 10 years post-diagnosis, MAD was not linked to excess mortality, and although reassurance should be provided from the survival point of view, careful monitoring for arrhythmias is in order for MAD.
OBJECTIVES: The aim of this study was to assess in patients with mitral valve prolapse (MVP) mitral annular disjunction (MAD) prevalence, phenotypic characteristics, and long-term outcomes (clinical arrhythmic events and excess mortality). BACKGROUND: Clinical knowledge regarding MAD of MVP remains limited and controversial, and its potential link with untoward outcomes is unsubstantiated. METHODS: A cohort of 595 (278 women, mean age 61 ± 16 years) consecutive patients with isolated MVP, with comprehensive clinical, rhythmic, Doppler echocardiographic, and consistent MAD assessment, were examined. MAD prevalence, associated MVP phenotypes, and outcomes (survival, clinical arrhythmic events) starting at diagnostic echocardiography were analyzed. To balance important baseline differences, propensity scoring matching was conducted among patients with and those without MAD. RESULTS: The presence of MAD was common (n = 186 [31%]) in patients with MVP, generally in younger patients, and was not random but was independently associated with severe myxomatous disease involving bileaflet MVP and marked leaflet redundancy (both P ≤ 0.0002). The presence of MAD was also independently associated with a larger left ventricle (P = 0.005). Age-matched cohort survival after MVP diagnosis was not worse with MAD (10-year survival 93% ± 2% for patients without MAD and 97% ± 1% for those with MAD; P = 0.40), even adjusted comprehensively for MVP characteristics (P = 0.80) and accounting for time-dependent mitral surgery (P = 0.60). During follow-up, 170 patients had clinical arrhythmic events (ventricular tachycardia, n = 159; arrhythmia ablation, n = 14; cardioverter-defibrillator implantation, n = 14; sudden cardiac death, n = 3). MAD was independently associated with higher risk for arrhythmic events (adjusted HR: 2.60; 95% CI: 1.87-3.62; P < 0.0001). The link between MAD and arrhythmic events persisted with time-dependent mitral surgery (adjusted HR: 2.54; 95% CI: 1.84-3.50; P < 0.0001), was strong under medical management (adjusted HR: 3.21; 95% CI: 2.03-5.06; P < 0.0001) but was weaker after mitral surgery (adjusted HR: 2.07; 95% CI: 1.24-3.43; P = 0.005). CONCLUSIONS: This large cohort with MVP comprehensively characterized shows that MAD is frequent at MVP diagnosis and is strongly linked to advanced myxomatous degeneration. The presence of MAD was independently associated with long-term excess incidence of clinical arrhythmic events. However, within the first 10 years post-diagnosis, MAD was not linked to excess mortality, and although reassurance should be provided from the survival point of view, careful monitoring for arrhythmias is in order for MAD.
Authors: Sadie Bennett; Jacopo Tafuro; Marcus Brumpton; Caragh Bardolia; Grant Heatlie; Simon Duckett; Paul Ridley; Prakash Nanjaiah; Chun Shing Kwok Journal: Echo Res Pract Date: 2022-07-13
Authors: Maciej Kubala; Christian de Chillou; Yohann Bohbot; Patrizio Lancellotti; Maurice Enriquez-Sarano; Christophe Tribouilloy Journal: Front Cardiovasc Med Date: 2022-02-15
Authors: Sanne A Groeneveld; Feddo P Kirkels; Maarten J Cramer; Reinder Evertz; Kristina H Haugaa; Pieter G Postema; Niek H J Prakken; Arco J Teske; Arthur A M Wilde; Birgitta K Velthuis; Robin Nijveldt; Rutger J Hassink Journal: J Am Heart Assoc Date: 2022-08-05 Impact factor: 6.106