The Prevalence, Pathophysiological Role and Determinants of Mitral Annular Disjunction Among Patients with Mitral Valve Prolapse: A Systematic Review

Background: Over the last two decades, a number of imaging studies have evaluated the characteristics and clinical implications of mitral annular disjunction (MAD) among patients with mitral valve prolapse (MVP). The present systematic review has been primarily designed to summarize the main findings of these studies and to examine the overall impact of MAD in MVP patients. Methods: All imaging studies assessing the prevalence, pathophysiological role and determinants of MAD in MVP individuals, selected from the PubMed and EMBASE databases, were included. There was no limitation in terms of time period. The risk of bias was assessed by using the National Institutes of Health (NIH) Quality Assessment Tool for Observational Cohort and Cross-Sectional Studies. Results: The full texts of 23 studies on 7718 MVP individuals were analyzed. The overall pooled prevalence of MAD in MVP individuals was 40% (range 5.4-90%). When considering the different imaging modalities for assessing MAD, the average MAD prevalence was 20% for cardiac computed tomography studies, 31.3% for transthoracic echocardiography (TTE) studies, 44.7% for transesophageal echocardiography studies and 47% for cardiac magnetic resonance studies. MAD presence was more commonly associated with female sex, young age, narrow antero-posterior thoracic diameter, symptoms of palpitations and syncope, T-wave inversion in inferolateral leads and frequent and/or complex ventricular arrhythmias (VAs) on electrocardiogram, myxomatous leaflets, bileaflet prolapse, larger mitral valve annulus and non-severe mitral regurgitation on TTE. A total of 12 studies (52.2%) provided follow-up data. Over a median follow-up time of 3.9 yrs (range 1-10.3 yrs), MVP individuals with MAD showed increased risk of clinical arrhythmic events, no difference in survival rate and good surgical outcomes. Conclusions: MAD was present in more than one-third of MVP patients, with a wide range of variability depending on the specific imaging method used for assessing MAD presence and on a nonunivocal MAD definition, with a possible overestimation due to Pseudo-MAD rather than True-MAD measurement. A multimodality imaging approach comprehensive of noninvasive chest shape assessment might improve MAD detection among MVP individuals. It appears that careful serial monitoring for VAs should be mandatory for MAD patients.