Mitral annulus disjunction in consecutive patients undergoing cardiovascular magnetic resonance: Where is the boundary between normality and disease?

Background: The presence of mitral annulus disjunction (MAD) has been considered a high-risk feature for sudden cardiac death based on selected study populations. We aimed to assess the prevalence of MAD in consecutive patients undergoing clinically indicated cardiovascular magnetic resonance (CMR), its association with ventricular arrhythmias, mitral valve prolapse (MVP), and other CMR features. Methods: This single-center retrospective study included consecutive patients referred to CMR at our institution between June 2021 and November 2021. MAD was defined as a ≥1 mm displacement between the left atrial wall-mitral valve leaflet junction and the left ventricular wall during end-systole. MAD extent was defined as the maximum longitudinal displacement. Associates of MAD were evaluated at univariable and multivariable regression analysis. The study endpoint, a composite of (aborted) sudden cardiac death, unexplained syncope, and sustained ventricular tachycardia, was evaluated at a 12-month follow-up. Results: Four hundred and forty-one patients 55 ± 18 years, 267/441 (61%) males) were included, and 29/441 (7%) had MVP. The prevalence of MAD ≥1 mm, 4 mm, and 6 mm was 214/441 (49%), 63/441 (14%), and 15/441 (3%), respectively. Patients with MVP showed a higher prevalence of MAD greater than 1 mm (26/29 (90%) vs 118/412 (46%)); p < 0.001), 4 mm (14/29 (48%) vs 49/412 (12%)); p < 0.001), and 6 mm (3/29 (10%) vs 12/412 (3%)); p = 0.03), and a greater MAD extent (4.2 mm, 3.0-5.7 mm vs 2.8 mm, 1.9-4.0 mm; p < 0.001) compared to patients without MVP. MVP was the only morpho-functional abnormality associated with MAD at multivariable analysis (p < 0.001). A high burden of ventricular ectopic beats at baseline Holter-electrocardiogram was associated with MAD ≥4 mm and MAD extent (p < 0.05). The presence of MAD ≥1 mm (0.9% vs 1.8%; p = 0.46), MAD ≥4 mm (1.6% vs 1.3%; p = 0.87), or MVP (3.5% vs 1.2%; p = 0.32) were not associated with the study endpoint, whereas patients with MAD ≥6 mm showed a trend toward a higher likelihood of the study endpoint (6.7% vs 1.2%; p = 0.07). Conclusion: MAD of limited severity was common in consecutive patients undergoing CMR. Patients with MVP showed higher prevalence and greater extent of MAD. Extended MAD was rarer and showed association with ventricular arrhythmias at baseline. The mid-term prognosis of MAD seems benign; however, prospective studies are warranted to search for potential "malignant MAD extents" to improve patients' risk stratification.