Key result
Extensive mitral annular disjunction in Marfan syndrome is linked to ~186% higher aortic event risk.
Why the study?
The clinical significance of mitral annular disjunction (MAD) for patients with Marfan syndrome remains largely unexplored.
Does the presence of mitral annular disjunction associate with increased cardiovascular and arrhythmic events in patients with Marfan syndrome?
Cohort (n=142)
No
Does the presence of mitral annular disjunction associate with increased cardiovascular and arrhythmic events in patients with Marfan syndrome?
In patients with Marfan syndrome, the presence of mitral annular disjunction is associated with a higher risk of arrhythmic events, mitral valve interventions, and, when extensive, aortic events.
Answers are grounded in this paper and cite their sources.
MAD may warrant arrhythmia surveillance in Marfan patients; leaves open whether routine assessment improves aortic or arrhythmic outcomes.
Importance: Mitral annular disjunction (MAD) has received particular interest in patients with mitral valve prolapse, ventricular tachycardia, and sudden cardiac death. The clinical significance of MAD for patients with Marfan syndrome (MFS) remains largely unexplored. Objective: To define the prevalence of MAD and examine its association with cardiovascular outcomes and arrhythmia among patients with MFS. Design, Setting, and Participants: This retrospective, single-center cohort study included 142 patients with a diagnosis of MFS based on the revised Ghent criteria and a confirmed (likely) pathogenic variant in the FBN1 gene who underwent regular follow-up between January 1, 2004, and December 31, 2019. Main Outcomes and Measures: The presence of MAD was assessed by echocardiography, and the extent of MAD was categorized in tertiles. Patients also underwent resting electrocardiography and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (defined as sustained ventricular tachycardia or sudden cardiac death), and mitral valve surgery. Results: A total of 142 patients (72 female patients [51%]; median age at first examination, 25 years [range, 2-64 years]) were evaluated. Forty-eight patients (34%) had MAD. Patients with MAD had larger aortic root z scores than patients without MAD (4.1 [interquartile range, 2.8-5.7] vs 3.0 [interquartile range, 1.8-4.0]; P < .001) and more often had mitral valve prolapse (34 of 48 [71%] vs 14 of 94 [15%]; P < .001), ventricular ectopy (14 of 33 [42%] vs 15 of 70 [21%]; P = .03), and nonsustained ventricular tachycardia (13 of 33 [39%] vs 12 of 70 [17%]; P = .01). During follow-up, aortic events occurred at similar rates among patients with vs without MAD (15 of 43 [35%] vs 21 of 84 [25%]; P = .24), but patients in the upper MAD tertile (>10 mm) showed a higher occurrence of aortic events compared with patients with MAD of 10 mm or smaller (9 of 15 [60%] vs 6 of 28 [21%]; P = .01). Patients with arrhythmic events (n = 5) and patients requiring mitral valve surgery (n = 7) were observed exclusively in the group displaying MAD. Conclusions and Relevance: This study suggests that MAD among patients with MFS is associated with the occurrence of arrhythmic events, a higher need for mitral valve intervention, and, among patients with extensive MAD, more aortic events. Cardiac imaging for patients with MFS should consider the assessment of MAD as a potential marker for adverse outcomes.
No takes yet. Share an insight, caveat, or question.
Demolder et al. (2021) conducted a cohort in Marfan syndrome (n=142). Mitral annular disjunction vs. No mitral annular disjunction was evaluated on Aortic events (aortic dissection or prophylactic aortic surgery) (p=.24). Mitral annular disjunction in Marfan syndrome was associated with arrhythmic events and mitral valve surgery exclusively, while extensive MAD >10 mm increased aortic events (60% vs 21%; P=.01).
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: