Mitral annular disjunction and cardiovascular outcome in Marfan syndrome

Abstract

Abstract Background Mitral annular disjunction (MAD) has received growing interest in patients with mitral valve prolapse (MVP), ventricular tachycardia (VT) and sudden cardiac death (SCD). The clinical significance of MAD in Marfan syndrome (MFS) remains largely unexplored. Purpose To define the prevalence of MAD and examine the relation with cardiovascular outcomes and arrhythmia in patients with MFS. Methods The presence of MAD was assessed by echocardiography and the extent of MAD was categorized in tertiles. Patients also underwent resting ECG and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (VT/SCD) and mitral valve surgery. Results A total of 142 patients (median age 25 years [range 2–64], 51% women) were evaluated. Forty-eight (34%) patients had MAD. Patients with MAD had larger aortic root Z-scores (4.1 [2.8–5.7] vs. 3.0 [1.8–4.0], p<0.001) and more often showed MVP (71% vs. 15%, p<0.001), ventricular ectopy (42% vs. 21%, p=0.027) and non-sustained ventricular tachycardia (39% vs. 17%, p=0.014) than patients without MAD. During follow-up, aortic events occurred at similar rates in patients with vs. without MAD (35% vs. 25%, p=0.103), but patients in the upper MAD tertile (>10mm) showed a higher occurrence of aortic events (60% vs. 21%, p=0.011) compared to patients with MAD(+) ≤10mm. Arrhythmic events and a need for mitral valve surgery were observed exclusively in patients with MAD (p=0.004 and p<0.001, respectively). Conclusions MAD is associated with a higher need for mitral valve intervention, occurrence of arrhythmic events and, in patients with extensive MAD, more aortic events. Cardiac imaging in MFS should consider the assessment of MAD as a potential marker for adverse outcome. Funding Acknowledgement Type of funding sources: None. Cardiovascular outcome and arrhythmia