Mitral annulus disjunction is associated with adverse outcome in patients with Marfan syndrome and Loeys-Dietz syndrome

Abstract Funding Acknowledgements Type of funding sources: Public Institution(s). Main source(s): South-Eastern Norway Regional Health Authority Background Mitral valve prolapse is a common finding in patients with Marfan (MFS) and Loeys-Dietz syndromes (LDS). annulus disjunction (MAD) an atrial displacement the hinge point mitral that frequently coexists prolapse, but its clinical relevance connective tissue disorders unknown. Purpose To explore association between MAD severity aortic disease MFS LDS. Methods We included consecutive LDS fulfilling established diagnostic criteria. was identified by echocardiography defined as distance from ventricular myocardium to posterior leaflet (Figure, panel A). Aortic surgery emergency for dissection or prophylactic aneurysm. recorded need including repair replacement. Results 168 whom 103 (61%) had 65 (39%) 69 (41%) patients. performed 112 (67%) (27 dissections 85 interventions). Patients were younger at time than those without (p log rank = 0.02) B). needing greater (8 [7-10] mm vs. 7 [6-8] mm, p 0.04). 12 (7%) patients, more (16% 1%, < 0.001, 0.001) C). Conclusion frequent detected 41% associated severe phenotype age surgery. Screening may provide prognostic information be relevant planning surgical interventions. Figure