Rapid Fire Abstracts
Kenan Kaya, MD
Resident
University of Cologne, Germany
Kenan Kaya, MD
Resident
University of Cologne, Germany
Jonathan Kottlors, MD
Resident
University of Cologne, Germany
Thorsten Gietzen, MD
Resident
University of Cologne, Germany
Leon Bischoff
Physician
University Hospital Bonn, Germany
Moritz C. Halfmann, MD
Radiology Resident
University Medical Center Mainz, Germany
Jan M. Brendel, MD
Resident
University of Tuebingen, Germany
Philipp von Stein, MD
Consultant
University of Cologne, Germany
Jan Paul Janßen, MD
Resident
University of Cologne, Germany
Roman Johannes Gertz, MD
Resident
University of Cologne, Germany
David Maintz, MD
Head of Department
University Hospital of Cologne, Department of Radiology, Germany
Kilian Weiss, PhD
Clinical Scientist
Philips Healthcare, Hamburg, Germany, Germany
Christopher Hohmann, MD
Consultant
University of Cologne, Germany
Patrick Krumm, MD
Professor
University of Tuebingen, Germany
Tilman Emrich, MD
Junior Consultant
University Medical Center Mainz, Germany
Julian A. Luetkens, MD
Physician
University Hospital Bonn, Germany
Carsten H. Gietzen, MD
Consultant
University of Cologne, Germany
Lenhard Pennig, MD
Consultant
University of Cologne, Germany
Mitral annular disjunction (MAD) refers to the separation of the mitral valve annulus from the left ventricular (LV) myocardium. MAD has recently gained interest in research and clinical practice; however, its prevalence in Marfan syndrome (MFS) based on cardiovascular magnetic resonance (CMR) imaging data is unknown. The purpose of this study was to evaluate the prevalence of MAD in MFS patients using CMR and to examine its association with other CMR findings.
Methods:
This retrospective multicenter study included baseline CMR studies of patients treated for MFS at four tertiary care medical centers with diagnosis based on the revised Ghent criteria and a confirmed (likely) pathogenic FBN1 gene variant. The authors evaluated the datasets for MAD (at the attachment of the mitral valve to the anterior, anterolateral, inferolateral, and inferior segments; ≥1 mm), mitral valve prolapse (MVP; ≥2 mm), aortic root z-score, LV ejection fraction (LVEF), LV end-diastolic diameter (LVEDD), and left atrial (LA) size.
Results:
Among 91 patients (28.9±14.0 years, 43 (47.3%) female, body surface area (BSA) 1.9±0.4), 74 (81.3%) had MAD (6.1±2.6 mm), most commonly at the inferior ventricular wall (66 (72.5%), 6.9±3.2 mm). The remaining sites showed a similar prevalence (anterior: 56 (61.5%), 6.0±2.5 mm; anterolateral: 55 (60.4%), 6.1±3.1 mm; inferolateral: 54 (59.3%), 7.0±3.6 mm). There were no significant differences (all p >0.05) between MAD and no MAD groups in aortic root z-score (5.3±3.1 vs. 5.5±3.1), LVEF (62.3±8.0 vs. 57.7±14.7 %), LVEDD/BSA (29.7±7.7 vs. 30.0±8.6 mm/m2), and LA size (11.2±3.1 vs. 11.4±2.7 cm/m2). Decreased LVEF was associated with anterolateral (r=-0.46) and inferolateral (r=-0.39) MAD, whereas increased LVEDD/BSA was mainly observed in anterior (r=0.63) and anterolateral (r=0.46) MAD. Thirty-five patients (38.5%) had MVP, predominantly in patients with MAD (33 (36.3%) vs. 2 (2.2%), p=0.017), especially in anterior MAD (r=0.69).
Conclusion:
MAD is highly prevalent in MFS and mostly located at the inferior site. While aortic dimensions, LV parameters, and LA size in patients with MAD were not different in patients without MAD, certain locations showed an association with decreased LV function and increased diameter. MVP was almost exclusively found in MAD and was mostly associated with anterior MAD.
Figure legend: Cardiovascular magnetic resonance (2-chamber view (A), 4-chamber view (B), 3-chamber view (C and D)) in a 29-year-old male with Marfan syndrome depicting mitral annular disjunction at all four sites (blue arrow: anterior, green arrow: inferior, red arrow: anterolateral, yellow arrow: inferolateral; A-C). Note the concomitant bileaflet mitral valve prolapse (green line: anterior mitral leaflet, black line: posterior mitral leaflet, black interrupted line: virtual annular plane, D).
Figure Mitral Annular Disjunction SCMR.pdf
