Mitral annulus disjunction (MAD) is characterized by a separation between the atrial
wall mitral junction and the left ventricular (LV) free wall. Little is known regarding
cardiac magnetic resonance (CMR) performance to detect MAD and its prevalence in mitral
valve prolapse (MVP). Based on 89 MVP patients (63 women; mean age 64 ± 13) referred
for CMR assessment of MR, either from myxomatous mitral valve disease (MMVP) (n = 40;
45%) or fibroelastic disease (n = 49; 55%), we sought to assess the frequency of MAD
and its consequences on LV morphology. Patients were classified in 2 groups according
to MAD presence (MAD+) or absence (MAD−). MAD (measuring 8 ± 4 mm) was diagnosed in
35% (31 of 89) of MVP patients, more frequently in MMVP than fibroelastic disease
(60% vs 14%). MAD+ was associated with MMVP; bileaflet MVP and nonsustain ventricular
tachycardia but not with the severity of MR. Diagnostic accuracy of transthoracic
echocardiography for the detection of MAD was fair (65% sensitivity, 96% specificity)
with CMR as reference. MAD+ showed significantly enlarged basal and mid LV diameters
and enlarged mitral-annulus diameter. In patients with late gadolinium enhancement,
presence of LV fibrosis at level of papillary muscle was more frequent in MAD+. After
adjustment on age and MR severity, MMVP, and enlarged end-systolic mitral annulus
diameter were independently associated with MAD+. In conclusion, MAD was present in
about 1/3 of MVP patients, mostly in MMVP and independent of MR severity. Enlarged
mitral-annulus and basal LV diameters, nonsustain ventricular tachycardia and papillary
muscle fibrosis were associated with MAD presence.
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Article info
Publication history
Published online: September 10, 2019
Received in revised form:
August 21,
2019
Received:
July 15,
2019
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