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Abnormal "curling" Contraction Is Not Unique To Barlow's Disease
Mario Castillo-Sang1, Rochus K. Voeller2.
1Saint Elizabeth Healthcare, Edgewood, KY, USA, 2University of Minnesota, Minneapolis, MN, USA.

Objective: Barlow's disease has a characteristic contractile pattern of ‘curling motion' of the basal wall. Previously it has been proposed the accompanying bileaflet prolapse was due to the large leaflets acting as ‘sails' pulling the papillary muscles upwards in systole. Clinical observation of this contractile pattern in non-Barlow's patients with bileaflet prolapse suggests this hypothesis is wrong and that this bileaflet prolapse with regurgitation originates from a contractile pattern. We previously showed a weakness of the basal-inferior and inferolateral walls on echocardiographic strain accompanies this contractile pattern and described it as end-systolic basal eversion (ESBE). Methods:Three non-Barlow's disease patients with ESBE contractile pattern underwent mitral repair for posterior ruptured chordae. Echocardiograms over 3 years prior showed ESBE with bileaflet prolapse without flail segments or annular disjunction.Results: All initial echocardiograms confirmed ESBE with apical/outward displacement of the annulus and base, atrial projection of both leaflets, and loss of coaptation with late central mitral regurgitation. All three patients had decreased strain in the basal-inferior and inferolateral segments. Intraoperative evaluation ruled-out Barlow's disease and all were successfully repaired will complete annuloplasty rings which resolved the anterior component of the prolapse and neochordae to the flail posterior segment.Conclusions: The ‘curling' contraction typical of Barlow's disease projecting the base and annulus outward and apical with atrial leaflet motion can be present in non-Barlow's patients and can precede posterior chordal rupture. This contractile pattern is better described as end-systolic basal eversion and is repaired with complete annuloplasty and neochords to flail segments. Resolution of anterior leaflet prolapse despite no direct intervention suggests that the bileaflet prolapse is provoked by ESBE contractile pattern.


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