Calcagno Tess, El Roumi Joseph, Black Christopher, James Karen, Tang Wilson, Nguyen Christopher, Kwon Deborah
Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio, USA.
JACC Case Rep. 2025 Jun 11;30(14):103685. doi: 10.1016/j.jaccas.2025.103685.
Mitral annular disjunction (MAD) is a structural abnormality increasingly recognized in association with mitral valve prolapse (MVP) and cardiomyopathy. Its clinical significance in nonischemic cardiomyopathy (NICM) is less understood.
A 60-year-old man with NICM presented with progressive dyspnea and atrial fibrillation. Initial cardiac magnetic resonance imaging (CMR) showed severe left ventricular (LV) dilation, reduced ejection fraction (16%), and mild MVP without MAD. After 18 months of guideline-directed medical therapy, LV function normalized, but severe mitral regurgitation and significant MAD became more apparent. Surgical repair with a quadrangular resection and annuloplasty was successful, resolving symptoms.
This case highlights the role of CMR in identifying MAD in NICM, and demonstrates how reverse LV remodeling can unmask MAD and MVP. Reduced tethering forces on mitral chordae after LV size reduction likely contributed to revealing the underlying pathology of bi-leaflet redundancy and prolapse.
二尖瓣环分离(MAD)是一种与二尖瓣脱垂(MVP)和心肌病相关的结构异常,越来越受到关注。其在非缺血性心肌病(NICM)中的临床意义尚不太清楚。
一名患有NICM的60岁男性出现进行性呼吸困难和心房颤动。最初的心脏磁共振成像(CMR)显示严重左心室(LV)扩张、射血分数降低(16%)以及轻度MVP但无MAD。经过18个月的指南指导药物治疗后,LV功能恢复正常,但严重二尖瓣反流和明显的MAD变得更加明显。采用四边形切除术和瓣环成形术进行手术修复成功,症状得以缓解。
该病例突出了CMR在识别NICM中MAD的作用,并展示了左心室逆向重构如何揭示MAD和MVP。左心室大小减小后二尖瓣腱索上的牵拉力降低可能有助于揭示双叶瓣冗余和脱垂的潜在病理。